Neurology_Adams

Neurology_Adams. Perhaps most problematic in classification but seen regularly in medical practice is the “borderline personality disorder.” As with other personality types, the pattern of behaviors is pervasive and lifelong in the affected individual. An ensemble of poorly regulated emotions, impulsive and aggressive actions, and repeated self-injury form the core aspects of the disorder. These patients typically express a range of profound emotional “pains” and a sense of dysphoria, often rapidly changing from one mood to another without provocation. There may be additional nonpsychotic problems that border on paranoia with feelings of low self-esteem. Interpersonal relationships are made unstable as a result of fear of being left alone combined with argumentativeness. These are among the most distressing individuals for families and physicians to deal with and little success has been achieved in treatment. The potential biologic roots of the borderline personality disorder were reviewed by Lieb and


Neurology_Adams

Neurology_Adams. individuals for families and physicians to deal with and little success has been achieved in treatment. The potential biologic roots of the borderline personality disorder were reviewed by Lieb and colleagues and the clinical features, by Gunderson, who emphasizes the difficulties in treating these people, currently limited mainly to various types of insight-oriented psychotherapies.


Neurology_Adams

Neurology_Adams. The defining features of the personality disorders therefore fall short of meeting the diagnostic criteria for more serious mental illness. Yet, an understanding of these personal peculiarities and their less obtrusive traits may be of great help to the physician. This knowledge makes it possible to appreciate their role as sources of perennial complaint, self-concern, and family discord, and to explain a patient’s reactions that have interfered with diagnostic and therapeutic procedures during a medical illness. It is, however, quite common for extremes of personality to create depression and anxiety, either of which is amenable to medical and psychotherapeutic treatment. As a final comment, one should never underestimate the power of maturation to ameliorate the turmoil of adolescence and to settle the young mind. ANXIETY DISORDERS (SEE ALSO CHAP. 23)


Neurology_Adams

Neurology_Adams. Although considered to be the most frequent of mental disturbances, the anxiety disorders are among the least understood. They were established as clinical entities in the late 19th century, but there are still major unresolved issues with respect to their nature, classification, and etiology. Descriptively, they are meant to include (1) anxiety disorder; (2) phobic disorder, which includes phobia of illness, social phobia, and agoraphobia; (3) obsessive-compulsive states; (4) hysteria; and (5) hypochondriasis. Former classifications included additional types called neurasthenia (dysthymia or depressive neurosis), which is now considered with the depressive illnesses, and “depersonalization neurosis” (dissociative disorders), which is a form of hysterical illness. Although each of these syndromes is clinically separable when occurring in pure form, experience shows that most patients suffer from symptoms of more than 1 type and thus are said to have “mixed neuroses.” In recent


Neurology_Adams

Neurology_Adams. these syndromes is clinically separable when occurring in pure form, experience shows that most patients suffer from symptoms of more than 1 type and thus are said to have “mixed neuroses.” In recent classifications, all of the neuroses have been again subsumed in 3 broad categories: (1) anxiety disorders (which include panic states, with and without agoraphobia, and the phobic and obsessive- compulsive neuroses); (2) somatoform disorders (comprising hysterical neurosis, or conversion disorder, and hypochondriasis); and (3) dissociative disorders.


Neurology_Adams

Neurology_Adams. An interesting view of the relative frequency and expression of mental disorders of the day was provided by an analysis of 1,045 consecutive psychiatric consultations at the New England Center Hospital during the years 1955 and 1956 in which the dominant psychiatric syndrome in approximately 20 percent of patients was an anxiety state. Other epidemiologic studies continued to disclose a strikingly high incidence of anxiety disorders in the general population (see the review of Winokur and Coryell). Lifetime prevalence figures indicate that at least 11 percent of the population is so affected—that is, some 25 million persons in the United States. Such information as is available suggests that the incidence of the neuroses is much the same in an urban population (midtown


Neurology_Adams

Neurology_Adams. New York) as it is in a rural one (Stirling County, Nova Scotia), indicating that socioeconomic, racial, and cultural factors are of limited importance. Furthermore, in times of calamity, such as the bombing of London, the incidence of neurotic symptoms was said not to have increased. Thus, it is probably an oversimplification to view neuroses as merely by-products of life in civilized society or reactions to environmental stress (see also Chap. 23). This raises the issue alluded to earlier and discussed more extensively further on: namely, the role of background personality traits in promoting extreme and persistent anxiety after a traumatizing event (posttraumatic stress disorder, PTSD; see Chap. 23).


Neurology_Adams

Neurology_Adams. The symptoms of the anxiety disorders typically arise in late childhood, adolescence, or early adult life. Admittedly, symptoms may be recognized for the first time after this age, but a good clinical rule is to suspect any mental illness that appears for the first time after the age of about 40 years to be either a depression or a dementia. As mentioned, the term anxiety neurosis was introduced by Freud to describe a syndrome of general irritability, anxious expectation, anxiety attacks, somatic accompaniments or equivalents of anxiety (breathlessness, chest pain, asthenia), and nightmares. In anxiety neurosis, this symptom complex constitutes the entire illness. However, parts of this constellation also appear with many other psychiatric diseases—bipolar disease, schizophrenia, hysteria, and phobic neurosis. Its closest link is with depression, which it resembles in another respect, namely, a strong hereditary factor, as pointed out by Cohen and White in 1949.


Neurology_Adams

Neurology_Adams. Anxiety disorder is a chronic state, some would say a disease, punctuated by recurrent attacks of acute anxiety or panic. The acute attacks are the hallmark of the disease and some psychiatrists are reluctant to make a diagnosis of anxiety neurosis in their absence. Because of the clinical features of panic attacks and particularly their episodic nature, simulating an acute medical condition, they are of special interest to neurologists and general physicians. While anxiety may be inferred from observing the activities of young children, and is reported as a form of nervousness by older children and adolescents, more often there are physical complaints at times of transition or stress during the day or year.


Neurology_Adams

Neurology_Adams. Panic attacks in their full form are prone to begin after this age and are almost as dramatic as seizures. They often begin with distressing feelings of dread and foreboding. The patient is assailed by a sense of strangeness, as though his body had changed or the surroundings were unreal. He is frightened, sometimes by the prospect of imminent death (angor animi) or of losing his mind or self-control. There may be a feeling of smothering. “I am dying” or “I can’t breathe” are characteristic expressions of alarm and panic. The heart races, breathing comes in rapid gasps, the pupils may be dilated, and the patient may sweat or tremble. The palpitation and breathing difficulties are so prominent that a cardiologist is often consulted. Some of our psychiatric colleagues identify breathlessness or a suffocating feeling as central to the diagnosis of panic (and attribute psychologic meaning to the symptom), but this is not sustained in our observations of affected patients. The symptoms


Neurology_Adams

Neurology_Adams. or a suffocating feeling as central to the diagnosis of panic (and attribute psychologic meaning to the symptom), but this is not sustained in our observations of affected patients. The symptoms abate spontaneously after 15 to 30 min, leaving the patient shaken, tense, perplexed, and often embarrassed. There is no confusion, and after the episode there is full memory of the event.


Neurology_Adams

Neurology_Adams. Most anxiety attacks are of lesser severity with complaints of apprehension, slight faintness, palpitations, or a feeling of postural instability referred to by the patient as dizziness. Breathlessness, vague chest or upper abdominal discomfort, a palpitating sensation as if the heart were beating too hard, and a generalized “washed out” feeling (asthenia) are other common symptoms. More than 50 years ago Cohen and White listed the following symptoms in order of frequency in the patients they observed: palpitation, 97 percent; easy fatigue, 93 percent; breathlessness, 90 percent; nervousness, 88 percent; chest pain, 85 percent; sighing, 79 percent; dizziness, 78 percent; apprehensiveness, 61 percent; headache, 58 percent; paresthesias, 58 percent; weakness, 56 percent; insomnia, 53 percent; unhappiness, 50 percent. What is evident from this listing is that the experience of anxiety is a polysymptomatic physical syndrome. This was the basis of the James-Lang theory of emotion—that the


Neurology_Adams

Neurology_Adams. unhappiness, 50 percent. What is evident from this listing is that the experience of anxiety is a polysymptomatic physical syndrome. This was the basis of the James-Lang theory of emotion—that the physical-sensory experience was the central one and emotion followed. It is not surprising, therefore, that many patients with chronic or recurrent symptoms first consult a physician not with a complaint of “anxiety” but with symptoms referable to the cardiorespiratory system or gastrointestinal system (dyspepsia, loss of appetite, or “irritable colon”).


