Neurology_Adams

Neurology_Adams. brain tumors, and others have commented on the same type of headache with parenchymal tumors. These are severe headaches that reach their peak intensity in a few seconds, last for several minutes or as long as an hour, and then subside quickly. When they are associated with vomiting, transient blindness, leg weakness causing “drop attacks,” and loss of consciousness, there is a possibility of brain tumor with greatly elevated intracranial pressure. With respect to its onset, this headache almost resembles that of subarachnoid hemorrhage, but the latter is far longer-lasting and even more abrupt in onset. In its entirety, this paroxysmal headache is most typical of the aforementioned colloid cyst of the third ventricle, but it can occur with other tumors as well, including craniopharyngiomas, pinealomas, and cerebellar masses.


Neurology_Adams

Neurology_Adams. Headaches of Temporal Arteritis (Giant Cell Arteritis) (See Also Chap. 33) This type of inflammatory disease of cranial arteries is an important cause of headache in older persons. All of our patients have been older than 55 years of age, most of them older than age 65. From a state of normal health, the patient develops an increasingly intense throbbing or nonthrobbing headache, often with superimposed sharp, stabbing pains. In a few patients the headache has had an almost explosive onset. The pain is usually unilateral, sometimes bilateral, and often localized to the site of the affected arteries in the scalp. The pain persists to some degree throughout the day and is particularly severe at night. It lasts for many months if untreated. The superficial temporal and other scalp arteries are frequently thickened and tender and without pulsation. Jaw claudication and ischemic nodules on the scalp, with ulceration of the overlying skin, have been described in severe cases.


Neurology_Adams

Neurology_Adams. Many of the patients feel generally unwell and have lost weight; some have a low-grade fever and anemia. Usually the sedimentation rate is greatly elevated (>50 mm/h and typically >75 mm/h) but elevation of the C-reactive protein (CRP) level is a more sensitive indicator of this inflammatory condition and is particularly helpful when the sedimentation rate is only mildly elevated. A few patients have a peripheral neutrophilic leukocytosis. Half of patients have generalized aching of proximal limb muscles, reflecting the presence of polymyalgia rheumatica (see Chap. 45, “Polymyalgia Rheumatica”). A relation of temporal arteritis to herpes zoster has been proposed.


Neurology_Adams

Neurology_Adams. The importance of early diagnosis relates to the threat of blindness from thrombosis of the ophthalmic or posterior ciliary arteries. This may be preceded by several episodes of amaurosis fugax (transient monocular blindness). Ophthalmoplegia may also occur but is less frequent, and its cause, whether neural or muscular, is not settled.


Neurology_Adams

Neurology_Adams. Masticatory claudication is a specific but not particularly sensitive symptom of cranial arteritis. The large intracranial vessels are occasionally affected, thereby causing stroke. Once vision is lost, it is seldom recoverable. For this reason, the earliest suspicion of cranial arteritis should lead to the administration of corticosteroids and then to biopsy of the appropriate scalp artery. Microscopic examination discloses an intense granulomatous or “giant cell” arteritis. If biopsy on one side fails to clarify the situation and there are sound clinical reasons for suspecting the diagnosis, the other side should be sampled. Arteriography of the external carotid artery branches is probably the most sensitive test but is seldom used, because of its relatively higher risk. Ultrasonographic examination of the temporal arteries may display a dark halo and irregularly thickened vessel walls. This technique has not been incorporated into routine evaluation because its sensitivity has not


Neurology_Adams

Neurology_Adams. examination of the temporal arteries may display a dark halo and irregularly thickened vessel walls. This technique has not been incorporated into routine evaluation because its sensitivity has not been established; our own experience suggests that it may miss cases, but it could be useful in choosing the site for biopsy of the temporal artery.


Neurology_Adams

Neurology_Adams. The administration of prednisone, 45 to 60 mg/d in single or divided doses over a period of several weeks, is indicated in all cases, with gradual reduction to 10 to 20 mg/d and maintenance at this dosage for several months or years, if necessary, to prevent relapse. The headache can be expected to improve within a day or two of beginning treatment; failure to do so brings the diagnosis into question. When the sedimentation rate or CRP is elevated, its return to normal, usually over months, is a reliable index of therapeutic response. Whether symptoms or the blood tests are a better guide to reducing the steroid dose is unclear, one should probably be cautious in lowering the medication if the erythrocyte sedimentation rate (ESR) and CRP remain high. Headaches of Pseudotumor Cerebri (Benign or Idiopathic Intracranial Hypertension, See Chap. 29)


Neurology_Adams

Neurology_Adams. Headaches of Pseudotumor Cerebri (Benign or Idiopathic Intracranial Hypertension, See Chap. 29) The headache of pseudotumor cerebri assumes a variety of forms. Most typical is a feeling of occipital pressure that is greatly worsened by lying down, but many patients have—in addition, or only—headaches of migraine or tension type. Indeed, some of them respond to medications such as propranolol and ergot compounds. None of the proposed mechanisms for pain in pseudotumor cerebri seems to be adequate as an explanation, particularly the idea that cerebral vessels are displaced or compressed, as neither has been demonstrated. It is worth noting that facial pain may also be a feature of the illness, albeit rare. Chapter 29 has a more complete description of the clinical features and treatment. After successful treatment for pseudotumor, some patients have persistent headaches that have the flavor of migraine or tension headache.


Neurology_Adams

Neurology_Adams. These are commonly known to neurologists, as noted earlier in this chapter. They occur after lumbar punctures in approximately 5 percent of procedures. The headache is associated with the greatly reduced pressure of the CSF compartment and probably caused by vertical traction on cranial blood vessels. Neck pain may be a prominent, or the only, feature. Assuming the supine position almost immediately relieves the cranial pain and eliminates vomiting, but a blood-patch procedure may be required in persistent cases. In a limited number of cases, success has been obtained by the use of intravenous caffeine injections. The diagnosis is evident either from a lumbar puncture that demonstrates low or zero pressure or perhaps more definitely, by MRI with gadolinium, which has characteristic enhancement of the pachymeninges (dura). The condition and its treatment are discussed in Chaps. 2 and 29 “Lumbar Puncture Headache” and “Spontaneous Intracranial Hypotension.”


Neurology_Adams

Neurology_Adams. Menstrual (Catamenial) Migraine and Other Headaches Linked to the Hormonal Cycle The relation of headache to a drop in estradiol levels during the late luteal phase of ovulation was mentioned in “Migraine” above. There it was also indicated that the mechanism is probably more complex. In practice, factors such as sleep deprivation are probably important in triggering perimenstrual headaches. Premenstrual headache, taking the form of migraine or a combined tension-migraine headache, usually responds to the administration of an NSAID begun 3 days before the anticipated onset of the menstrual period; oral sumatriptan (25 to 50 mg qid) and zolmitriptan (2.5 to 5 mg bid) are also equally effective. Manipulation of the hormonal cycle with danazol (a testosterone derivative) or estradiol has also been effective but is rarely necessary.


Neurology_Adams

Neurology_Adams. The management of migraine during pregnancy poses special problems because one wishes to restrict exposure of the fetus to medications. Beta-adrenergic compounds and tricyclic antidepressants may be used safely in the small proportion of women whose headaches persist or intensify during pregnancy. From a limited registry of patients who were given sumatriptan during pregnancy, and from several small trials summarized by Fox and colleagues, no teratogenic effects or adverse effects on pregnancy arose, but serotonin agonist drugs should be used advisedly until their safety is further confirmed. Ergots (DHE) are obviously interdicted because of their capacity to precipitate uterine contractions or labor. For those women who use antiepileptic drugs as a means of headache prevention, it is recommended that the drugs be stopped prior to pregnancy or as soon as it is known that pregnancy has begun.


Neurology_Adams

Neurology_Adams. In the special circumstance of true and debilitating status migrainosus during pregnancy, infusions of magnesium and metoclopramide (in doses previously mentioned in this chapter) are often used but repeated administration and monitoring of blood pressure and tendon reflexes may be needed. This should probably precede resorting to opioids, which may nevertheless become necessary in some cases. In all instances of headache in late pregnancy, the possibilities of toxemia and cerebral venous thrombosis should be considered.


Neurology_Adams

Neurology_Adams. A patient may complain of very severe, transient cranial pain on coughing, sneezing, laughing heartily, lifting heavy objects including weight lifting, stooping, and straining at stool. Pain is usually felt in the front of the head, sometimes occipitally, and may be unilateral or bilateral. As a rule, it follows the initiating action within a second or two and lasts a few seconds to a few minutes. The pain is often described as having a bursting quality and may be of such severity as to cause the patient to cradle his head in his hands, thereby simulating the headache of acute subarachnoid hemorrhage.


Neurology_Adams

Neurology_Adams. Most often this syndrome is a benign idiopathic state that recurs over a period of several months to a year or two and then disappears. Many decades ago, Symonds emphasized the benignity of the condition. In a report of 103 patients followed for 3 years or longer, Rooke found that additional symptoms of neurologic disease developed in only 10. The cause and mechanism have not been determined. During the headache, the CSF pressure is normal. Bilateral jugular compression may induce an attack, possibly because of traction on the walls of large veins and dural sinuses. In a few instances, we have observed this type of headache after lumbar puncture or after a hemorrhage from an arteriovenous malformation.


Neurology_Adams

Neurology_Adams. Patients with cough or strain headache will only occasionally be found to have serious intracranial disease; when present, particularly if a first attack, subarachnoid hemorrhage may be suspected. In other infrequent cases, this type of headache has been traced to lesions of the posterior fossa and foramen magnum, arteriovenous malformation, subdural hematoma, Chiari malformation, or tumor. It may be necessary, therefore, to supplement the neurologic examination by appropriate lumbar puncture, CT and MRI. Far more common, of course, are the temporal and maxillary pains that are caused by dental or sinus disease, which may also be worsened by coughing.


Neurology_Adams

Neurology_Adams. A special variant of exertional headache is “weight-lifter’s headache.” It occurs either as a single event or repeatedly over a period of several months, but each episode of headache may last many hours or days, again raising the suspicion of subarachnoid hemorrhage. The pain begins immediately or within minutes of heavy lifting. If the pain resolves in an hour or less and there is no meningismus or sign of bleeding on the CT, we have foregone lumbar puncture and angiography but have suggested that weight lifting not be resumed for several weeks. Athletes and runners in general seem to suffer exertional headaches quite often in our experience, and the episodes usually have migrainous features.


Neurology_Adams

Neurology_Adams. Indomethacin is usually effective in controlling exertional headaches; this has been confirmed in controlled trials. Useful alternatives are NSAIDs, ergot preparations, and propranolol. In a few of our patients, lumbar puncture appeared to immediately resolve the problem in some inexplicable way.