Neurology_Adams

Neurology_Adams. Many patients experience a constant uneasiness that the spells may reoccur, especially in public; hence the patient may be fearful of leaving home lest help not be available should an attack occur (agoraphobia). Except in minor details, it is notable that the attacks are alike in any one individual. Between attacks, most patients feel relatively well but many complain of the symptoms of anxiety and asthenia in lesser but persistent fashion. Hyperventilation is a special, although not invariable, feature of the anxiety attack. Hyperventilation itself, by reducing the Pco2, will cause giddiness, paresthesias of the fingers, tongue, and lips, and, at times, frank tetany. However, contrary to what is stated in some textbooks, in only a minority of patients does a 3-min period of deep breathing reproduce the symptoms of an anxiety or panic attack. Nonetheless, this maneuver may be used to assist the patient in describing certain aspects of an attack.


Neurology_Adams

Neurology_Adams. Attacks in minor form, without the full force of the physical accompaniments may occur at infrequent intervals or several times a day. Much to the patient’s surprise, they usually occur in situations where there are no easily recognizable sources of fear, as when the patient is sitting quietly at home or has just awakened from sleep. In other instances, a trying or upsetting experience induces an attack, which is nonetheless excessive for the condition that provoked it. In some patients, attacks are brought on consistently by confinement to a closed space (claustrophobia)—an elevator, for example—or by crowded surroundings, as in a church, restaurant, or theater. An anxiety state frequently follows an accident and then may, according to Modlin, be a source of ongoing disability, a condition more akin to posttraumatic stress disorder. Similar spells of anxiety are also a prominent feature of the postconcussive and posttraumatic stress syndromes.


Neurology_Adams

Neurology_Adams. From the patient’s life history, 2 patterns of anxiety neurosis are discernible. In one, there is a nearly lifelong history of poor exercise tolerance, little stamina, and inability to do heavy physical work or participate in vigorous sports, tenseness, nervousness, and intolerance of crowds, that is, what had in the past been called neurasthenia (or nervous exhaustion), named by the neurologist George Beard in 1869 and renamed innumerable times by others as a result of its widespread applicability in all branches of medicine. When these symptoms arose during military service, they were designated, since the American Civil War as neurocirculatory asthenia, “irritable heart,” or “soldier’s heart.”


Neurology_Adams

Neurology_Adams. The course of anxiety traits is variable. A 20-year followup study by Wheeler and associates showed that symptoms were still present in 88 percent but persisted in being moderately or severely disabling in only 15 percent. Most affected patients were able to work and to enjoy a reasonably normal family and social life. Their only liability to further psychiatric illness was to later anxious depression, whereas so-called psychosomatic illnesses and other psychiatric illnesses did not occur more frequently than in the general population. Those with uncomplicated anxiety neurosis rarely commit suicide. Anxiety disorder has been attributed to a genetic abnormality, to a “constitutional weakness” of the nervous system, to social and psychologic factors, and to physiologic and biochemical derangements; but none of these factors provides a completely satisfactory explanation of the primary problem.


Neurology_Adams

Neurology_Adams. The onset of both acute and chronic anxiety, as mentioned, is rare before age 18 years or after 35 to 40 years of age (average age of onset 25 years). The condition in some series is twice as frequent in women as in men and there is clearly a high familial incidence. In one study (Wheeler et al) there was a prevalence of 49 percent among the grown children of patients with anxiety neurosis, compared with 5 percent in the general population. Slater and Shields found that there was a concordance rate of 40 percent in identical twins, compared with 4 percent in dizygotic twins. Among the relatives of index cases, the mothers suffered from anxiety neurosis more often than did the fathers; in the latter, alcoholism was more frequent than in the population at large (Modlin). A clear pattern of inheritance has not been established, but it approximates that of autosomal dominance with incomplete penetrance. The psychodynamic theories that attempt to provide a unified explanation of these


Neurology_Adams

Neurology_Adams. of inheritance has not been established, but it approximates that of autosomal dominance with incomplete penetrance. The psychodynamic theories that attempt to provide a unified explanation of these diverse anxiety states were reviewed by Nemiah and we do not dismiss them but cannot comment authoritatively.


Neurology_Adams

Neurology_Adams. The symptoms of an anxiety resemble those of fear in many ways, although nearly always the former are longer in duration and less distinct. The most important difference, however, is that the cause of fear is known to the patient, whereas that of anxiety is not. The most extreme but not inconceivable interpretation of anxiety is the James-Lange theory of emotion, mentioned earlier, which attributes the psychologic experience entirely to the accompanying physical symptoms.


Neurology_Adams

Neurology_Adams. On the physiologic and biochemical side, it has been observed that anger provokes an excessive secretion of norepinephrine, whereas fear is accompanied by increased secretion of epinephrine. Actually, fear activates the autonomic nervous system as a whole and the increase in epinephrine is more than counterbalanced by a parasympathetic discharge. Attention has been focused on overactivity of the locus ceruleus and upper brainstem nuclei as the possible anatomic substrates of anxiety (Judd et al). Other studies have implicated serotonergic centers. Evidently, the responsiveness of the autonomic nervous system in these patients remains heightened and a number of stimuli (cold, pain, muscular effort) may produce abnormal responses in pulse, respiration, oxygen consumption, and work performance. Another interesting abnormality (noted by Cohen et al) is that the blood lactic acid levels in response to exercise are higher than normal. The presence of these changes does not necessarily mean


Neurology_Adams

Neurology_Adams. Another interesting abnormality (noted by Cohen et al) is that the blood lactic acid levels in response to exercise are higher than normal. The presence of these changes does not necessarily mean that they are causal; they are as likely secondary to other factors such as poor physical condition and apprehension associated with the syndrome. Nevertheless, some investigators have found that infusions of lactic acid can trigger panic attacks in persons with anxiety neurosis (Liebowitz et al). Subsequently, numerous other theories of causation have been proposed based on the reported provocation of panic attacks by a number of different substances—carbon dioxide, yohimbine, gamma-aminobutyric acid (GABA), isoproterenol, and others. None provides a comprehensive biologic explanation.


Neurology_Adams

Neurology_Adams. Studies correlating cerebral function and blood flow indicate that when panic is induced by an intravenous injection of sodium lactate, there is an immediate increase in blood flow to the cortex of both temporal lobes. In states of fear, the tips of the temporal lobes and the amygdaloid nuclei are known to become activated. In the relaxed period between panic attacks, the right limbic system and the parahippocampal gyrus are abnormally active in some studies. As with the aforementioned biochemical models, these seem to be more reflections of brain activity in response to the psychic experience than they are explanations. Nevertheless, parts of the limbic system are presumably involved in a germinal way in the production and perpetuation of anxiety and its related states.


Neurology_Adams

Neurology_Adams. The discovery that the benzodiazepines bind to specific sites of the GABA receptor complex and that the sedative and amnesic effects (α1 subunit) of these drugs seem to be separable from their anxiolytic effects (α2 subunit) raises the possibility that abnormalities of the GABA system directly underlie anxiety. However, there is only indirect evidence for this mechanism. Of potentially greater importance is the finding of genetic polymorphisms that relate statistically to the presence of anxiety states, such as one in the serotonin transporter gene (Lesch et al). It has been estimated that allelic differences on the chromosome contribute perhaps 10 percent to the overall anxiety tendency. One presumes that there are numerous additional genes that participate in a similar way. Others have not found this particular association or have found it only in patients with generalized anxiety and not in those with panic attacks. Consequently, the precise relationship of genetic polymorphisms to


Neurology_Adams

Neurology_Adams. found this particular association or have found it only in patients with generalized anxiety and not in those with panic attacks. Consequently, the precise relationship of genetic polymorphisms to anxiety states cannot be stated at this moment, but a heritable component is undoubted.


Neurology_Adams

Neurology_Adams. Shorn of the psychologic components of apprehension and fear, the anxiety and panic attack consists essentially of an excessive autonomic, mainly sympathetic, discharge. Some of the autonomic symptoms are therefore duplicated by pheochromocytoma. The prominence of chest discomfort and respiratory distress during an acute anxiety attack may be mistaken for myocardial ischemia, in which case the patient is often subjected to a series of studies of cardiac function. Another form of the illness—in which nondescript dizziness, vague difficulty with visual clarity, and fear of losing consciousness are the most prominent features—may be mistaken for an vestibular problem (see “Nonvertiginous Types of Dizziness” in Chap. 14) or for epilepsy. In contrast, headache is (surprisingly) a relatively infrequent experience and the diagnosis should be suspect if it is a prominent feature. Other medical diseases that may be brought to mind by isolated elements of an anxiety state are pulmonary


Neurology_Adams

Neurology_Adams. infrequent experience and the diagnosis should be suspect if it is a prominent feature. Other medical diseases that may be brought to mind by isolated elements of an anxiety state are pulmonary embolism, cardiac arrhythmias, hypoglycemia, hypoparathyroidism, alcohol, drug, nicotine withdrawal and especially, complex partial seizures. The autoantibody syndrome associated with NMDA antibodies and some instances without these antibodies may have anxiety as its opening feature. In regard to seizures as imitators of anxiety, however, loss of consciousness, incontinence, and clonic or myoclonic movements do not occur in anxiety spells. Adherence to the diagnostic criteria of these disease states readily permits their differentiation from acute anxiety, but diagnosis may be difficult if the symptoms are brief.


Neurology_Adams

Neurology_Adams. Of equal importance is the relationship of anxiety to depression. A large proportion of patients with depression have symptoms of anxiety. Indeed, some psychiatrists believe that anxiety neurosis is accounted for mainly by a variant of depression. As has been mentioned, an anxiety state appearing for the first time after the 40th year usually proves to be primarily a depression, although it may be that a predisposing personality trait is involved. The presence of symptoms such as overwhelming fatigue, self-deprecation, and feelings of hopelessness and, of course, ideas of self-destruction makes depression the fundamental diagnosis, with anxiety an associated feature (anxious depression). As mentioned, a very small number of patients with a condition diagnosed as pure anxiety neurosis have committed suicide, but this does not hold if depression is the central illness.


Neurology_Adams

Neurology_Adams. Schizophrenia may also begin with prominent anxiety symptoms. Here the diagnosis rests on finding the characteristic thought disorder of schizophrenia, which may emerge only with time and after several interviews. Hysteria may include anxiety symptoms, though they are seldom prominent, and phobic and obsessive-compulsive neuroses constantly create an anxious state in affected patients, but each has distinguishing attributes.


Neurology_Adams

Neurology_Adams. Certain medications, particularly anxiolytics and antidepressants, are effective in suppressing panic attacks and creating a sense of well-being. Among these, the benzodiazepine alprazolam had been favored by some psychiatrists, but lorazepam and clonazepam are almost as effective and are considered less likely to cause dependence. They are all effective within hours. In mild cases, the benzodiazepines may be used intermittently rather than several times daily, but they tend to be less useful once a panic attack has become established. Panic attacks tend to recur when the medications are discontinued, even after prolonged (6 to 12 months) administration. Any reduction in the amount of these medications should be gradual.


Neurology_Adams

Neurology_Adams. Tricyclic antidepressants and drugs that raise serotonin concentrations in the nervous system (selective serotonin reuptake inhibitors [SSRIs]) may also be effective in the prevention of panic attacks and agoraphobia, but their onset of action is delayed for weeks. They become useful for symptoms of anxiety that recur or persist for more than several months. The doses are similar to those used to treat depression, and small differences between them do not seem to be clinically important (see Chap. 48). Buspirone, a specific serotonin 5-HT2 agonist, has been promoted as effective in the treatment of anxiety and as a surrogate for benzodiazepines, but to us its benefit has seemed to be slight. It is important to point out that during the initial weeks of administration of antidepressants, the underlying anxiety symptoms may worsen and an anxiolytic is usually required until the antidepressant becomes effective. Propranolol, 10 to 20 mg tid, or a long-acting form of adrenergic blocker,


Neurology_Adams

Neurology_Adams. the underlying anxiety symptoms may worsen and an anxiolytic is usually required until the antidepressant becomes effective. Propranolol, 10 to 20 mg tid, or a long-acting form of adrenergic blocker, can reduce many of the autonomic accompaniments of anxiety and is useful to many patients. Psychiatric consultation is, of course, invaluable. With regard to psychotherapy, behavioral therapy (progressive exposure of the patient to panic-provoking situations) is said to be beneficial, particularly if agoraphobia is a major symptom. Relaxation activities, including biofeedback and meditation, help many patients, although persistence is required in performing these exercises at least once daily and they are less helpful once a panic attack has begun. Cognitive-behavioral psychotherapy, which is discussed in relation to the treatment of depression in Chap. 48, also appears to be useful in the treatment of panic disorder, according to Andreasen and Black.


Neurology_Adams

Neurology_Adams. A cardiac consultation and some simple tests (electrocardiogram, chest films) are often needed to reinforce to the patient the benign nature of the cardiac and respiratory symptoms and to alleviate fear of heart disease. These and other concepts of the treatment of anxiety were discussed by Goodwin and Guze. Anxiety symptoms arising in relation to a particular threatening event in a nonneurotic individual carry the best prognosis, but the symptoms may be prolonged, a feature of posttraumatic stress disorder.


Neurology_Adams

Neurology_Adams. In this state, patients are overwhelmed by an intense and irrational fear of some animal, object, social situation, or disease. Although acknowledging that there are no grounds for a particular fear (hence it is not a delusion) and that such provocative stimuli are for the most part innocuous, the patient is nonetheless powerless to suppress it. This disorder was known to Hippocrates, who drew a distinction between normal and morbid fears. Westphal, in 1871, was the first to give morbid fears the status of a disease.


Neurology_Adams

Neurology_Adams. Unlike an anxiety attack, a phobia focuses on a specific object or situation. The patient is chronically fearful of a particular animal or situation and becomes extremely anxious or panic stricken and incapacitated when placed in a situation that evokes the phobia. These situations are avoided at all costs. As a result, it may be impossible for the patient to leave the house or neighborhood unaccompanied or at all, mingle in a crowd, walk across a bridge, or travel by air. This fear of being in places or situations from which escape might be difficult or extremely embarrassing is spoken of as agoraphobia. (Agoraphobia, however, is a secondary feature of other psychiatric disorders, the most frequent being anxiety with panic attacks as already mentioned.) The most common phobia—and one that is not disabling for the most part—is claustrophobia, the fear of being confined in a close space such as an elevator or a magnetic resonance scanner. Other phobias are those of open (agoraphobia),


Neurology_Adams

Neurology_Adams. is not disabling for the most part—is claustrophobia, the fear of being confined in a close space such as an elevator or a magnetic resonance scanner. Other phobias are those of open (agoraphobia), closed, or high places, dogs, cats, insects, dirt, sprays and other contaminants, air travel, HIV, cancer, insanity, and death. Feelings of helplessness, pessimism, and despondency, the hallmarks of a depressive illness, result after years of phobic suffering. Often there are obsessive-compulsive tendencies as well, and some patients are hypochondriacal. Phobias are essentially obsessive fears and are somehow allied with this latter category of neurosis. The present authors have observed a number of patients whose phobic (or obsessive- compulsive) states became greatly exaggerated as an endogenous depression developed. Recovery from the depression returned them to their earlier and milder phobic state.


Neurology_Adams

Neurology_Adams. Like the pure phobic states, a state dominated by obsessions and compulsions is relatively rare, occurring in less than 5 percent of patients seeking help in a psychiatric outpatient clinic, but it can be extremely disabling. Minor compulsions (e.g., not stepping on cracks in the sidewalk), like minor phobias, are common in children, cause little or no distress, and disappear in later life. A few, such as rechecking a locked door or a gas stove, may persist throughout life. Also, certain habits and rigid, obsessional ways of thinking, stubbornness, extreme punctuality, and excessive attention to detail may be persistent but excite little attention medically unless they interfere with a diagnostic procedure or the treatment of a medical disease.


Neurology_Adams

Neurology_Adams. Obsessive-compulsive disorder begins in adolescence or early adult years, although treatment may not be sought until middle age. The 2 sexes are equally affected. The onset is usually gradual and often cannot be accurately dated, but in some cases it is precipitated by a particular event in the patient’s life, such as the death of a relative. The family history often discloses a high incidence of obsessional or phobic personality in other members. There is usually a prevailing undercurrent of insecurity and anxiety.