Neurology_Adams

Neurology_Adams. All manner of headache has been attributed to Chiari type I malformation (with tonsils descended at least 3 mm below the lip of the foramen magnum), but with limited justification. However, some instances of exertional and Valsalva-induced suboccipital pain can be attributed to this developmental abnormality. In the survey by Pascual and colleagues of 50 patients with Chiari type I malformations, approximately a quarter described a fairly specific pattern of pain consisting of bursting, dull, throbbing, or lancinating discomfort for seconds to far longer following Valsalva-related activities, either in the occipital or frontal region and radiating to one or both shoulders. Only the degree of tonsillar descent correlated with the presence of exertional headache and skull abnormalities such as basilar impression were not clearly associated with this type of headache. It follows that suboccipital decompressive operations for headache in this condition should be undertaken only


Neurology_Adams

Neurology_Adams. such as basilar impression were not clearly associated with this type of headache. It follows that suboccipital decompressive operations for headache in this condition should be undertaken only selectively. Chiari malformation is discussed further in Chap. 37.


Neurology_Adams

Headaches Related to Sexual Activity

Neurology_Adams. Headaches Related to Sexual Activity


Neurology_Adams

Neurology_Adams. Lance (1976) described 21 cases of this type of headache, 16 in males and 5 in females. The headache took one of two forms: one in which pain typical of tension headache developed as sexual excitement increased, and another in which a severe, throbbing, “explosive” headache occurred at the time of orgasm and persisted for several minutes or hours (orgasmic headache). The latter headaches were of such abruptness and severity as to suggest a ruptured aneurysm but the neurologic examination was negative in every instance, as was arteriography in 7 patients who were subjected to this procedure. In 18 patients who were followed for a period of 2 to 7 years, no other neurologic symptoms developed. Characteristically, the headache occurred on several consecutive occasions and then disappeared. In cases of repeated coital headache, indomethacin has been effective. Of course, so-called orgasmic headache is not always benign; a hypertensive hemorrhage, rupture of an aneurysm or vascular


Neurology_Adams

Neurology_Adams. In cases of repeated coital headache, indomethacin has been effective. Of course, so-called orgasmic headache is not always benign; a hypertensive hemorrhage, rupture of an aneurysm or vascular malformation, carotid artery dissection, or myocardial infarction may occur during the exertion of sexual intercourse. While there is no authoritative direction, it is justified to perform a spinal tap if a sexual-related headache is the first occurrence of headache in a patient’s history.


Neurology_Adams

Neurology_Adams. This is a severe headache of very abrupt onset and numerous causes, most being less serious than the nature of the symptoms suggest. Of course, the headache of subarachnoid hemorrhage caused by rupture of a saccular (berry) aneurysm is among the most abrupt and dramatic of cranial pains (see Chap. 33). It was in relation to headaches of this nature that the term thunderclap was introduced by Day and Raskin. They attributed the symptoms to an unruptured cerebral aneurysm but the term is now used to denote headache of this description from various causes.


Neurology_Adams

Neurology_Adams. Patients have offered colorful descriptions, such as “being kicked in the back of the head.” Thunderclap headache, as pointed out by Dodick, has been a symptom of pituitary apoplexy, cerebral venous thrombosis, cervical arterial dissection, nonaneurysmal perimesencephalic hemorrhage, or hypertensive crisis (Table 9-4). To this list should be added diffuse idiopathic arterial spasm (Call-Fleming syndrome; see “Diffuse and Focal Cerebral Vasospasm” in Chap. 33) and cerebral vasospasm as the result of the administration of sympathomimetic or serotonergic drugs, including cocaine and the triptan group of medications for the treatment of migraine. The coital and exertional headaches described above may also be considered of this nature. Recurrent thunderclap pain may be particularly indicative of multifocal or diffuse vasospasm, as pointed out by Chen and colleagues, who found this vasculopathy in 39 percent of their patients with recurrent thunderclap pain.


Neurology_Adams

Neurology_Adams. Because the pain of thunderclap headache may be indistinguishable from that caused by subarachnoid hemorrhage, even to the extent of being accompanied by vomiting and acute hypertension, the diagnosis is clarified after lumbar puncture and cerebral imaging, and the pain resolves in hours or less. Most cases turn out to be idiopathic. Wijdicks and colleagues confirmed that thunderclap headache is usually a benign condition; among 71 patients followed for more than 3 years they found no serious cerebrovascular lesions. For this reason, these idiopathic thunderclap headaches have been presumed by some workers to be a form of migraine (“crash migraine”). This opinion is based in part on a history of preceding or of subsequent headaches and migrainous episodes in affected individuals; however, in our experience not all of such patients have had migraine in the past. There is a notable tendency for thunderclap headaches to recur as mentioned above.


Neurology_Adams

Neurology_Adams. An intense, generalized, throbbing headache may occur in conjunction with flushing of the face and hands and numbness of the fingers (erythromelalgia). Episodes tend to be present on awakening from sound sleep. This condition, called erythrocyanotic, has been reported in a number of unusual settings: (1) in mastocytosis (infiltration of tissues by mast cells, which elaborate histamine, heparin, and serotonin); (2) with carcinoid tumors; (3) with serotonin-secreting tumors; (4) with some tumors of the pancreatic islets; and (5) with pheochromocytoma. Seventy-five percent of patients with pheochromocytoma reportedly have vascular-type headaches coincident with paroxysms of hypertension and release of catecholamines (Lance and Hinterberger) but the flushing phenomenon has been rare in our experience. Headache Related to Various Medical Diseases


Neurology_Adams

Neurology_Adams. Headache Related to Various Medical Diseases A cardinal feature of meningitis of varied causes is headache. When accompanied by fever and stiff neck, the diagnosis is almost assured. However, severe headache may occur with a number of infectious illnesses caused by banal viral infections, by organisms such as Mycoplasma, and particularly by influenza. There is often accompanying neck pain and slight stiffness. The suspicion of meningitis is raised, even subarachnoid hemorrhage, but there is no reaction in the CSF (“meningism”). The mild aseptic meningitis that accompanies HIV seroconversion may also be accompanied by headache. When persistent and moderately severe, the headache may be classified with “new daily persistent headache” described above.


Neurology_Adams

Neurology_Adams. Approximately 50 percent of patients with chronic and essential hypertension complain of headache, but the relationship of one to the other is probably coincidental. Minor elevations of blood pressure may be a result rather than the cause of headaches. Severe (accelerated) hypertension, with diastolic pressures of more than 120 mm Hg is, however, associated with headache, and measures that reduce the blood pressure relieve the cranial pain. In preeclampsia, the headaches occur at minor degrees of hypertension or normal levels in a woman who has otherwise low blood pressure. Abrupt elevations of blood pressure, as occur in patients who take monoamine oxidase inhibitors and then ingest tyramine-containing food, can cause headaches that are severe enough to simulate subarachnoid hemorrhage. However, it is the individual with moderately severe hypertension and frequent severe headaches that typically confronts the practitioner. In some of these patients, the headaches are of the common


Neurology_Adams

Neurology_Adams. However, it is the individual with moderately severe hypertension and frequent severe headaches that typically confronts the practitioner. In some of these patients, the headaches are of the common migrainous or tension type, but in others, they defy classification. The acute headache of pheochromocytoma correlates with the rate of increase of blood pressure rather than its absolute value. Curiously, headaches that occur toward the end of renal dialysis or soon after its completion are associated with a fall in blood pressure (as well as a decrease in blood sodium levels and osmolality).


Neurology_Adams

Neurology_Adams. Headaches frequently follow a seizure, having been recorded in half of one large series of epileptic patients analyzed by Schon and Blau but the pain was infrequently severe. In migraineurs, the postseizure headache may reproduce a typical migraine attack. Experienced physicians are aware of many other conditions in which headache may be a principal symptom. These include fevers of any cause, carbon monoxide exposure, chronic lung disease with hypercapnia (headaches often nocturnal or early morning), sleep apnea, hypothyroidism, thrombocythemia, Cushing disease, withdrawal from corticosteroid medication or alcohol, mountain (altitude) sickness, exposure to nitrates, cyanotic heart disease, occasionally in adrenal insufficiency, and acute anemia with hemoglobin well below 10 g. No attempt is made here to discuss the symptomatic treatment of headache that may accompany these many medical conditions. Obviously, the guiding principle is to address the underlying disease.


Neurology_Adams

Neurology_Adams. No attempt is made here to discuss the symptomatic treatment of headache that may accompany these many medical conditions. Obviously, the guiding principle is to address the underlying disease. Headache Related to Diseases of the Cervical Spine


Neurology_Adams

Neurology_Adams. Headache Related to Diseases of the Cervical Spine Headaches that accompany diseases of the upper cervical spine are well recognized, but their mechanism is obscure and their frequency possibly overestimated. Recent writings have focused on a wide range of causative lesions, such as apophyseal (facet) arthropathy, C2 dorsal root entrapment, calcified ligamentum flavum, hypertrophied posterior longitudinal ligament, and rheumatoid arthritis of the atlantoaxial region. As summarized by Bogduk and Govind, the most credible evidence for this group of disorders comes from systematic injection of anesthetics into cervical structures and effecting complete relief of headache. Even this is not uniformly successful in patients whose cranial pain has been attributed to a cervicogenic mechanism. CT and MRI have divulged a number of these abnormalities. One special variety is discussed further below, under “‘Third Occipital Nerve’ Headache,” and further in Chap. 10.


Neurology_Adams

OTHER CRANIOFACIAL PAINS (SEE CHAP. 44)

Neurology_Adams. OTHER CRANIOFACIAL PAINS (SEE CHAP. 44)


Neurology_Adams

Neurology_Adams. This is a common disorder of middle age and later life, consisting of paroxysms of intense, stabbing pain in the distribution of the mandibular and maxillary divisions (rarely the ophthalmic division) of the fifth cranial nerve. The pain seldom lasts more than a few seconds or rarely a minute or two, but it is often so intense that the patient winces involuntarily; hence the term tic. It is uncertain whether the tic is reflexive or quasivoluntary. The paroxysms recur frequently, both day and night, for several weeks or months at a time. Another characteristic feature is the initiation of a jab or a series of jabs of pain by stimulation of certain areas of the face, lips, or gums, as in shaving or brushing the teeth, or by movement of these parts in chewing, talking, or yawning, or even by a breeze—the so-called trigger factors. Sensory or motor loss in the distribution of the fifth nerve cannot be demonstrated, though there are minor exceptions to this rule. In addition to the


Neurology_Adams

Neurology_Adams. even by a breeze—the so-called trigger factors. Sensory or motor loss in the distribution of the fifth nerve cannot be demonstrated, though there are minor exceptions to this rule. In addition to the paroxysmal pain, some patients complain of a more or less continuous discomfort, itching, or sensitivity of restricted areas of the face, features regarded as atypical even though not infrequent.


Neurology_Adams

Neurology_Adams. In studying the relationship between stimuli applied to the trigger zones and the paroxysms of pain, touch and possibly tickle are more likely to be precipitants rather than painful or thermal stimulus. Usually a spatial and temporal summation of impulses is necessary to trigger a paroxysm of pain, which is followed by a refractory period of up to 2 or 3 min.