Neurology_Adams

Neurology_Adams. Obsessions are defined as imperative and distressing thoughts and impulses that persist in the patient’s mind despite a desire to resist and to get rid of them. They take various forms; the most common are intellectual obsessions, in which phrases, rhymes, ideas, or vivid images (these are often absurd, blasphemous, obscene, and sometimes frightening) constantly intrude into consciousness; impulsive obsessions, in which the mind is dominated by an impulse to kill oneself, to stab one’s children, or to perform some other objectionable act; and inhibiting obsessions, in which every act must be ruminated upon and analyzed before it is carried out—a state that is cleverly called doubting mania. Every effort at distraction fails to rid the patient of the obsessive thought. It engulfs the individual’s mind, rendering the person dysphoric and, often, inefficient. Probably the most disturbing obsessions are the impulsive ones, in which patients constantly struggle with the fear that they will


Neurology_Adams

Neurology_Adams. mind, rendering the person dysphoric and, often, inefficient. Probably the most disturbing obsessions are the impulsive ones, in which patients constantly struggle with the fear that they will put some terrible thought into action. Even as they tell of the obsession, they reveal a severe underlying anxiety and seek reassurance that they will not yield to it. Fortunately, such patients rarely obey their pathologic impulses. Phobias, as mentioned earlier, are considered by some authorities to be essentially obsessive fears and are included in this category of neurosis.


Neurology_Adams

Neurology_Adams. Compulsions are acts that result from obsessions. These are single acts or a series of acts (rituals) that the patient must carry out in order to put his mind at ease. Examples are repeated checking of the gas jets or the locks on doors, adjusting articles of clothing, repeated hand washing, using a clean handkerchief to wipe objects that have been touched by others, tasting foods in specific ways, and touching or arranging objects in a particular sequence. The most common of these obsessions and compulsions center around contamination concerns that lead to repeated hand washing or bathing. Other obsessions and compulsions can be identified as clusters of thoughts that derive from the above-mentioned concern about harm to oneself or to others and consequent checking on others. Less common clusters involve excessive focus on symmetry, precision, and ordering, and on saving and hoarding.


Neurology_Adams

Neurology_Adams. Certain motor disturbances—namely, habit spasms or tics—are, in a sense, motor compulsions. They consist of repetitious movements of the shoulders, arms, hands, and certain of the facial muscles (see Chap. 4). One feature that separates quasivoluntary tics from involuntary movements of extrapyramidal type is the patient’s feeling that the tics must be carried out to relieve an inner tension. Unlike compulsions, however, tics are not usually based directly on obsessive thoughts—except perhaps the Gilles de la Tourette syndrome, in which multiple tics are combined with compulsive utterances, often offensive ones (see later).


Neurology_Adams

Neurology_Adams. In all these obsessions and compulsions and in the phobias, patients recognize the irrationality of their ideas and behaviors, yet are powerless to control them. It is this insight into the obsessional experience and the struggle against it that distinguish obsessions from delusions. After the condition has persisted for a time, they may become depressed and suffer from typical anxiety attacks. Mechanisms of Obsessive Disorder


Neurology_Adams

Neurology_Adams. For many years, a number of psychodynamic conceptualizations held sway of obsessional states as the product of intrapsychic conflicts. Only relatively recently has a more reasonable neurobiologic model been advanced. These are largely derived from the findings of functional imaging, which have been quite consistent in demonstrating increased metabolic activity in the orbitofrontal cortex, cingulate, and, to a lesser extent, striatum. The orbitofrontal cortex and amygdala were reported to be shrunken in other cases. In a study of 13 patients who developed elements of obsessiveness and compulsive disorder after incurring focal brain lesions, Berthier and colleagues found lesions in diverse loci, including the cingulate, frontal, and temporal cortices, as well as the basal ganglia. Two of the most accurately localized lesions in their series were a hamartoma of the right parahippocampal gyrus and an infarction in the posterior putamen. The presence of brain injuries and seizure disorders


Neurology_Adams

Neurology_Adams. most accurately localized lesions in their series were a hamartoma of the right parahippocampal gyrus and an infarction in the posterior putamen. The presence of brain injuries and seizure disorders in other of their patients made precise localization less certain.


Neurology_Adams

Neurology_Adams. Additional insight into obsessive-compulsive disorder may be obtained from the many cases in which acquired striatal damage may be linked to obsessional behavior. One such entity is a poststreptococcal tic disorder termed PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections), discussed in Chap. 4. This disorder is presumably related to another extrapyramidal condition, Sydenham chorea, in which tics and similar movement disorders, as well as obsessive behavioral abnormalities, have long been known to coexist. Functional imaging studies in patients with PANDAS have yielded variable findings but generally there has been increased activity in the caudate nucleus and orbitofrontal cortex in association with the patient’s compulsive thoughts.


Neurology_Adams

Neurology_Adams. The Gilles de la Tourette syndrome of multiple tics, including vocal ones, beginning in childhood or adolescence and lasting more than a year, has a strong component of obsessive-compulsive disorder in more than half of patients. Both disorders occur in a pattern of inheritance that is close to an autosomal dominant trait with incomplete penetrance (Kurlan). Because dopamine antagonists (Haldol was the first of these to be tried systematically, by Shapiro) have been beneficial in the management of the Tourette syndrome, a number of etiologic hypotheses revolve around serotonergic and dopaminergic neurotransmitter systems (Baxter). The neurochemical alterations in obsessive-compulsive disorder have been based in part on the responses to medications, notably to the serotonin reuptake inhibitors, as noted further on. These agents are found to be of therapeutic benefit, as are stereotactic neurosurgical lesions in the cingulate gyri (see further on). More extensive comments on the Gilles


Neurology_Adams

Neurology_Adams. as noted further on. These agents are found to be of therapeutic benefit, as are stereotactic neurosurgical lesions in the cingulate gyri (see further on). More extensive comments on the Gilles de la Tourette syndrome are found in Chap. 4.


Neurology_Adams

Neurology_Adams. This is best left to the experienced psychiatrist. At the least there should be a trial of therapy using behavioral modification techniques. In the case of phobic neurosis, the aim is to reduce the patient’s fear to the extent that exposure to the phobic situation can be tolerated. A popular form of therapy is systematic desensitization, which consists of increasing and graded exposure of the patient to the object or situation that arouses the fear. Psychotherapy, if undertaken, need not be intensive, consisting instead of repeated explanation, reassurance, and guidance in dealing with symptoms. As with phobic neurosis, several reports have indicated that compulsive rituals can often be abolished by the techniques of behavior therapy. Much in vogue is cognitive-behavioral psychotherapy discussed in Chap. 48.


Neurology_Adams

Neurology_Adams. Certain medications, particularly the SSRI types such as fluoxetine, are considered to be effective in reducing obsessions and compulsions in more than half of patients. The less selective agent, clomipramine, is also highly effective, as were the commonly used tricyclic antidepressants in the past, but clomipramine is not nearly as well tolerated as are the conventional SSRI drugs (see review by Stein). In the past, cingulotomy produced symptomatic improvement in both phobic and obsessional neuroses and was considered a reasonable procedure. This measure is largely outdated as the implantation of electrical stimulating electrodes (deep brain stimulation) in this region or in the subthalamic nucleus has proved effective for intractable and disabling obsessive compulsive disorder but without affecting the degree of anxiety and at the expense of a moderate number of surgical complications (Mallet et al).


Neurology_Adams

Neurology_Adams. This subject is of great importance to neurologists and general physicians because of its frequency. Any sense that this was a disorder of the Victorian era belies any modern neurologist’s daily experience. What has changed for the better is the reduced stigmatization of the disorder and a more straightforward, non-judgmental approach to treatment.


Neurology_Adams

Neurology_Adams. Although hysteria has been known since ancient times, many writers credit the first description of the syndrome to the French physician Briquet in 1859. Charcot later elaborated certain manifestations of the disease, particularly those with a theatrical aspect, and thereby interested Freud and Janet in the problem. Charcot demonstrated that the symptoms could be produced and relieved by hypnosis (mesmerism). There have been many extensive expositions of Charcot’s work in this field and his consideration of hysteria as an organic disease, perhaps allied with epilepsy but representing in any case a “functional” disorder of brain. One of the authors of this text has devoted a book to the subject (Ropper and Burrell). Charcot’s acolyte, Janet postulated a dissociative state of mind to account for certain features, such as trances and fugue states, a term that has reappeared in modern psychiatry. Freud and his followers conceived of hysterical symptoms as a product of “ego defense


Neurology_Adams

Neurology_Adams. account for certain features, such as trances and fugue states, a term that has reappeared in modern psychiatry. Freud and his followers conceived of hysterical symptoms as a product of “ego defense mechanisms” in which psychic energy, generated by unconscious sexual conflicts, was “converted” into physical symptoms. This latter concept was widely accepted, to the point where the term conversion became incorporated into the nomenclature of the neuroses and the terms conversion symptoms and conversion reaction came to be equated with the disease hysteria. We see no merit in the dichotomy, based on unsubstantiated psychodynamic theory, of a conversion type as separate from a dissociative type of hysteria, as claimed by the DSM classification. Nemiah, who is in other respects partial to the psychoanalytic interpretation, agrees. The term hysteria is probably best reserved for a disease and is characterized by a distinctive age of onset, sex predilection, natural history, and certain


Neurology_Adams

Neurology_Adams. the psychoanalytic interpretation, agrees. The term hysteria is probably best reserved for a disease and is characterized by a distinctive age of onset, sex predilection, natural history, and certain somatic symptoms and signs, which typically include physical symptoms that cannot be explained on the basis of the structure and function of the nervous system, dissociative reactions, or states of “multiple personality.” We use the term “psychogenic” in this chapter and throughout the book as an etiologic term broadly attached to such dramatic neurologic signs and symptoms that are not explicable on the basis of a lesion of the nervous system.