Neurology_Adams

Neurology_Adams. The diagnosis of tic douloureux rests on the strict clinical criteria enumerated above, so that the condition can be distinguished from other forms of facial and cephalic neuralgia and pain arising from diseases of the jaw, teeth, or sinuses. Most cases of trigeminal neuralgia have no obvious cause (idiopathic), in contrast to symptomatic trigeminal neuralgia, in which paroxysmal facial pain is because of involvement of the fifth nerve by some other disease: multiple sclerosis (may be bilateral), aneurysm of the basilar artery, or tumor (acoustic or trigeminal schwannoma, meningioma, epidermoid) in the cerebellopontine angle. Each of the forms of symptomatic trigeminal neuralgia may give rise only to pain in the distribution of the trigeminal nerve, or it may produce a loss of sensation as well. Vascular Loop as a Cause of Trigeminal Neuralgia


Neurology_Adams

Neurology_Adams. Vascular Loop as a Cause of Trigeminal Neuralgia It has become apparent that a proportion of ostensibly idiopathic cases are caused by compression of the trigeminal roots by a small tortuous branch of the basilar artery. This was originally pointed out by Dandy and brought to greater attention by Jannetta, who has observed it frequently and has devised a procedure that is now widely used to relieve the pain by decompression of the trigeminal root. The offending small vessel is removed from contact with the proximal portion of the nerve (see below). The tortuous vessel can often be visualized by special MRI sequences or by MR angiography but these vessels can be found asymptomatic patients as well. This and other disorders of the fifth nerve, some of which give rise to facial pain, are discussed in Chap. 44.


Neurology_Adams

Neurology_Adams. Carbamazepine is effective in 70 to 80 percent of patients (600 to 1,200 mg/d), but half become tolerant over a period of several years. Other antiepileptic drugs such as phenytoin (300 to 400 mg/d), valproic acid (800 to 1,200 mg/d), clonazepam (2 to 6 mg/d), gabapentin (300 to 900 mg/d or more), pregabalin (150 to 300 mg/d), and carbamazepine in combination with other medications, suppress or shorten the duration and severity of the attacks in most patients for varying times. Baclofen may be useful in patients who cannot tolerate carbamazepine or gabapentin, but it is most effective as an adjunct to one of the anticonvulsant drugs. Capsaicin applied locally to the trigger zones or the topical instillation in the eye of an anesthetic has been helpful in some patients. By temporizing and using these drugs, one may permit a spontaneous remission to occur in perhaps 1 in 5 patients over a year or two.


Neurology_Adams

Neurology_Adams. Many patients with intractable pain, however, come to vascular surgery or a surgical form of root destruction. The vascular decompression procedure, which requires a posterior fossa craniotomy but leaves no sensory loss, has been the most consistent approach. Barker and colleagues reported that 70 percent of 1,185 patients were relieved of pain by repositioning a small branch of the basilar artery that was found to compress the fifth nerve, and this benefit persisted with a recurrence rate of less than 1 percent annually for 10 years. The procedure has been done sometimes without confirmation of a vascular loop by vascular imaging.


Neurology_Adams

Neurology_Adams. A procedure employed more in the past but still finds use, was stereotactically controlled thermocoagulation of the trigeminal roots (Sweet and Wepsic). In recent years, there has been a preference for microvascular decompression on the basis of its sparing of sensation, especially late in the course of the illness (Fields). Several forms of stereotactic radiation are less intrusive alternatives, but their full effect is not evident for months. In practice, an antiepileptic medication is often required for some period of time after any of these procedures, and it must be reinstituted when symptoms reoccur, as they often do.


Neurology_Adams

Neurology_Adams. This syndrome is much less common than trigeminal neuralgia but resembles the latter in many respects. The pain is intense and paroxysmal; it originates in the throat, approximately in the tonsillar fossa, and is provoked most commonly by swallowing but also by talking, chewing, yawning, laughing, etc. The pain may be localized in the ear or radiate from the throat to the ear, implicating the auricular branch of the vagus nerve. For this reason, White and Sweet suggested the term vagoglossopharyngeal neuralgia. This is the main craniofacial neuralgia that may be accompanied by bradycardia and even by syncope, presumably because of the triggering of cardioinhibitory reflexes by afferent vagal pain impulses. There is no demonstrable sensory or motor deficit. Rarely, tumors, including carcinoma, lymphoma or epithelioma of the oropharyngeal-infracranial region or peritonsillar abscess may give rise to pain that is clinically indistinguishable from glossopharyngeal neuralgia.


Neurology_Adams

Neurology_Adams. For idiopathic glossopharyngeal neuralgia, a trial of carbamazepine, gabapentin, pregabalin, or baclofen may be useful. If these are unsuccessful, the conventional surgical procedure had been to interrupt the glossopharyngeal nerve and upper rootlets of the vagus nerve near the medulla but recent observations suggest that a vascular decompression procedure similar to the one used for trigeminal neuralgia and directed to a small vascular loop under the ninth nerve relieves the pain in a proportion of patients.


Neurology_Adams

Neurology_Adams. The common pain and herpetic eruption caused by herpes zoster infection of the gasserian ganglion are practically always limited to the first division (herpes zoster ophthalmicus). Ordinarily, the rash appears within 4 to 5 days or less after the onset of the pain, thereby making the clinical diagnosis difficult; however, treatment should be instituted (see below) based on the clinical likelihood of zoster infection. If the eruption does not appear, some cause other than herpes zoster will almost invariably declare itself; nevertheless, a few cases have been reported in which the characteristic location of pain with serologic evidence of herpes zoster infection was not accompanied by skin lesions.


Neurology_Adams

Neurology_Adams. The acute discomfort associated with the herpetic eruption usually subsides after several days or weeks, or it may linger for several months. It is mostly in the elderly that the pain becomes chronic and intractable. Usually it is described as a constant burning, with superimposed waves of stabbing pain, and the skin in the territory of the preceding eruption is exquisitely sensitive to the slightest tactile stimuli, even though the threshold of pain and thermal perception is elevated. This unremitting postherpetic neuralgia of long duration represents one of the most difficult pain problems with which the physician must deal. Some relief may be provided by application of capsaicin cream, use of a mechanical or electrical cutaneous stimulator, or administration of one of the antiepileptic drugs.


Neurology_Adams

Neurology_Adams. Neuralgia associated with a vesicular eruption caused by the herpes zoster virus may affect the external auditory meatus and pinna and sometimes of the palate and occipital region—with or without deafness, tinnitus, and vertigo—is combined with facial paralysis. This syndrome, since its original description by Ramsay Hunt, has been known as geniculate herpes, and also Ramsay Hunt syndrome (see also Chap. 44). It is clear that the skin of the external ear canal, tympanic membrane and in some patients, the skin behind the ear are supplied by somatic sensory branches that travel with the chorda tympani and greater superficial petrosal nerves and have their cell bodies in the geniculate ganglion.


Neurology_Adams

Neurology_Adams. Treatment with acyclovir, along the lines indicated in Chap. 32, will shorten the period of eruption and the acute pain, but the drug does not prevent its persistence as a chronic pain. There is little data upon which to judge the utility of corticosteroids but they are generally not used (whereas they do provide benefit in Bell’s palsy and antiviral agents are not clearly useful).


Neurology_Adams

Neurology_Adams. Antidepressants such as amitriptyline and fluoxetine are helpful in some patients, and Bowsher has suggested, on the basis of a small placebo-controlled trial, that treatment with amitriptyline during the acute phase may prevent persistent pain. The use of preemptive measures, such as gabapentin or pregabalin administered at the outset, may be effective but a properly performed clinical trial is lacking. The addition of amitriptyline up to 75 mg at bedtime has proved to be a useful measure. Probably equivalent results are obtained by a combination of valproic acid and an antidepressant, as reported by Raftery. King has reported that two 325-mg aspirin tablets crushed and mixed with cold cream or chloroform (15 mL) and spread over the painful zone on the face or trunk relieved the pain for several hours in most patients with postzoster neuralgia. Ketamine cream has been suggested as an alternative. Extensive trigeminal rhizotomy or other destructive procedures should be avoided, as


Neurology_Adams

Neurology_Adams. for several hours in most patients with postzoster neuralgia. Ketamine cream has been suggested as an alternative. Extensive trigeminal rhizotomy or other destructive procedures should be avoided, as these surgical measures are not for long successful and may lead to a superimposed diffuse refractory dysesthetic component on the original neuralgia (anesthesia dolorosa).


Neurology_Adams

Neurology_Adams. Under the heading of “primary trochlear headache,” Yanguela and colleagues have described a periorbital pain that emanates from the superomedial orbit in the region of the trochlea (the pulley of the superior oblique muscle). Most of their patients were women. The pain was worsened by adduction and (paradoxically for the superior oblique) upgaze of the globe on the affected side, in the direction of action of the superior oblique muscle. The authors describe a diagnostic method of examination that begins by having the patient look downward so that the trochlea can be palpated and compressed; the patient then looks upward, eliciting or exaggerating the pain, while the examiner continues compression. Injection of the trochlea with corticosteroids relieved the pain in almost all of these patients. The authors made a distinction between primary trochlear headache and “trochleitis,” which seems to us an ambiguous difference. There is no limitation of ocular movement or autonomic change and


Neurology_Adams

Neurology_Adams. The authors made a distinction between primary trochlear headache and “trochleitis,” which seems to us an ambiguous difference. There is no limitation of ocular movement or autonomic change and imaging of the orbit is normal. This syndrome, with which we have no experience, brings to mind the entity of the Brown syndrome of trochlear entrapment with diplopia and pain (Chap. 13). The above authors were also of the opinion that the trochlea may be a trigger point for migraine.


Neurology_Adams

Neurology_Adams. Pain localized in and around one ear is occasionally a primary complaint. It is commonly the incipient symptom of Bell’s palsy or an outbreak of shingles but there are a number of different causes and mechanisms. During neurosurgical operations in awake patients, stimulation of cranial nerves V, VII, IX, and X causes ear pain, yet interruption of these nerves usually causes no or limited demonstrable loss of sensation in the ear canal or the ear itself (superficial sensation in this region is supplied by the great auricular nerve, which is derived from the C2 and C3 roots). The neurosurgical literature cites examples of otalgia that were relieved by section of the nervus intermedius (sensory part of VII) or of nerves IX and X. In otalgic cases, one is also prompted to search for a nasopharyngeal tumor, vertebral artery aneurysm or dissection or to anticipate an outbreak of zoster as mentioned. Formerly, lateral sinus thrombosis was a common cause in children. When these possibilities


Neurology_Adams

Neurology_Adams. tumor, vertebral artery aneurysm or dissection or to anticipate an outbreak of zoster as mentioned. Formerly, lateral sinus thrombosis was a common cause in children. When these possibilities are eliminated by appropriate studies, there always remain examples of primary idiopathic otalgia, lower cluster headache, and glossopharyngeal neuralgia. Some patients with migraine have pain centered in the ear region and occiput, but we have never observed a trigeminal neuralgia in which the ear was the predominant site of pain. Occasionally, temporomandibular joint disease is the cause (see below).


Neurology_Adams

Neurology_Adams. Paroxysmal pain may occur in the distribution of the greater or lesser occipital nerves (suboccipital, occipital, and posterior parietal areas). While tenderness may be localized to the region where these nerves cross the superior nuchal line, there is only questionable evidence of an occipital nerve lesion at this site. The finding of hypesthesia in the distribution of the occipital nerves makes the possibility of an entrapment neuropathy more convincing. Carbamazepine or gabapentin may provide some relief. Blocking the nerves with lidocaine may abolish the pain and encourage attempts to section one or more occipital nerves or the second or third cervical dorsal root, but the results of the sectioning procedure have had variable success, and several such patients who had these procedures were later referred to us with disabling anesthesia dolorosa. Experts advise repeated injections of local anesthetic agents and the use of steroids, botulinum toxin, and analgesic and


Neurology_Adams

Neurology_Adams. procedures were later referred to us with disabling anesthesia dolorosa. Experts advise repeated injections of local anesthetic agents and the use of steroids, botulinum toxin, and analgesic and anti-inflammatory drugs. The pain at times may be difficult to distinguish from that arising in the upper three cervical facet joints, one type of which is discussed below. The approach of treating migraine by injection of the occipital nerves, mentioned earlier, is controversial.


Neurology_Adams

Neurology_Adams. This condition, a unilateral occipital and suboccipital ache, may be a prominent symptom in patients with neck pain, particularly after neck injuries (a prevalence of 27 percent, according to Lord et al). Bogduk and Marsland attribute it to a degenerative or traumatic arthropathy involving the C2 and C3 facet joints with impingement on the “third occipital nerve” (a branch of the C3 dorsal ramus that crosses the dorsolateral aspect of the facet joint. Elimination of the neck pain and headache by percutaneous blocking of the third occipital nerve near the facet joint under fluoroscopic control is diagnostic and temporarily therapeutic. More sustained relief (weeks to months) has been obtained by radiofrequency coagulation of the nerve or steroid injections in and around the joint. NSAIDs also may provide some relief.


Neurology_Adams

Neurology_Adams. Carotidynia was coined by Temple Fay in 1927 to designate a special type of cervicofacial pain that could be elicited by pressure on the common carotid arteries of patients with atypical facial neuralgia. Compression of the artery in the neck in these patients, or mild electrical stimulation at or near the bifurcation, produced a dull ache that was referred to the ipsilateral face, ear, jaws, and teeth or down the neck. This type of carotid sensitivity occurs as part of cranial (giant cell) arteritis and of the rare condition known as Takayasu arteritis (Chap. 33), and during attacks of migraine or cluster headache. It has also been described with displacement of the carotid artery by tumor and dissecting aneurysm of its wall; among these causes, the last is of greatest concern. The idiopathic variety of carotidynia may have to do with a swelling or inflammation of the tissue surrounding the carotid bifurcation, a change that has been demonstrated on MRI by Burton and colleagues, but


Neurology_Adams

Neurology_Adams. variety of carotidynia may have to do with a swelling or inflammation of the tissue surrounding the carotid bifurcation, a change that has been demonstrated on MRI by Burton and colleagues, but the problem has been seen most frequently in migraineurs.


Neurology_Adams

Neurology_Adams. Roseman has described a variant of carotidynia that has a predilection for young adults. This syndrome takes the form of recurrent, self-limited attacks of pain and tenderness at the carotid bifurcation lasting a week or two. Dissection of the carotid artery, as discussed below, is always a concern. During the attack, aggravation of the pain by head movement, chewing, and swallowing is characteristic. This condition is treated with simple analgesics. Yet another possible variety of carotidynia appears at any stage of adult life and recurs in attacks lasting minutes to hours in association with throbbing headaches indistinguishable from common migraine (Raskin and Prusiner). This form responds favorably to the administration of ergotamine and other drugs that are effective in the treatment of migraine.


Neurology_Adams

Neurology_Adams. Although most pain of carotid or vertebral artery dissection is localized to the site of injury in the anterior or posterior neck, Arnold and colleagues have emphasized the frequency with which ipsilateral headache, and not neck pain, was the sole feature. Some had a paroxysmal (“thunderclap”) onset but most had throbbing and progressive pain over days, sometimes bilaterally. The combination of focal neck pain and localized headache over an eye is particularly suggestive of carotid dissection and, of course, if there are corresponding symptoms of fluctuating or static regional brain ischemia, Horner syndrome, or lower cranial nerve palsies, the diagnosis is likely.


Neurology_Adams

Neurology_Adams. This is a form of craniofacial pain from dysfunction of one temporomandibular joint. Malocclusion because of ill-fitting dentures or loss of molar teeth on one side with alteration of the normal bite may lead to distortion of and ultimately degenerative changes in the joint and to pain in front of the ear, with radiation to the temple and over the face (see Guralnick et al). Most patients, according to Scrivani and colleagues report deviation of the mandible to the affected side on jaw opening and clicking noises emanating from the joint. Locking of the jaw in either the open or closed position is another feature. The diagnosis is supported by the findings of tenderness over the joint, crepitus on opening the mouth, and limitation of jaw opening. The favored diagnostic maneuver involves palpating the joint from its posterior aspect by placing a finger in the external auditory meatus and pressing forward. The diagnosis can be made with some confidence only if this entirely reproduces


Neurology_Adams

Neurology_Adams. palpating the joint from its posterior aspect by placing a finger in the external auditory meatus and pressing forward. The diagnosis can be made with some confidence only if this entirely reproduces the patient’s pain. CT and plain films are rarely helpful, but effusions have been shown in the joints by MRI. Management consists of careful adjustment of the bite by a dental specialist. Small doses of amitriptyline at bedtime may be helpful. In our experience, most of the putative diagnoses of Costen syndrome that reach the neurologist have been uncertain, and the number of headaches and facial pains that are attributed to “temporomandibular joint dysfunction” is probably excessive, especially if judged by the response to treatment. The temporomandibular joint may also be the source of pain when involved with rheumatoid arthritis and other connective tissue diseases.


Neurology_Adams

Neurology_Adams. Facial Pain of Dental or Sinus Origin Maxillary and mandibular discomfort is a common effect of nerve irritation from deep caries, abscess, dental pulp degeneration, or periodontal disease. The pain of dental nerve origin is usually most severe at night, slightly pulsating, and often associated with local tenderness at the root of the tooth in response to heat, cold, or pressure. The diagnosis can be confirmed by infiltrating the base of the tooth with lidocaine, and the pain is eradicated by proper dental management. Trigeminal neuritis following dental extractions or oral surgery is another vexing problem. There may be sensory loss in the tongue or lower lip and weakness of the masseter or pterygoid muscle.


Neurology_Adams

Neurology_Adams. Trigeminal neuritis following dental extractions or oral surgery is another vexing problem. There may be sensory loss in the tongue or lower lip and weakness of the masseter or pterygoid muscle. Sometimes the onset of “atypical facial pain” (see below) can be dated to a dental procedure such as tooth extraction, and, as usually happens, neither the dentist nor the neurologist is able to find a source for the pain or any malfunction of the trigeminal nerve. Roberts and coworkers, as well as Ratner and associates, have pointed out that residual microabscesses and subacute bone infection account for some of these cases. They isolated the affected region by using local anesthetic blocks, curetted the bone, and administered antibiotics, following which the pain resolved. The removed bone fragments showed vascular and inflammatory changes and infection with oral bacterial flora, but there was no control material. Facial Pain of Uncertain Origin (Idiopathic, “Atypical” Facial Pain)


Neurology_Adams

Neurology_Adams. Facial Pain of Uncertain Origin (Idiopathic, “Atypical” Facial Pain) There remains, after all the aforementioned facial pain syndromes, a fair number of patients with pain in the face for which no cause can be found. These patients are most often young women, who describe the pain as constant and unbearably severe, deep in the face, or at the angle of cheek and nose, and unresponsive to all varieties of analgesic medication. Because of the failure to identify an organic basis for the pain, one is tempted to attribute it to psychologic or emotional factors. Depression of varying severity is found in some. Some such patients, with or without depression, respond to tricyclic antidepressants and selective serotonin reuptake inhibitors (SSRI) medications. Differentiated from this group is the condition of trigeminal neuropathy with facial numbness, described in Chap. 44.


Neurology_Adams

Neurology_Adams. Facial pain of the “atypical type,” like other chronic pain of indeterminate cause, requires close observation of the patient, looking for lesions such as nasopharyngeal carcinoma or apical lung carcinoma to become apparent. The pain can be managed by the conservative methods outlined in the preceding chapter and not by destructive surgery. Antidepressants may be helpful, especially if the patient displays obsessive characteristics in relation to the pain; some European neurologists favor clomipramine for various facial and scalp pains. Other Rare Types of Facial Pain


Neurology_Adams

Neurology_Adams. Other Rare Types of Facial Pain Neuralgia may arise in the terminal branches of the trigeminal, ciliary, nasociliary, and supraorbital nerves; some of which have already been mentioned. Some of these are vague entities at best and merely descriptive terms given to pains localized around the eye and nose. The Tolosa-Hunt syndrome of pain behind the eye and granulomatous involvement of some combination of cranial nerves III, IV, VI, and ophthalmic V, responsive to steroids, is discussed in Chap. 44. A kind of reflex sympathetic dystrophy of the face is postulated as another rare form of persistent facial pain that may follow dental surgery or penetrating injuries to the face. It is characterized by severe burning pain and hyperpathia in response to all types of stimuli. Sudomotor, vasomotor, and trophic changes are lacking, unlike causalgia that affects the limbs. Nevertheless, this form of facial pain is said to respond to repeated blockade or resection of the stellate ganglion.


Neurology_Adams

Neurology_Adams. Under the title of neck–tongue syndrome, Lance and Anthony have described the occurrence of a sharp pain and tingling in the upper neck or occiput with numbness of the ipsilateral half of the tongue on sudden rotation of the neck. They attribute the syndrome to stretching of the C2 ventral ramus, which contains proprioceptive fibers from the tongue; these fibers run from the lingual nerve to the hypoglossal nerve and thence to the second cervical root. A problem that has gone by the self-evident name burning mouth syndrome (stomatodynia) occurs mainly in middle-aged and older women, as commented in


Neurology_Adams

Neurology_Adams. A problem that has gone by the self-evident name burning mouth syndrome (stomatodynia) occurs mainly in middle-aged and older women, as commented in Chap. 11. The tongue or other oral sites may be most affected or the entire oral mucosa may burn. A few patients are found to have diabetes, vitamin B12 deficiency, or Sjögren syndrome as possible causes. A hint to the last diagnosis is the inability to feel food in the mouth. The oral mucosa is normal when inspected, and no one treatment has been consistently effective, but gabapentin combined with antidepressants or clonazepam may be tried (see the review by Grushka et al). One of our patients with a limited form of this condition, which affected only the upper palate and gums, benefited from dental nerve blocks with lidocaine. Anderson CD, Frank RD: Migraine and tension headache: is there a physiological difference? Headache 21:63, 1981.