Neurology_Adams

Neurology_Adams. In clinical neurology one encounters two types of psychogenic neurologic signs, both identified as having no possibility of explanation in disease of the nervous system: (1) a chronic illness marked by multiple and often dramatically presented symptoms and somatic abnormalities of “classic hysteria,” almost limited to girls and women and (2) an illness predominantly of men but also of women who develop physical symptoms or remain inexplicably disabled for the purpose of obtaining compensation, influencing litigation, avoiding military duty or imprisonment, or for the manipulation of some other interpersonal or societal situation. This latter state is called compensation neurosis, compensation hysteria, or hysteria with sociopathy, in other words, malingering.


Neurology_Adams

Neurology_Adams. This in the past accounted for 1 to 2 percent of admissions to a neurologic service and a greater number of outpatient visits but now is represented mainly by hysterical seizures and convulsions, “psychogenic nonepileptic seizures”, or PNES (see further on and Chap. 15). The more general chronic form that does not include epileptic-like attacks usually has its onset in the teens or early twenties, almost exclusively in young women; a very few cases begin before puberty. Once established, the symptoms recur intermittently, although with reduced frequency, throughout the adult years even to an advanced age. No doubt there are cases of lesser severity in which symptoms occur only a few times or perhaps only once, just as there are mild forms of other diseases. The patient may be seen for the first time during middle life or later, and the earlier history may not at first be forthcoming. Careful probing almost invariably reveals that the earliest manifestations of the chronic form of


Neurology_Adams

Neurology_Adams. the first time during middle life or later, and the earlier history may not at first be forthcoming. Careful probing almost invariably reveals that the earliest manifestations of the chronic form of illness had appeared before the age of about 25 years.


Neurology_Adams

Neurology_Adams. Other important data are also revealed by eliciting a careful past history. During late childhood and adolescence, the normal activities of the patient, including education, had often been interrupted by periods of ill-defined illness. In the past, rheumatic fever, and in the current era, chronic fatigue, Lyme disease, sick building syndrome, or multiple environmental allergy may be carried as explanatory diagnoses from other physicians or the patient’s research on the Internet. Later in life, problems in work adjustment and marriage are frequent. Notable in many cases is a high incidence of marital incompatibility, separation, and divorce. The patient’s life history is punctuated by symptoms that do not conform to recognizable patterns of medical and surgical disease. For these ailments, many forms of therapy including surgical operations may have been performed.


Neurology_Adams

Neurology_Adams. In the past, rarely had adult life been reached without at least one abdominal operation for vague abdominal pain, persistent nausea and vomiting, or an obscure gynecologic complaint. Often the indications for the surgical procedures were unclear; moreover, the same symptoms or others often recurred to complicate the convalescence. The biographies of these patients are replete with disorders that center about menstrual, sexual, and procreative functions. Menstrual periods may be painfully prostrating, irregular, or excessive. Sexual intercourse may be painful or unpleasant. Pregnancies may be exceedingly difficult; the common vomiting of the first trimester may persist all through the gestational period, with weight loss and prostration; labor may be unusually difficult and prolonged, and all manner of unpredictable complications are said to have occurred during and after parturition.


Neurology_Adams

Neurology_Adams. Hysteria in its fullest form is a polysymptomatic disorder, implicating at some time and in some patients, almost every organ system. In a study of 50 unmistakable cases of hysteria (as compared with a control group of 50 healthy women), the most frequent symptoms reported by Purtell and colleagues were headache, blurred vision, lump in the throat, loss of voice, dyspnea, palpitation, anxiety attacks, anorexia, nausea and vomiting, abdominal pain, unusual food allergies, severe menstrual pain, urinary retention, painful intercourse, paresthesias, dizzy spells, nervousness, and easy crying.


Neurology_Adams

Neurology_Adams. The mental examination of the patient with hysteria demonstrates a lack of precision in relating the details of the illness. Questions regarding the chief complaint usually elicit a narration of a series of incidents or problems, many of which prove to have little or no relevance to the question. Memory defects (amnesic gaps) are apparent while the history is being taken; the patient appears to have forgotten important segments of the history, some of which he had clearly described in the past and are part of the medical record. The description of symptoms is dramatic and not in accord with the facts as elicited from other members of the family. Often, a rather casual demeanor is manifest, the patient insisting that everything in her life is quite normal and controlled, when, in fact, her medical record is checkered with instances of dramatic and unexplained illness. This calm attitude toward a turbulent illness and seemingly disabling physical signs is so common that it has been


Neurology_Adams

Neurology_Adams. her medical record is checkered with instances of dramatic and unexplained illness. This calm attitude toward a turbulent illness and seemingly disabling physical signs is so common that it has been singled out as an important characteristic of hysteria, la belle indifference. Other patients, however, are obviously tense and anxious and report frank anxiety attacks. Emotional reactions are superficial and scenes that are disturbing to others are quickly forgotten. Claims of early life sexual or physical abuse are common and often prove to be true, or sometimes are not valid; when present, they probably play a role in the genesis of some cases (see further on).


Neurology_Adams

Neurology_Adams. There are no pathognomonic findings. Although many in the past have commented on the rather youthful, girlish appearance and coquettish (“seductive”) manner of the patients, these by no means characterize most patients in the current era. The abdomen may be diffusely and exceedingly tender but without other signs of abdominal disease. The so-called stigmata of hysteria—that is, corneal anesthesia; absence of gag reflex; spots of pain and tenderness over the scalp, sternum, breasts, lower ribs, and ovaries—are often suggested by the examiner and are too inconsistent to be of much help in diagnosis. The relative value of various features of the history, examination and psychological profile that may be of value in diagnosis were summarized by Lazare, and the psychiatric features were summarized by Raskin et al (in now outdated terms) but the point being that few are definitive. The variation and pleomorphism of the physical signs are limited only by the patient’s ability to produce them


Neurology_Adams

Neurology_Adams. by Raskin et al (in now outdated terms) but the point being that few are definitive. The variation and pleomorphism of the physical signs are limited only by the patient’s ability to produce them by voluntary effort. Accordingly, symptoms and signs that are beyond volitional control should not be accepted as manifestations of hysteria. Sometimes the patient’s physical signs are an imitation of those of another member of the family (“folie à deux”) or are evoked by a stressful event in the patient’s personal life. However, this may not be disclosed at the time of the first examination.


Neurology_Adams

Neurology_Adams. Special Neurologic Syndromes of Psychogenic Origin A few hysterical syndromes occur with regularity that every physician may expect to encounter them. Most are neurologic in nature. They constitute some of the most puzzling diagnostic problems in medicine.


Neurology_Adams

Neurology_Adams. This may involve any part of the body; generalized or localized headache, “atypical facial pain,” vague abdominal pain, and chronic back pain with camptocormia are the most frequent and troublesome. In many of these patients the response to analgesic drugs has been unusual or excessive, and some of them are addicted. The hysterical patient may respond readily to a placebo as though it were a potent drug, but it should be pointed out that this is a notoriously unreliable means of distinguishing hysterical pain from that of other diseases. A greater error is to mistake the pain of osteomyelitis or visceral tumor—before other symptoms have developed—for a manifestation of hysteria. There are several helpful diagnostic features of hysterical pain: (1) the patient’s inability to give a clear, concise description of the type of pain; (2) the location of the pain does not conform to the pattern of pain in the familiar medical syndromes; (3) the dramatic elaborations of its intensity


Neurology_Adams

Neurology_Adams. clear, concise description of the type of pain; (2) the location of the pain does not conform to the pattern of pain in the familiar medical syndromes; (3) the dramatic elaborations of its intensity (speaking in inflated metaphors—“like a giant knife stabbing”) and its effects on the body (“tearing my limb off”); (4) its persistence, either continuous or intermittent, for long periods of time; (5) the assumption of bizarre postures; and, most important; and (6) the coexistence of other clinical features or previous attacks of hysterical nature.