Neurology_Adams

Neurology_Adams. Anderson CD, Frank RD: Migraine and tension headache: is there a physiological difference? Headache 21:63, 1981. Arnold M, Cumurcivc R, Stapf C, et al: Pain as the only symptom of cervical artery dissection. J Neurol Neurosurg Psychiatry 77:1021, 2006. Ashina M, Lassin LH, Bendsten L, et al: Effect of inhibition of nitric oxide synthetase on chronic tension type headache: a randomised crossover trial. Lancet 353:287, 1999. Barker FG, Jannetta PJ, Bissonette DJ, et al: The long-term outcome of microvascular decompression for trigeminal neuralgia. N Engl J Med 334:1077, 1996. Bartleson JD, Swanson JW, Whisnant JP: A migrainous syndrome with cerebrospinal fluid pleocytosis. Neurology 31:1257, 1982. Bartsch T, Pirsker MO, Rasche D, et al: Hypothalamic deep brain stimulation for cluster headache: experience from a new multi-centre series. Cephalalgia 28:285, 2008. Basser LS: Benign paroxysmal vertigo in childhood. Brain 87:141, 1964.


Neurology_Adams

Neurology_Adams. Basser LS: Benign paroxysmal vertigo in childhood. Brain 87:141, 1964. Bates D, Ashford E, Dawson R, et al: Subcutaneous sumatriptan during the migraine aura: Sumatriptan Aura Study Group. Neurology 44:1587, 1994. Berg MJ, Williams LS: The transient syndrome of headache with neurologic deficits and CSF lymphocytosis. Neurology 45:1648–1654, 1995. Bickerstaff ER: Basilar artery migraine. Lancet 1:15, 1961. Bigal, ME, Kurth T, Santanello N, et al: Migraine and cardiovascular disease: a population-based study. Neurology 74:628–735, 2010. Blau JN, Dexter SL: The site of pain origin during migraine attacks. Cephalalgia 1:143, 1981. Bogduk N, Govind J: Cervicogenic headache: an assessment of the evidence on clinical diagnosis, invasive tests, and treatment. Lancet Neuro 8:959, 2009. Bogduk N, Marsland A: On the concept of third occipital headache. J Neurol Neurosurg Psychiatry 49:775, 1986.


Neurology_Adams

Neurology_Adams. Bogduk N, Marsland A: On the concept of third occipital headache. J Neurol Neurosurg Psychiatry 49:775, 1986. Bowsher D: The effects of pre-emptive treatment of postherpetic neuralgia with amitriptyline: a randomized, double-blind, placebo-controlled trial. J Pain Symptom Manage 13:327, 1997. Broderick JP, Swanson JW: Migraine-related strokes. Arch Neurol 44:868, 1987. Burton BS, Syms MJ, Peterman GW, Burgess LP: MR imaging of patients with carotidynia. AJNR Am J Neuroradiol 21:766, 2000. Caplan LR: Migraine and vertebrobasilar ischemia. Neurology 41:55, 1991. Chen SP, Fuh JL, Lirng JF, et al: Recurrent thunderclap headache and benign CNS angiopathy. Neurology 67:2164, 2006. Cutrer FM: Pain-sensitive cranial structures: chemical anatomy. In: Silberstein SD, Lipton RD, Dalessio DJ (eds): Wolff’s Headache and Other Head Pain, 7th ed. Oxford, UK, Oxford University Press, 2001, pp 50–56.


Neurology_Adams

Neurology_Adams. Cutrer FM, Sorensen AG, Weisskoff RM, et al: Perfusion-weighted imaging defects during spontaneous migraine aura. Ann Neurol 43:25, 1998. Dandy WE: Concerning the cause of trigeminal neuralgia. Am J Surg 24:447, 1934. Day JW, Raskin NH: Thunderclap headache symptomatic of unruptured cerebral aneurysm. Lancet 2:1247, 1986. Dodick DW: Thunderclap headache. J Neurol Neurosurg Psychiatry 72:6, 2002. Drummond PD, Lance JW: Contribution of the extracranial circulation to the pathophysiology of headache. In: Olesen J, Edvinsson L (eds): Basic Mechanisms of Headache. Amsterdam, Elsevier, 1988, pp 321–330. Ducros A, Denier C, Joutel A, et al: The clinical spectrum of familial hemiplegic hemianopic migraine associated with mutations in a neuronal calcium channel. N Engl J Med 345:17, 2001. Ekbom K: cited by Kudrow L (see below). Ekbom K, Greitz T: Carotid angiography in cluster headache. Acta Radiol Diagn (Stockh) 10:177, 1970.


Neurology_Adams

Neurology_Adams. Ekbom K: cited by Kudrow L (see below). Ekbom K, Greitz T: Carotid angiography in cluster headache. Acta Radiol Diagn (Stockh) 10:177, 1970. Favier I, van Vilet JA, Roon KI, et al: Trigeminal autonomic cephalgias due to structural lesions. Arch Neurol 64:25, 2007. Ferrari MD, Haan J, Blokland JAK, et al: Cerebral blood flow during migraine attacks without aura and effect of sumatriptan. Arch Neurol 52:135, 1995. Ferrari MD, Roon KI, Lipton RB, et al: Oral triptans (serotonin 5-HT1B/1D agonists) in acute migraine treatment: a meta-analysis of 53 trials. Lancet 358:1668, 2001. Fields HL: Treatment of trigeminal neuralgia. N Engl J Med 334: 1125, 1996. Fisher CM: Late-life migraine accompaniments—further experience. Stroke 17:1033, 1986. Fox AW, Chambers CD, Anderson PO, et al: Evidence-based assessment of pregnancy outcome after sumatriptan exposure. Headache 42:8, 2002.


Neurology_Adams

Neurology_Adams. Fox AW, Chambers CD, Anderson PO, et al: Evidence-based assessment of pregnancy outcome after sumatriptan exposure. Headache 42:8, 2002. Friedman BW, Greenwald P, Bania TC, et al: Randomized trial of IV dexamethasone for acute migraine in the emergency department. Neurology 69:2038, 2007. Gardner WJ, Stowell A, Dutlinger R: Resection of the greater superficial petrosal nerve in the treatment of unilateral headache. J Neurosurg 4:105, 1947. Garg P, Servoss SJ, Wu JC, et al: Lack of association between migraine headache and patent foramen ovale: results of a case-control study. Circulation 121:1406-1412, 2010. Goadsby PJ: Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurol 1:37, 2002. Goadsby PJ: Recent advances in understanding migraine mechanisms, molecules and therapeutics. Trends Mol Med 13:39, 2007.


Neurology_Adams

Neurology_Adams. Goadsby PJ: Recent advances in understanding migraine mechanisms, molecules and therapeutics. Trends Mol Med 13:39, 2007. Gomez-Aranda F, Cañadillas F, Marti-Masso JF, et al: Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis: a report of 50 cases. Brain 120:1105, 1997. Graham JR: Migraine. Clinical aspects. In: Vinkin PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Vol. 5, Headaches and Cranial Neuralgias. Amsterdam, North-Holland Publishing Company, 1968, pp 45–58. Graham JR, Wolff HG: Mechanism of migraine headache and action of ergotamine tartrate. Arch Neurol Psychiatry 39:737, 1938. Grushka M, Epstein JB, Gorski M: Burning mouth syndrome. Am Fam Physician 65:615, 2002. Gudmundsson LS, Scher AI, Aspelund T, et al: Migraine with aura and risk of cardiovascular and all cause mortality in men and women: prospective cohort study. BMJ 341:c3966, 2010.


Neurology_Adams

Neurology_Adams. Gudmundsson LS, Scher AI, Aspelund T, et al: Migraine with aura and risk of cardiovascular and all cause mortality in men and women: prospective cohort study. BMJ 341:c3966, 2010. Guralnick W, Kaban LB, Merrill RG: Temporomandibular-joint afflictions. N Engl J Med 299:123, 1978. Harris N: Paroxysmal and postural headaches from intra- ventricular cysts and tumours. Lancet 2:654, 1944. Ho TW, Connor KM, Zhang Y, et al: Randomized controlled trial of the CGRP receptor antagonist telcagepant for migraine prevention. Neurology 83:958, 2014. Hunt JR: The sensory field of the facial nerve: a further contribution to the symptomatology of the geniculate ganglion. Brain 38:418, 1915. Iversen HK, Nielsen TH, Olesen J: Arterial responses during migraine headache. Lancet 336:837, 1990. Jannetta PJ: Structural mechanisms of trigeminal neuralgia: Arterial compression of the trigeminal nerve at the pons in patients with trigeminal neuralgia. J Neurosurg 26:159, 1967.


Neurology_Adams

Neurology_Adams. Jannetta PJ: Structural mechanisms of trigeminal neuralgia: Arterial compression of the trigeminal nerve at the pons in patients with trigeminal neuralgia. J Neurosurg 26:159, 1967. Jarrar RG, Black DF, Dodick DW, et al: Outcome of trigeminal nerve section in the treatment of chronic cluster headache. Neurology 60:1360, 2003. King RB: Topical aspirin in chloroform and the relief of pain due to herpes zoster and postherpetic neuralgia. Arch Neurol 50:1046, 1993. Kittrelle JP, Grouse DS, Seybold ME: Cluster headache. Arch Neurol 42:496, 1985. Klapper J, Mathew N, Nett R: Triptans in the treatment of basilar migraine and migraine with prolonged aura. Headache 41:981, 2001. Kruit MC, van Buchem, MA, Hofman PAM, et al: Migraine as a risk factor for subclinical brain lesions. JAMA 291:427, 2004.


Neurology_Adams

Neurology_Adams. Kruit MC, van Buchem, MA, Hofman PAM, et al: Migraine as a risk factor for subclinical brain lesions. JAMA 291:427, 2004. Kruit MC, van Buchem, MA, Launer LJ, et al: Migraine is associated with an increased risk of deep white matter lesions, subclinical posterior circulation infarcts and brain iron accumulation: the population-based MRI CAMERA study. Cephalalgia 30(2): 129, 2010. Kudrow L: Cluster Headache: Mechanisms and Management. Oxford, UK, Oxford University Press, 1980. Kunkle EC: Clues in the tempos of cluster headache. Headache 22:158, 1982. Kunkle EC, Pfeiffer JB Jr, Wilhoit WM, Lamrick LW Jr: Recurrent brief headaches in cluster pattern. N C Med J 15:510, 1954. Kurth T, Mohamed S, Maillard P, et al: Headache, migraine, and structural brain lesions and function: population based epidemiology of vascular ageing-MRI study. BMJ 342:7357, 2011. Lance JW: Headaches related to sexual activity. J Neurol Neurosurg Psychiatry 39:1226, 1976.


Neurology_Adams

Neurology_Adams. Lance JW: Headaches related to sexual activity. J Neurol Neurosurg Psychiatry 39:1226, 1976. Lance JW, Anthony M: Neck-tongue syndrome on sudden turning of the head. J Neurol Neurosurg Psychiatry 43:97, 1980. Lance JW, Curran DA: Treatment of chronic tension headache. Lancet 1:1236, 1964. Lance JW, Goadsby PJ: Mechanism and Management of Headache, 7th ed. Philadelphia, Elsevier, 2005. Lance JW, Hinterberger H: Symptoms of pheochromocytoma, with particular reference to headache, correlated with catecholamine production. Arch Neurol 33:281, 1976. Lashley KS: Pattern of cerebral integration indicated by the scotomas of migraine. Arch Neurol Psychiatry 46:331, 1941. Lauritzen M, Olesen J: Regional cerebral blood flow during migraine attacks by xenon 133 inhalation and emission tomography. Brain 107:447, 1984. Leão AAP: Spreading depression of activity in cerebral cortex. J Neurophysiol 7:359, 1944.


Neurology_Adams

Neurology_Adams. Leão AAP: Spreading depression of activity in cerebral cortex. J Neurophysiol 7:359, 1944. Leone M, Franzini A, Broggi G, et al: Hypothalamic stimulation for intractable cluster headache. Neurology 67:150, 2006. Li D, Rozen TD: The clinical characteristics of new daily persistent headache. Cephalalgia 22:66, 2002. Lipton RB, Bigal ME, Diamond M, et al: Migraine prevalence, disease burden, and the need for preventive therapy. Neurology 68:343, 2007. Loder E: Triptan therapy in migraine. New Engl J Med 363:63, 2010. Lord SM, Barnsley L, Wallis BJ, Bogduk N: Third occipital nerve headache: a prevalence study. J Neurol Neurosurg Psychiatry 57:1187, 1994. Malleson A: Whiplash and Other Useful Illnesses. Montreal, McGill-Queen’s University Press, 2002. May A, Bahra A, Büchel C, et al: Hypothalamic activation in cluster headache attacks. Lancet 352:275, 1998.


Neurology_Adams

Neurology_Adams. May A, Bahra A, Büchel C, et al: Hypothalamic activation in cluster headache attacks. Lancet 352:275, 1998. Meschia JF, Malkoff MD, Biller J: Reversible segmental cerebral artery spasm and cerebral infarction: possible association with excessive use of sumatriptan and Midrin. Arch Neurol 55:712, 1998. Monteith TS, Gardener H, Rundek T, et al: Migraine and risk of stroke in older adults. Northern Manhattan Study. Neurology 85:715, 2015. Moskowitz MA: Neurogenic inflammation in the pathophysiology and treatment of migraine. Neurology 43(Suppl 3):S16, 1993. Olesen J: Headache Classification Committee of the International Headache Society: classification and diagnostic criteria for headache disorders, cranial neuralgia, and facial pain. Cephalalgia 8(Suppl 7):1, 1988. Olesen J: The ischemic hypothesis of migraine. Arch Neurol 44:321, 1987.


Neurology_Adams

Neurology_Adams. Olesen J: The ischemic hypothesis of migraine. Arch Neurol 44:321, 1987. Olesen J, Diener H-C, Husstedt IW, et al: Calcitonin gene-related peptide receptor antagonist BIBN 4096 BS for the acute treatment of migraine. N Engl J Med 350:1104, 2004. Olsen TS, Friberg L, Lassen NA: Ischemia may be the primary cause of the neurologic defects in classic migraine. Arch Neurol 44:156, 1987. Pannullo SC, Reich JB, Krol G, et al: MRI changes in intracranial hypotension. Neurology 43:919, 1993. Pascual J, Berciano J: Experience with headaches that start in elderly people. J Neurol Neurosurg Psychiatry 57:1255, 1994. Pascual J, Oterino A, Berciano J: Headache in type 1 Chiari malformation. Neurology 42:1519, 1992. Patten J: Neurological Differential Diagnosis. London, Harold Starke, 1977. Price RW, Posner JB: Chronic paroxysmal hemicrania: a disabling headache syndrome responding to indomethacin. Ann Neurol 3:183, 1978.


Neurology_Adams

Neurology_Adams. Price RW, Posner JB: Chronic paroxysmal hemicrania: a disabling headache syndrome responding to indomethacin. Ann Neurol 3:183, 1978. Raftery H: The management of postherpetic pain using sodium valproate and amitriptyline. Ir Med J 72:399, 1979. Rascol A, Cambier J, Guiraud B, et al: Accidents ischemiques cerebraux au cours de crises migraineuses. Rev Neurol 135:867, 1980. Raskin NH: Repetitive intravenous dihydroergotamine as therapy for intractable migraine. Neurology 36:995, 1986. Raskin NH: The hypnic headache syndrome. Headache 28:534, 1988. Raskin NH: Serotonin receptors and headache. N Engl J Med 325:353, 1991. Raskin NH, Prusiner S: Carotidynia. Neurology 27:43, 1977. Ratner EJ, Person P, Kleinman JD, et al: Jawbone cavities and trigeminal and atypical facial neuralgias. Oral Surg Oral Med Oral Pathol 48:3, 1979. Ray BS, Wolff HG: Experimental studies on headache: pain sensitive structures of the head and their significance in headache. Arch Surg 41:813, 1940.


Neurology_Adams

Neurology_Adams. Ray BS, Wolff HG: Experimental studies on headache: pain sensitive structures of the head and their significance in headache. Arch Surg 41:813, 1940. Robbins MS, Farmakidis C, Dayal AK, Lipton RB: Acute headache diagnosis in pregnant women: a hospital-based study. Neurology 85:1024, 2015. Roberts AM, Person P, Chandra NB, Hori JM: Further observations on dental parameters of trigeminal and atypical facial pain. Oral Surg Oral Med Oral Pathol 58:121, 1984. Rooke ED: Benign exertional headache. Med Clin North Am 52:801, 1968. Roseman DM: Carotidynia. Arch Otolaryngol 85:103, 1967. Rundek T, Elkind MSV, DiTullio MR, et al: Patent foramen ovale and migraine: a cross-sectional study from the Northern Manhattan study (NOMAS). Circulation 118:1419–1424, 2008. Sakai F, Ebihara S, Akiyama M, Horikawa M: Pericranial muscle hardness in tension-type headache: a non-invasive measurement method and its clinical application. Brain 118:523, 1995.


Neurology_Adams

Neurology_Adams. Sakai F, Ebihara S, Akiyama M, Horikawa M: Pericranial muscle hardness in tension-type headache: a non-invasive measurement method and its clinical application. Brain 118:523, 1995. Scher AI, Gudmundsson LS, Sigurdsson S, et al: Migraine headache in middle age and late-life brain infarcts. JAMA 301(24):2563, 2009. Schon F, Blau JN: Post-epileptic headache and migraine. J Neurol Neurosurg Psychiatry 50:1148, 1987. Schüks M, Rist PM, Bigal ME, et al: Migraine and cardiovascular disease: systematic review and meta-analysis. BMJ 339:b3914, 2009. Scrivani SJ, Keith DA, Kaban LB: Temporomandibular disorders. New Engl J Med 359:2693, 2008. Singhal AB, Caviness VS, Begleiter MD, et al: Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology 58:130, 2002. Sjaastad O, Dale I: A new (?) clinical headache entity “chronic paroxysmal hemicrania.” Acta Neurol Scand 54:140, 1976.


Neurology_Adams

Neurology_Adams. Sjaastad O, Dale I: A new (?) clinical headache entity “chronic paroxysmal hemicrania.” Acta Neurol Scand 54:140, 1976. Spector JT, Kahn SR, Jones MR, et al: Migraine headache and ischemic stroke risk: an updated meta-analysis. Am J Med 123:612, 2010. Somerville BW: The role of estradiol withdrawal in the etiology of menstrual migraine. Neurology 22:355, 1972. Stewart WF, Lipton RB, Liberman J: Variation in migraine prevalence by race. Neurology 47:52, 1996. Subcutaneous Sumatriptan International Study Group: Treatment of migraine attacks with sumatriptan. N Engl J Med 325:316, 1991. Sweet WH: The treatment of trigeminal neuralgia (tic douloureux). N Engl J Med 315:174, 1986. Sweet WH, Wepsic JG: Controlled thermocoagulation of trigeminal ganglion and rootlets for differential destruction of pain fibers. J Neurosurg 40:143, 1974. Symonds CP: Cough headache. Brain 79:557, 1956.


Neurology_Adams

Neurology_Adams. Symonds CP: Cough headache. Brain 79:557, 1956. Watson P, Steele JC: Paroxysmal dysequilibrium in the migraine syndrome of childhood. Arch Otolaryngol 99:177, 1974. Weatherall MW, Telzerow AJ, Cittadini E, et al: Intravenous aspirin (lysine acetylsalicylate) in the inpatient management of headache. Neurology 75:1098, 2010. White JC, Sweet WH: Pain and the Neurosurgeon. Springfield, IL, Charles C Thomas, 1969, p 265. Wijdicks EF, Kerkhoff H, Van Gijn J: Long-term follow up of 71 patients with thunderclap headache mimicking subarachnoid hemorrhage. Lancet 2:68, 1988. Wolf ME, Szabo K, Griebe M, et al: Clinical and MRI characteristics of acute migrainous infarction. Neurology 76:1191, 2011. Wolff HG: Headache and Other Head Pain, 2nd ed. New York, Oxford University Press, 1963. Woods RP, Iacoboni M, Mazziotta JC: Bilateral spreading cerebral hypoperfusion during spontaneous migraine headache. N Engl J Med 331:1690, 1994.


Neurology_Adams

Neurology_Adams. Woods RP, Iacoboni M, Mazziotta JC: Bilateral spreading cerebral hypoperfusion during spontaneous migraine headache. N Engl J Med 331:1690, 1994. Yanguela J, Sanchez-del-Rio M, Bueno A, et al: Primary trochlear headache. A new cephalgia generated and modulated on the trochlear region. Neurology 62:1134, 2004. Ziegler DK, Hurwitz A, Hassanein RS, et al: Migraine prophylaxis: a comparison of propranolol and amitriptyline. Arch Neurol 44:48, 1987. Figure 9-1. Drawings by K.S. Lashley of his own expanding scotoma with fortification spectra at the edges. “X” indicates point of fixation. The visual aberration expands over minutes (indicated by numbers) and slowly moves peripherally. (Reproduced with permission from Lashley KS: Archives of Neurology 46:331, 1941.) Pain in the Back, Neck, and Extremities


Neurology_Adams

Neurology_Adams. Pain in the Back, Neck, and Extremities We include an extensive chapter on this subject in recognition of the fact that back pain is among the most frequent of medical complaints. Up to 80 percent of adults have low back pain at some time in their lives and, according to Kelsey and White, an even larger percentage will be found at autopsy to have degenerative disc disease. The diagnosis of pain in these parts of the body often requires the assistance of a neurologist. One task is to determine whether a disease of the spine has compressed the spinal cord or the spinal roots. To do this effectively, a clear understanding of the structures involved and some knowledge of orthopedics and rheumatology is necessary.


Neurology_Adams

Neurology_Adams. The parts of the back that possess the greatest freedom of movement, and hence are most frequently subject to injury, are the lumbar, lumbosacral, and cervical. In addition to bending, twisting, and other voluntary movements, many actions of the spine are reflexive in nature and are the basis of erect posture. As pains in the lower part of the spine and legs are caused by different types of disease than those in the neck, shoulder, and arms, they are considered separately in this chapter.