Neurology_Adams

Neurology_Adams. This is often combined with pain and tenderness in the lower abdomen and had many times in the past resulted in unnecessary appendectomies and removal of pelvic organs in adolescent girls and young women. The vomiting often occurs after a meal, leaving the patient hungry and ready to eat again; it may be induced by unpleasant circumstances. Some of these patients can vomit at will, regurgitating food from the stomach like a ruminant animal. Vomiting may persist for weeks with no cause being found. Weight loss may occur, but seldom to the degree anticipated. As remarked earlier, the usual first-trimester vomiting of pregnancy may continue throughout the entire 9 months, and occasionally pregnancy will be interrupted because of it. Anorexia may be a prominent associated symptom and must be differentiated from anorexia nervosa–bulimia, another closely related disease of young women. Factitious elf-induced vomiting is more aligned with malingering, with which hysteria may be allied.


Neurology_Adams

Neurology_Adams. Psychogenic Seizures (PNES), Trances, and Fugues (See Also Chap. 15)


Neurology_Adams

Neurology_Adams. These conditions seem to be no less frequent than in the days of Charcot, when la grande attaque d’hysterie was often exhibited before medical audiences, but it is quite familiar to all neurologists and one of the main concerns of epileptologists. To witness an attack is of great assistance in diagnosis but electroencephalogram (EEG) monitoring is often required for certainty. The lack of an aura, initiating cry, hurtful fall, or incontinence; the presence of peculiar movements such as grimacing, squirming, thrashing and flailing of the limbs, side-to-side motions of the head, and striking at or resisting those who offer assistance; the retention of consciousness during a motor seizure that involves both sides of the body; a long duration of the seizure, its abrupt termination by strong sensory stimulation, lack of postictal confusion, and failure to produce a rise in creatine kinase—are all typical of the psychogenic attack. Sometimes hyperventilation will initiate an attack and is


Neurology_Adams

Neurology_Adams. sensory stimulation, lack of postictal confusion, and failure to produce a rise in creatine kinase—are all typical of the psychogenic attack. Sometimes hyperventilation will initiate an attack and is therefore a useful diagnostic maneuver. Both epilepsy, particularly of frontal-lobe type, and hysteria may occur in the same patient, a combination that invariably causes difficulty in diagnosis as discussed in Chap. 15.


Neurology_Adams

Neurology_Adams. Hysterical trances or fugues, in which the patient wanders about for hours or days and carries out complex acts may simulate temporal lobe epilepsy or any of the conditions that lead to confusional psychosis. The most reliable point of differentiation comes from observation of the patient, who, if hysterical, is likely to indicate a degree of alertness and promptness of response not seen in temporal lobe seizures or confusional states. Following the episode, an interview with the patient—under the influence of hypnosis, strong suggestion, or midazolam (formerly used was amobarbital [Amytal])—will often reveal memories of what happened during the episode. This helps to exclude the possibility of an epileptic spell. Hysterical Paralyses, Gait, Sensory Loss, and Tremors (See Also Chaps. 3, 4, and 6)


Neurology_Adams

Neurology_Adams. Hysterical palsies may involve an arm, a leg, one side of the body, or both legs. If the affected limb can be moved at all, muscle action is weak and tremulous. Movements are slow, tentative, and poorly sustained; often it can be demonstrated that the strength of voluntary movement is proportional to the resistance offered by the examiner, thus imparting a “give-way” character, as noted in the discussion of these signs in Chap. 3. One can detect by palpation that agonist and antagonist muscles are contracting simultaneously, thereby holding the limb in place rather than opposing the examiner, and when the resistance is suddenly withdrawn, there is no follow-through or rebound, as is normally the case. Many other signs have been devised to demonstrate inconsistencies with normal physiologic principles and a purposive lack of cooperation. These are elaborated upon in the articles by Stone and associates (2002b and 2013a, b). The discrepancies are usually found by testing an agonist,


Neurology_Adams

Neurology_Adams. principles and a purposive lack of cooperation. These are elaborated upon in the articles by Stone and associates (2002b and 2013a, b). The discrepancies are usually found by testing an agonist, antagonist, or fixator movement while the patient is focused on making an effort with another group of muscles (e.g., the Hoover sign; see Chap 3). Muscular tone in the affected limbs is usually normal but slight resistance may sometimes be found. A seeming lack of effort and the absence of full compliance with the examiner’s requests during the testing of muscle strength, while common in hysterical patients, are not confined to them; one encounters such findings not infrequently during the examination of suggestible but nonhysterical patients who harbor a neurologic disease and in those with a painful condition in an adjacent joint.


Neurology_Adams

Neurology_Adams. Walking and standing may be impossible (astasia-abasia) or the gait may be bizarre with collapsing legs that bring the patient to a squat, or a “skating” gait in which 1 foot is pushed ahead of the body. Other forms—some quite absurd, as noted in Chap. 6—are easily recognized as inconsistent with the makings of the nervous system in disease. Weakness and poor balance are combined elements in both the quadriparetic and hemiparetic forms. In Keane’s informative series of 60 cases of hysterical gait, the hemiparetic and monocrural forms were twice as frequent as the quadriparetic. The gait disorder is sometimes difficult to describe because of its variability. Sudden falls without voluntary protective movements and inconsistencies of balance are helpful features. Difficulty in walking and moving the legs that disappears when the patient is seated is, of course, not unique to hysteria; it also occurs in so-called frontal lobe gait apraxia and in ataxia from midline cerebellar lesions and


Neurology_Adams

Neurology_Adams. the legs that disappears when the patient is seated is, of course, not unique to hysteria; it also occurs in so-called frontal lobe gait apraxia and in ataxia from midline cerebellar lesions and in hydrocephalus.


Neurology_Adams

Neurology_Adams. In a most remarkable and recalcitrant form of psychogenic movement disorder, maintenance of the limbs in a rigid or dystonic posture for a long time may result in a bed-bound, crippled state with severe flexion pseudocontractures of the limbs. We have seen 1 such case of 18 years’ duration. The tendon reflexes are usually normal if they can be tested, but with hysterical rigidity and muscular contractures, the abdominal and plantar reflexes may be suppressed.


Neurology_Adams

Neurology_Adams. Anesthesia or hypesthesia is almost always inadvertently suggested by the physician’s examination. Seldom is sensory loss a spontaneous complaint, although “numbness” and paresthesias are not uncommon in hysterics. The sensory loss may involve one or more limbs below a sharp line (stocking and glove distribution), or may involve precisely one half of the body, or vibratory sense may be lost over precisely one-half of the skull (a test favored to demonstrate hysterical hemianesthesia). Touch, pain, taste, smell, vision, and hearing may all be affected on that side, which is an anatomic impossibility from a single lesion. Other aspects of psychogenic sensory disturbance are discussed in Chap. 8. The closest syndrome is that produced by a thalamic infarction but this, too, is easily distinguishable from psychogenic hemianesthesia.


Neurology_Adams

Neurology_Adams. The sometimes-stated notion that hysterical paralysis and sensory deficits are more common on the left side is untrue, according to Stone and colleagues (2002a). The features of hysterical tremor and other movement disorders are described in Chap. 4. Emphasized here are the cessation of tremor with distracting tasks—for example, complex finger movement patterns on the side opposite the tremor (such as touching the fourth, second, and fifth fingers in sequence rapidly), or refixation of the eyes on a target, or walking on the outside of the heels. The ability of the examiner to “chase” the tremor to proximal or distal parts of the limb by holding and immobilizing one part is highly characteristic. A fairly dependable sign is worsening of a tremor with loading that is accomplished by placing a heavy object in the patient’s hand (most basal ganglionic and cerebellar tremors are muted by this maneuver).


Neurology_Adams

Neurology_Adams. Some general characteristics of psychogenic movement disorders that ring true to our experiences with patients are summarized in a review by Hinson and Haren; they include a typically acute onset and rapid progression of the movements, distractibility, variability and the simultaneous occurrence of various abnormal movements and of unexplainable paralysis, sensory loss, or pain. Needless to say the movements are not explainable by conventional characteristics of organic brain diseases, but as with all forms of hysteria, it is not on this feature alone that the diagnosis can rest. They point out, paradoxically, that an associated depressive or anxiety disorder is a good prognostic aspect. Hysterical Blindness (See Also Chap. 12)


Neurology_Adams

Neurology_Adams. This dramatic event may affect one or both eyes and may be coupled with hemiparesis or appear in isolation. The symptoms usually develop suddenly, often after an altercation or other emotionally charged event. The patient stares straight ahead blandly when undisturbed, but may squint or move the head as if straining to see when asked to view an object. Some such individuals can reduce reflexive blinking in response to a visual threat. The psychic nature of the problem may be recognized by a nurse who observes the patient reaching for a cup or for the phone. The preservation of vision is confirmed by the presence of normal pupillary reflexes and of optokinetic nystagmus, although one occasionally encounters a patient who has learned to suppress the latter response as well. A mirror passed slowly in the patient’s central vision often engages eye movements. Other similar maneuvers are favored by different examiners. The presence of visual evoked responses also confirms the intactness of


Neurology_Adams

Neurology_Adams. slowly in the patient’s central vision often engages eye movements. Other similar maneuvers are favored by different examiners. The presence of visual evoked responses also confirms the intactness of retinooccipital connections. The patient expresses little concern about the condition, which is usually short-lived. Cortical blindness and variants of the Balint syndrome are the main diagnostic considerations (see Chap. 21).


Neurology_Adams

Neurology_Adams. Convergence spasm, occurring as an isolated phenomenon, is practically always of hysterical nature. A related phenomenon involves the self-administration of mydriatic eyedrops by healthcare personnel. The patient arrives on the emergency ward complaining of reduced vision (expected) or with headache and claiming to have an intracranial mass. This behavior is perhaps more sociopathic (or malingering) than hysterical.


Neurology_Adams

Neurology_Adams. Patients brought to a hospital in a state of amnesia, not knowing their own identity, are usually hysterical females or sociopathic males involved in a crime. Usually, after a few hours or days, with encouragement, they divulge their life history. Epileptic patients or victims of a concussion, transient global amnesia, or acute confusional psychosis do not come to a hospital asking for help in establishing their identity. Moreover, the complete loss of memory for all previous life experiences by patients who are otherwise able to comport themselves normally is not observed in any other condition.


Neurology_Adams

Neurology_Adams. In the Ganser syndrome (amnesia, disturbance of consciousness, and hallucinations) patients pretend to have lost their memory or to have become insane. They may act in an absurd manner, simulating the way they believe that a deranged or demented person would act, and give senseless or only approximate answers to every question asked of them (calling the color red blue or answering 5 for 2 + 2). the Chapter)


Neurology_Adams

Neurology_Adams. As stated earlier, symptoms of the same nature as those in hysteria occur in men, most often in those trying to avoid legal difficulties or military service or attempting to obtain disability or compensation following injury. Sociopaths often present with this type of illness. Unless such a motivating factor can be identified, the diagnosis of hysteria in the male should be made with caution. In compensation neurosis, as in the classic form of hysteria, multiple symptoms are reported; many of the symptoms are the same as those listed under female hysteria. Or the patient may be monosymptomatic (e.g., “seizures”) and the symptoms, particularly chronic pain, may be confined to the neck, head, arm, or low back. The description of symptoms tends to be lengthy and circumstantial, and the patient fails to give details that are necessary for diagnosis. A tangible gain from the illness may be discovered by simple questioning. This is usually in the form of monetary compensation, which,


Neurology_Adams

Neurology_Adams. patient fails to give details that are necessary for diagnosis. A tangible gain from the illness may be discovered by simple questioning. This is usually in the form of monetary compensation, which, surprisingly, is sometimes less than that which the patient could earn if he returned to work. Most such patients are actively engaged in litigation when first seen. Another interesting feature is the frequency with which the patient expresses extreme dissatisfaction with the medical care given him; he is often hostile toward the physicians and nurses. Many of these patients have already been subjected to an excessive number of hospitalizations and rather dramatic mishaps have allegedly occurred in carrying out diagnostic and therapeutic procedures. The majority of these patients were previously suspected of malingering.


Neurology_Adams

Neurology_Adams. Curiously, women who suffer injury at work or are involved in auto accidents may exhibit the same symptoms and signs of compensation neurosis as men, but in our experience, do so infrequently or at least less overtly.


Neurology_Adams

Neurology_Adams. Psychoanalytic theory, which held that both conversion and dissociative symptoms were based on particular psychodynamic mechanisms, is impossible to affirm or refute. Although subject to some question of fabricated recall, we have been impressed at the high rate of childhood sexual abuse reported by women with severe monosymptomatic cases of hysteria or fugue states. This conforms to some extent with psychoanalytic views. An acknowledged history of childhood abuse related by the patient alerts the physician to the possibility of hysteria. Sociologic and educational factors may be important, for it has been observed that hysterical women as a group tend to be less intelligent and less educated than nonhysterical women but there are many exceptions. A genetic causation must also be considered since family studies have disclosed that approximately 20 percent of first-degree relatives of female hysterics have the same illness, an incidence 10 times that in the general population. This


Neurology_Adams

Neurology_Adams. since family studies have disclosed that approximately 20 percent of first-degree relatives of female hysterics have the same illness, an incidence 10 times that in the general population. This supports, in some views, the idea that hysteria is a disease and not merely a surfacing of a basic personality disorder (see Goodwin and Guze).


Neurology_Adams

Neurology_Adams. Pathophysiology of Hysteria Whether conversion symptoms are consciously produced by the patient or arise unconsciously, without the patient’s awareness, is a question that has been debated endlessly without resolution. Babinski attributed the symptoms to hypersuggestibility. In fact, he defined hysteria as an illness whose symptoms could be induced (and removed) by suggestion. There is strong evidence to support this idea, as most patients can be readily hypnotized and their symptoms temporarily eliminated by this procedure or by an interview and examination under the influence of midazolam. The present authors place great credence on this notion of hypersuggestibility, in keeping with older studies that emphasized these patients’ unusual susceptibility to hypnosis and suggestion. Fascinating in this regard were the observations of Charcot’s students that on their wards, the patients’ symptoms disappeared when he died.


Neurology_Adams

Neurology_Adams. Some insights can be obtained from studies of functional imaging in hysterical paralysis (see also Chap. 3). In general, the contralateral prefrontal cortex is suppressed when the patient with hysteria attempts to move a limb, suggesting to Spence and colleagues a “choice” of an active attempt not to move the limb. The pattern of activation was quite different from volunteers who purposefully feigned paralysis and who did not demonstrate such reduced prefrontal activity. When the hysteric with unilateral sensory loss is stimulated on the affected limb, there is no activation of the contralateral sensory cortex but bilateral stimulation results in activation of the appropriate regions in both hemispheres (Ghaffar et al). Some of the recent findings using functional imaging in hysterical states are reviewed by Carson and colleagues.


Neurology_Adams

Neurology_Adams. As pointed out by Carothers and by Guze and colleagues, hysteria and sociopathy may be closely related. Hysteria is a disease of women and sociopathy mainly of men. As restated by Cloninger and colleagues (1975), they may constitute expressions of a single underlying variable. This relationship is also supported by family studies. First-degree male relatives of hysterical women have an increased incidence of sociopathy and alcoholism; among first-degree female relatives of convicted male felons, there is an increased prevalence of hysteria. Moreover, careful histories of sociopathic girls reveal that many of them develop the full syndrome of hysteria. Women felons often present a mixed picture of hysteria and sociopathy, according to Cloninger and Guze.


Neurology_Adams

Neurology_Adams. The characteristic age of onset; the longitudinal history of recurrent multiple complaints as outlined earlier; the attitude of the patient and the manner of presenting her symptoms; the incongruity of affect and clinical state; the discrepancy between the neurologic deficit and the signs on examination; the impossibility of explaining the patient’s signs on an anatomic or physiologic basis; and the absence of symptoms and signs of other medical and surgical disease will permit an accurate diagnosis in the majority of cases. Certain tests designed to reveal normal functioning of a limb, of vision, and of gait already have been mentioned. Stone and colleagues (2009) assessed over 1000 patients who were given the diagnosis of a functional disorder and reported that only 0.4 percent were later found to have an organic explanation for their symptoms.