Neurology_Adams

Neurology_Adams. The bony spine is a complex structure, roughly divisible into an anterior and a posterior part. The anterior component consists of cylindric vertebral bodies, articulated by the intervertebral discs and held together by the anterior and posterior longitudinal ligaments. The posterior elements are more delicate and extend from the bodies as pedicles and laminae, which form the spinal canal by joining with the posterior aspects of the vertebral bodies and ligaments. Large transverse and spinous processes project laterally and posteriorly, respectively, and serve as the origins and insertions of the muscles that support and protect the spinal column. The bony processes are also held together by sturdy ligaments, the most important being the ligamentum flavum, which runs along the ventral surfaces of the posterior elements and is applied to the inner surface of the laminae. The posterior longitudinal ligament lies opposite it on the dorsal surfaces of the vertebral bodies. These two


Neurology_Adams

Neurology_Adams. surfaces of the posterior elements and is applied to the inner surface of the laminae. The posterior longitudinal ligament lies opposite it on the dorsal surfaces of the vertebral bodies. These two ligaments bound the posterior and anterior structures of the spinal canal, respectively.


Neurology_Adams

Neurology_Adams. The posterior parts of the vertebrae articulate with one another at the diarthrodial facet joints (also called apophyseal or zygapophyseal joints), each of which is composed of the inferior facet of the vertebra above and the superior facet of the one below. The facet and sacroiliac joints, which are covered by synovia, the compressible intervertebral discs, and the collagenous and elastic ligaments, permit a limited degree of flexion, extension, rotation, and lateral motion of the spine. Figure 10-1 illustrates these anatomic features in representative sections of the lumbar spine. The size and shape of thoracic and cervical vertebrae and their posterior elements differ but the essential relationships between adjacent vertebral bodies are similar.


Neurology_Adams

Neurology_Adams. Although the ligamentous structures are quite strong, neither they nor the vertebral body–disc complexes have sufficient integral strength to resist some of the enormous forces that may act on the spinal column. Consequently, the stability of the lower back is also dependent on the voluntary and reflex activity of the sacrospinalis, abdominal, gluteus maximus, and hamstring muscles, and on the integrity of the ligamentum flavum and posterior longitudinal ligament.


Neurology_Adams

Neurology_Adams. The vertebral and paravertebral structures derive their innervation from the meningeal branches of the spinal nerves (also known as recurrent meningeal or sinuvertebral nerves). These meningeal branches spring from the posterior divisions of the spinal nerves just distal to the dorsal root ganglia, reenter the spinal canal through the intervertebral foramina, and supply pain fibers to the intraspinal ligaments, periosteum of bone, outer layers of the annulus fibrosus (which enclose the disc), and capsule of the articular facets. Coppes and associates have found A-δ and C pain fibers extending into the inner layers of the annulus, and even into the nucleus pulposus. Although the spinal cord itself is insensitive, many of the conditions that affect it produce pain by involving these adjacent structures. For example, the sensory fibers from the lumbosacral and sacroiliac joints enter the spinal cord via the fifth lumbar and first sacral roots. Motor fibers exit through the corresponding


Neurology_Adams

Neurology_Adams. structures. For example, the sensory fibers from the lumbosacral and sacroiliac joints enter the spinal cord via the fifth lumbar and first sacral roots. Motor fibers exit through the corresponding anterior roots and form the efferent limb of segmental reflexes. This explains some of the patterns of pain referral from these joints.


Neurology_Adams

Neurology_Adams. The spinal roots in the lumbar region (cauda equina) angulate sharply to exit horizontally through the intervertebral foramina. Prior to entering the short foraminal canal, the lumbar spinal root lies in a shallow furrow along the inner surface of the pedicle, the lateral recess. This is a common site of root entrapment by disc fragments and bony overgrowth. Because the thoracic and cervical discs do not have to travel downward and laterally to their points of exit at the foramina, they exit horizontally from their points of formation in the spinal subarachnoid space.


Neurology_Adams

Neurology_Adams. Degeneration in the intervertebral discs and ligaments is a consequence of aging and the succession of inevitable minor traumas to the spine. Deposition of collagen and elastin and alterations of glycosaminoglycans combine to decrease the water content of the nucleus pulposus; concomitantly, the cartilaginous endplate becomes less vascular (Hassler). The dehydrated disc thins out and becomes more fragile. Similar changes occur in the annulus of the disc, which frays to an increasing degree with the passage of time, permitting the nucleus pulposus to bulge and, sometimes with injury, to extrude. This process can be observed by MRI, which shows a gradual reduction in the high signal of the nucleus pulposus with the passage of time. For example, among women who had MRI for gynecologic reasons, Powell and coworkers found an increasing frequency of lumbar disc degeneration and bulging, approaching 70 percent by the fiftieth year of life.


Neurology_Adams

Neurology_Adams. The problem of degenerative spinal disease has been conceptualized as having its genesis in shrinkage of the disc that subsequently alters the alignment of the articular facets and vertebral bodies, leading to facet arthropathy and bony spur formation across the disc space (a spondylitic “bar”). These reactive changes create stenosis of the spinal canal and compromise the lateral recesses and the intervertebral foramina, where they impinge on lumbar nerve roots and, at higher levels, on the spinal cord. Osteoporosis, especially in older women, is a further important cause of vertebral flattening or collapse, additionally narrowing the spinal canal.


Neurology_Adams

Neurology_Adams. The lower parts of the spine and pelvis, with their massive muscular attachments, are relatively inaccessible to palpation and inspection. Although some physical signs and imaging studies are helpful, diagnosis often depends on the patient’s description of the pain and his behavior during the execution of certain maneuvers. Seasoned clinicians appreciate the need for a systematic inquiry and method of examination. Clinical Features of Low Back Pain Of the symptoms of spinal disease (pain, stiffness, limitation of movement, and deformity), pain is foremost. Four types of pain may be distinguished: local, referred, radicular, and that arising from secondary muscular spasm. The origin of pain can often be discerned from the patient’s description, reliance being placed mainly on the character of the pain, its location, and conditions that modify it.


Neurology_Adams

Neurology_Adams. Local pain is steady and aching, but it may be intermittent and sharp, and, although not well circumscribed, is felt in or near the affected part of the spine. Local pain is caused by any pathologic process that impinges on structures containing sensory endings, including the periosteum of the vertebral body, capsule of apophyseal joints, annulus fibrosus, and ligaments. Destruction of the nucleus pulposus alone produces little or no pain but the annulus is innervated with small nerve fibers and, when disrupted, may produce considerable pain in the immediate region of the affected disc. Such local discomfort often accompanies the moment of disc rupture through the annulus, before the subsequent pain of nerve root compression arises. Pathologic change arising in spinal structures may also evoke discomfort in regions that share common innervation and thereby vaguely simulate the pain of radicular disease. These areas of referred projection may be considered similarly to the referred


Neurology_Adams

Neurology_Adams. evoke discomfort in regions that share common innervation and thereby vaguely simulate the pain of radicular disease. These areas of referred projection may be considered similarly to the referred pain of the “sclerotomes” discussed in Chap. 7 and below.


Neurology_Adams

Neurology_Adams. Referred pain in reference to the spine may be projected from the spine to viscera and other structures lying within the territory of the lumbar and upper sacral dermatomes, or conversely, projected from pelvic and abdominal viscera to the spine. For example, pain caused by disease of the upper part of the lumbar spine may be referred to the medial flank, lateral hip, groin, and anterior thigh. This has been attributed to irritation of the superior cluneal nerves, which are derived from the posterior divisions of the first three lumbar spinal nerves and innervate the superior portions of the buttocks. Pain from the lower part of the lumbar spine is usually referred to the lower buttocks and posterior thighs and is a result of irritation of lower spinal nerves, which activate the same pool of intraspinal neurons as the nerves that innervate the posterior thighs. Pain of this type is usually diffuse and has a deep, aching quality, but it tends to be more superficially projected. In


Neurology_Adams

Neurology_Adams. pool of intraspinal neurons as the nerves that innervate the posterior thighs. Pain of this type is usually diffuse and has a deep, aching quality, but it tends to be more superficially projected. In general, the intensity of referred pain parallels that of local pain and maneuvers that alter local pain have a similar effect on referred pain. McCall and colleagues and Kellgren have verified these areas of reference by the injection of hypertonic saline into the facet joints and the sclerotomes they determined are discussed in Chap. 7; however, as Sinclair and coworkers have pointed out, the sites of reference are inexact and cannot be relied on for precise anatomic localization.


Neurology_Adams

Neurology_Adams. The opposite situation, in which pain from visceral diseases is referred to the lumbar spine, is usually modified by the state of activity of the viscera and sometimes by assuming an upright or supine posture. What is notable in comparison to pain referred from the spine to the viscera is that the character and temporal relationship of the pain have little relationship to movement of the back.


Neurology_Adams

Neurology_Adams. Radicular or “root” pain has some of the characteristics of referred pain but differs in its greater intensity, distal radiation along the course of the nerve containing the affected root, and factors that excite it. The mechanism is stretching, irritation, or compression of a spinal root at any site within or central to the intervertebral foramen. The pain is sharp, often intense, and usually superimposed on the dull ache of referred pain; it nearly always radiates from a paracentral position near the spine to some part of the lower limb. Coughing, sneezing, and straining characteristically evoke this sharp radiating pain, although each of these actions may also jar or move the spine and enhance local pain. Any maneuver that stretches the nerve root—for example, “straight-leg raising” or bending at the waist in cases of sciatica—evokes radicular pain. The patterns of radicular pain are described in the sections on prolapsed discs further on in the chapter, and the distribution of


Neurology_Adams

Neurology_Adams. or bending at the waist in cases of sciatica—evokes radicular pain. The patterns of radicular pain are described in the sections on prolapsed discs further on in the chapter, and the distribution of cutaneous innervation of the spinal roots is shown in Figs. 8-2 and 8-3. The most common pattern is sciatica, pain that originates in the buttock and is projected along the posterior or posterolateral thigh. It results from irritation of the L5 or S1 nerve root as summarized in the review by Ropper and Zafonte. Paresthesia or superficial sensory loss, soreness of the skin, tenderness in certain regions along the nerve and loss of a tendon reflex usually accompany radicular pain. If the anterior roots are involved as well, there is weakness, atrophy, or muscular twitching.


Neurology_Adams

Neurology_Adams. In patients with severe circumferential constriction of the cauda equina due to spondylosis (lumbar spinal stenosis), sensorimotor impairment and referred pain are elicited by standing and walking. The symptoms are projected to the calves and the backs of the thighs thereby simulating the exercise-induced symptoms of iliofemoral vascular insufficiency—hence the term spinal claudication has been applied to the activity-induced symptoms of lumbar stenosis [see “Lumbar Stenosis (Spondylotic Caudal Radiculopathy)” later in this chapter].


Neurology_Adams

Neurology_Adams. Referred pain from structures of the lower back (sometimes called pseudoradicular) does not, as a rule, project below the knees and is not accompanied by neurologic changes other than sometimes a vague numbness without demonstrable sensory impairment. This is in contrast to the pain of root compression. Pain resulting from muscular spasm usually occurs in relation to local spinal irritation and may be thought of as a nocifensive reflex for the protection of the diseased parts against injurious motion. Chronic muscular contraction may give rise to a dull, sometimes cramping ache. One can sometimes feel the tautness of the sacrospinalis and gluteal muscles and demonstrate by palpation that the pain is localized to them. However, except for the most severe degrees of acute injuries of the back, the spasms are difficult to detect and their contribution to back pain has appeared to us to be relatively small.


Neurology_Adams

Neurology_Adams. In addition to assessing the character and location of the pain, one should determine the factors that aggravate and relieve it, its constancy, and its relationship to activity and to rest, posture, forward bending, coughing, sneezing, and straining. Frequently, the most important lead comes from knowledge of the mode of onset and the circumstances that initiated the pain. Inasmuch as many painful conditions of the back are the result of injuries incurred during work or in accidents, the possibility of exaggeration or prolongation of pain for purposes of compensation must be kept in mind. Examination of the Lower Back The main goals of the examination of the back are to differentiate pain that is caused by nerve root compression from musculoskeletal strains, metastatic spinal tumor, and infectious and inflammatory diseases of the spine and hips.


Neurology_Adams

Neurology_Adams. Some information may be gained by inspection of the back, buttocks, and lower limbs in various positions. The normal spine shows a thoracic kyphosis and lumbar lordosis in the sagittal plane, which in some individuals may be exaggerated. In the coronal plane, the spine is normally straight or shows a slight curvature, particularly in women. One should observe the spine for excessive curvature, a list, flattening of the normal lumbar lordosis, presence of a gibbus (a sharp, kyphotic angulation usually indicative of a fracture), pelvic tilt or obliquity (Trendelenburg sign), and asymmetry of the paravertebral or gluteal musculature. A sagging gluteal fold suggests involvement of the S1 root. In sciatica one may observe a flexed posture of the affected leg, presumably to reduce tension on the irritated nerve. Or, patients in whom a free fragment of lumbar disc material has migrated posterolaterally may be unable to lie down and extend the spine.


Neurology_Adams

Neurology_Adams. The next step in the examination is observation of the spine, hips, and legs during certain motions. No advantage accrues from determining how much pain the patient can tolerate. More important is to determine when and under what conditions the pain begins or worsens. Observation of the patient’s gait may disclose a subtle limp, a pelvic tilt, a shortening of step, or a stiffness of bearing—indicative of a disinclination to bear weight on a painful leg. One looks for limitation of motion while the patient is standing, sitting, and reclining. When standing, the motion of forward bending normally produces flattening and reversal of the lumbar lordotic curve and exaggeration of the thoracic curve. With lesions of the lumbosacral region that involve the posterior ligaments, articular facets, or sacrospinalis muscles and with ruptured lumbar discs, protective reflexes prevent flexion, which stretches these structures (“splinting”). As a consequence, the sacrospinalis muscles remain taut


Neurology_Adams

Neurology_Adams. or sacrospinalis muscles and with ruptured lumbar discs, protective reflexes prevent flexion, which stretches these structures (“splinting”). As a consequence, the sacrospinalis muscles remain taut and prevent motion in the lumbar part of the spine. Forward bending then occurs at the hips and at the thoracolumbar junction; also, the patient bends in such a way as to avoid tensing the hamstring muscles and putting undue leverage on the pelvis. In the presence of degenerative disc disease, straightening up from a flexed position is performed with difficulty.


Neurology_Adams

Neurology_Adams. Lateral bending is usually less instructive than forward bending but, in unilateral ligamentous or muscular strain, bending to the opposite side aggravates the pain by stretching the damaged tissues. With unilateral sciatica, the patient lists to one side and strongly resists bending to the opposite side, and the preferred posture in standing is with the leg slightly flexed at the hip and knee. When the herniated disc lies lateral to the nerve root and displaces it medially, tension on the root is reduced and pain is relieved by bending the trunk to the side opposite the lesion; with herniation medial to the root, tension is reduced by inclining the trunk to the side of the lesion. In the sitting position, flexion of the spine can be performed more easily, even to the point of bringing the knees in contact with the chest. The reason for this is that knee flexion relaxes tightened hamstring muscles and relieves the stretch on the sciatic nerve.


Neurology_Adams

Neurology_Adams. Examination with the patient in the reclining position yields much the same information as in the standing and sitting positions. With lumbosacral disc lesions and sciatica, passive lumbar flexion causes little pain and is not limited as long as the hamstrings are relaxed and there is no stretching of the sciatic nerve. Thus, with the knees flexed to 90 degrees, sitting up from the reclining position is unimpeded and not painful; with knees extended, there is pain and limited motion (Kraus-Weber test). With vertebral disease, passive flexion of the hips is free, whereas flexion of the lumbar spine may be impeded and painful.


Neurology_Adams

Neurology_Adams. The most helpful signs in detecting nerve root compression are passive straight-leg raising (possible up to almost 90° in normal individuals) with the patient supine and variations of this test. Raising the straight leg places the sciatic nerve and its roots under tension, thereby producing radicular, radiating pain from the buttock through the posterior thigh if there is compression of these neural structures. This maneuver is the usual way in which compression of the L5 or S1 nerve root is detected (Lasègue sign), however, it may also cause an anterior rotation of the pelvis around a transverse axis, increasing stress on the lumbosacral joint and causing milder radiating pain if this joint is arthritic or otherwise diseased. Straight raising of the opposite leg (“crossed straight-leg raising,” Fajersztajn sign) may also cause pain on the affected side and is a more specific sign of prolapsed disc than is the Lasègue sign but far less sensitive. The many derivatives of the


Neurology_Adams

Neurology_Adams. raising,” Fajersztajn sign) may also cause pain on the affected side and is a more specific sign of prolapsed disc than is the Lasègue sign but far less sensitive. The many derivatives of the straight-leg raising sign are discussed in the section on lumbar disc disease further on and summarized in the review by Ropper and Zafonte. Asking the seated patient to extend the leg so that the sole of the foot can be inspected is a way of checking for a feigned Lasègue sign.


Neurology_Adams

Neurology_Adams. A patient with lumbosacral strain or disc disease (except in the acute phase or if the disc fragment has migrated laterally) can usually extend or hyperextend the spine with little or no aggravation of pain. If there is an active inflammatory process or fracture of the vertebral body or posterior elements, hyperextension may be markedly limited. In disease of the upper lumbar roots, hyper-extension of the leg with the patient prone is the motion that is most limited and reproduces pain; however, in some cases of lower lumbar disc disease with thickening of the ligamentum flavum, this movement is also painful.


Neurology_Adams

Neurology_Adams. Maneuvers in the lateral decubitus position yield less information but are useful in eliciting joint disease. In cases of sacroiliac joint disease, abduction of the upside leg against resistance reproduces pain in the sacroiliac region, sometimes with radiation of the pain to the buttock, posterior thigh, and symphysis pubis. Hyperextension of the upside leg with the downside leg flexed is another test for sacroiliac disease. Rotation and abduction of the leg evoke pain in a diseased hip joint and with trochanteric bursitis. A helpful indicator of hip disease is the Patrick test: With the patient supine, the heel of the offending leg is placed on the opposite knee and pain is evoked by depressing the flexed leg and externally rotating the hip.


Neurology_Adams

Neurology_Adams. Gentle palpation and percussion of the spine are the last steps in the examination. It is preferable to first palpate the regions that are the least likely to evoke pain. At all times the examiner should know what structures are being palpated (Fig. 10-2). Localized tenderness is seldom pronounced in disease of the spine because the involved structures are so deep. Nevertheless, tenderness over a spinous process or jarring by gentle percussion may indicate the presence of local spinal inflammation (as in disc space infection), pathologic or traumatic compression fracture, metastasis, epidural abscess, or a disc lesion.


Neurology_Adams

Neurology_Adams. Tenderness over the interspinous ligaments or over the region of the articular facets between the fifth lumbar and first sacral vertebrae is consistent with lumbosacral disc disease (see Fig. 10-2, sites 2 and 3). Tenderness in this region and in the sacroiliac joints is also a frequent manifestation of ankylosing spondylitis. Arthritic changes at a facet may cause the same tenderness. Tenderness over the costovertebral angle often indicates genitourinary disease, adrenal disease, or an injury to the transverse process of the first or second lumbar vertebra (see Fig. 10-2, site 1). Tenderness on palpation of the paraspinal muscles may signify a strain of muscle attachments or injury to the underlying transverse processes of the lumbar vertebrae. Focal pain in the same parasagittal line along the thoracic spine points to inflammation of the costotransverse articulation between spine and rib (costotransversitis). Other sites of tenderness and the structures implicated by disease are


Neurology_Adams

Neurology_Adams. along the thoracic spine points to inflammation of the costotransverse articulation between spine and rib (costotransversitis). Other sites of tenderness and the structures implicated by disease are shown in the figure.


Neurology_Adams

Neurology_Adams. In palpating the spinous processes, one notes any deviation in the lateral plane (this may be indicative of fracture or arthritis) or in the anteroposterior plane. A “step-off” forward displacement of the spinous process and exaggerated lordosis are important clues to the presence of spondylolisthesis (see further on). Abdominal, rectal, and pelvic examinations may lead to the discovery of neoplastic or inflammatory diseases in these body parts that are referred to the lower part of the spine. On completion of the examination of the back and legs, one turns to a search for motor, reflex, and sensory changes in the lower extremities (see “Herniation of Lumbar Intervertebral Discs,” further on in this chapter).


Neurology_Adams

Neurology_Adams. Depending on the circumstances, these may include a blood count, the acute phase reactants, erythrocyte sedimentation rate and C-reactive protein (especially helpful in screening for spinal osteomyelitis, epidural abscess, or myeloma). Other useful blood tests are calcium, alkaline phosphatase, and prostate-specific antigen (if one suspects metastatic carcinoma of the prostate); serum protein immunoelectrophoresis (myeloma proteins); in special cases, a tuberculin test or serologic test for Brucella; a test for rheumatoid factor; and human leukocyte antigen (HLA) typing (for ankylosing spondylitis), all in the appropriate settings.


Neurology_Adams

Neurology_Adams. Conventional radiographs of the lumbar spine in the anteroposterior, lateral, and oblique planes (preferably with the patient standing) are not very useful in the routine evaluation of low back pain and sciatica but they can disclose some information. Readily demonstrable in plain films are narrowing of the intervertebral disc spaces, bony facetal or vertebral overgrowth, displacement of vertebral bodies (spondylolisthesis), compression and other fractures of the spine, and most importantly, unsuspected infiltration of bone by cancer or myeloma. However, in cases of suspected disc herniation or tumor infiltration of the spinal canal, one generally proceeds directly to MRI if subsequent action is to be taken.


Neurology_Adams

Neurology_Adams. As mentioned at different points in this chapter, there is a high rate of inconsequential lumbar spine abnormalities in the general population who have no back or radicular symptoms but it is interesting that disc herniation is found on MRI in under 1 percent (Jensen and colleagues). Administration of gadolinium at the time of MRI enhances regions of inflammation and tumor but is not particularly helpful in cases of degenerative and disc disease of the spine. The decision to administer an intravenous contrast agent depends on the degree of suspicion of infiltration of the bones or spinal canal by cancer or the need to detect spinal nerve root tumors. MRI has replaced conventional myelography for the examination of spinal disease but the latter examination, when combined with CT, provides detailed information about the dural sleeves that surround the spinal roots, disclosing subtle truncations caused by laterally situated disc herniations and at times revealing surface abnormalities of