Neurology_Adams

Neurology_Adams. There is a significant overlap of hysteria and other medical and neurologic diseases. On record are numerous studies in which patients with an initial diagnosis of hysteria by general physicians were followed for many years. Up to one-third of them (far less in most series) turned out eventually to have an “organic condition” that, in retrospect, explained the initial symptoms (Couprie et al). This emphasizes that the original clinical diagnosis of hysteria is sometimes erroneous, although numerous other surveys emphasize the opposite, as noted below. When the diagnostic criteria in these cases are closely analyzed, it becomes apparent that the diagnosis was made solely by the “discrepancy method”—that is, the patient’s symptoms or signs were not deemed to be credible manifestations of disease, based mainly on the clinical experience of the examiner. Of course, this assumes that the examiner has a wide experience; unfortunately for the novice, many syndromes are unknown or


Neurology_Adams

Neurology_Adams. of disease, based mainly on the clinical experience of the examiner. Of course, this assumes that the examiner has a wide experience; unfortunately for the novice, many syndromes are unknown or incomprehensible.


Neurology_Adams

Neurology_Adams. However, when diagnosis is based on the totality of the clinical picture and not on the “discrepancy method,” it can be quite accurate. The physician can be further reassured that in followup studies of patients with so-called conversion disorder (exclusive of pseudoseizures), virtually none develops a neurologic lesion that in retrospect was related to the initial episode as, for example, in the study by Stone and colleagues (2003). It is of interest that in the series cited, most patients had persistent functional disability from their conversion symptoms, even a decade later.


Neurology_Adams

Neurology_Adams. Projective tests (the Rorschach and Thematic Apperception Tests), which for a time were popular with dynamic psychiatrists, are apparently not helpful in diagnosis and are now used very little. The presence of extreme suggestibility and the tendency to dramatize symptoms as measured by one part of the Minnesota Multiphasic Personality Inventory and other psychometric tests is helpful in diagnosis but not pathognomonic; these traits appear under certain conditions in individuals who never develop hysteria. Finally, it should be emphasized that single bouts of isolated hysterical paresis, blindness, and anesthesia are quite common in neurologic practice and do not presage a chronic hysterical illness. The same is true for transient neurologic signs exhibited during the course of the examination, mainly pertaining to unusual or drifting sensory loss or asthenic weakness of a limb. Treatment of Hysteria and Hysterical Symptoms


Neurology_Adams

Neurology_Adams. Here, opinions differ but modern approaches that, as mentioned, are non judgmental, straightforward and aimed at returning the patient to good function have great appeal. Treatment may be considered from 2 aspects: the amelioration of the long-standing basic personality defect and relieving the recently acquired physical symptoms. Little or nothing can be done about the former. Psychotherapists have attempted to modify it by long-term reeducation, but their results are uninterpretable and there are no control studies for the few reports of therapeutic success. Many psychiatrists are inclined to regard the female with hysteria who has a lifelong history of ill health as having a severe personality disorder—that is, sociopathy. In other less severe cases and especially in those in whom hysterical symptoms have appeared under the pressure of a major crisis, explanatory and supportive psychotherapy appears to be helpful, and the patients have been able thereafter to resume their places in


Neurology_Adams

Neurology_Adams. symptoms have appeared under the pressure of a major crisis, explanatory and supportive psychotherapy appears to be helpful, and the patients have been able thereafter to resume their places in society.


Neurology_Adams

Neurology_Adams. The acute symptoms can usually be mitigated by persuasion and demonstration. A current approach is to explain the nature of the illness and point out how common it is, and offer support to reverse the main features. Reassurance that recovery is possible and that the patient seems to be in the process of recovering may be useful. Stone and Edwards, who have thought considerably about this subject, have suggested showing the patient objective evidence of the functional nature of weakness, such as Hoover sign, by way of demonstrating the correctness of the diagnosis and demonstrating its potential reversibility. The earlier this is done after the development of symptoms, the more likely they are to be relieved. Sometimes a single symptom such as hemiparesis or tremor can be halted by a particular maneuver and this demonstration suffices to begin recovery. In chronically bedridden patients, pressure to get out of bed and resume function must be applied.


Neurology_Adams

Neurology_Adams. Several approaches to discussing the symptomatology with the patient have been suggested. At one extreme is a confrontative approach in which the patient is told the symptoms are psychologic, or “in your head.” We have found this to be counterproductive and almost always provokes an angry response that does not aid clinical improvement. At the other extreme is complete avoidance on the part of the physician, an approach that is almost as unproductive. We prefer to ask the patient if the symptoms can in some way be the result of “stress” or an upsetting recent experience. On occasion, in private, we will inquire about childhood sexual abuse and often get an affirmative response from the patient, with later confirmation by a spouse or sibling. Very powerful is nonjudgmental but firm reassurance that there is no serious disease. We have found it useful to list the diseases that have been excluded by examination and testing: brain tumor, stroke, amyotrophic lateral sclerosis, multiple


Neurology_Adams

Neurology_Adams. that there is no serious disease. We have found it useful to list the diseases that have been excluded by examination and testing: brain tumor, stroke, amyotrophic lateral sclerosis, multiple sclerosis, etc. This often evokes an acknowledgment by the patient that one of the diseases had been a preoccupying concern. We then indicate, without using psychologic terms, that the brain may at times adopt certain patterns of behavior that do not reflect structural damage, and, furthermore, that these patterns can be unlearned with physical therapy and time, as described in the following text.


Neurology_Adams

Neurology_Adams. That the patient’s response to these conversations varies widely is not surprising. One group seems not to mind and to be relieved by the expression of concern and reassurance that there is no dangerous disease at the root of the problem. They can be sent to a physical therapist and may do well in the short run. Another group is indignant and unlikely to consult the physician again; several in the past have refused to pay the doctor’s bill. Some have objected to the explanation based on their own view, often derived from research on the Internet and with similarly afflicted persons, that Lyme disease, chronic viral infection, environmental toxins, allergies, etc., are to blame. A few of these cases have the flavor of a delusion. All that the physician can offer here is an openness to see and reexamine the patient in several months; “cure” has no meaning in these instances and there is a high likelihood that such individuals will see a long line of doctors.


Neurology_Adams

Neurology_Adams. Some of our better results have been obtained by indicating that the neurologic symptoms are a “pattern of brain circuits” or “constitutional” weakness that can be overcome by physical and other therapies. Once the current neurologic disorder has disappeared, it may be helpful to counsel the patient in ways to prevent its recurrence. Family members can be given the same explanation. A regimen of physical therapy should be instituted, using an experienced therapist and setting simple goals for success. Every subsequent illness in such patients should be evaluated objectively, so as not to overlook any medical or surgical disease, which may strike a hysterical patient just as it does any other person.


Neurology_Adams

Neurology_Adams. The success of any of these programs over a long period is unknown. The eradication of recently acquired hysterical symptom is relatively easy. The real test is whether it enables the patient to adjust satisfactorily to family and society and to perform daily activities effectively, and whether it prevents addiction, unnecessary medical treatments, and operations. Estimates of the recurrence rate of hysterical symptoms vary widely from 12 to 80 percent. In the series reported by Gatfield and Guze and by Merskey, the recurrence of somatic symptoms of similar or of other types was as high as in sociopathies. We have seen a few patients with monosymptomatic hysteria (paraparesis, bizarre gait, crippling dystonia) that persisted for years on end regardless of treatment. The use of a wheelchair for more than several days has been a bad prognostic sign in these cases.


Neurology_Adams

Neurology_Adams. This is the preoccupation with bodily functions or physical signs and sensations, leading to the fear or belief of having serious disease. Hallmarks of this condition are the failure of repeated examinations to disclose any physical basis for the patient’s symptoms and the failure of reassurance to affect either the patient’s symptoms or his conviction of being sick. It is estimated that 85 percent of hypochondriasis is secondary to other mental disorders, chiefly depression, but also schizophrenia and anxiety neuroses. In approximately 15 percent of cases, however, there appears to be no associated illness (primary hypochondriasis). Most patients in this latter category are habitués of medical outpatient clinics, who are passed from specialist to specialist, perplexing and angering doctors along the way, because their symptoms defy both satisfactory diagnosis and cure.


Neurology_Adams

Neurology_Adams. Related to hypochondriasis, but probably more delusional are young adults who present with a fixed somatic belief regarding a peculiar symptom such as that the tongue is swollen, the jaw is not properly aligned, or the penis is ulcerated, when in fact no such abnormalities are present. The troubling aspect to the family and physician of such an illness is the persistence of the symptom and disability that extends for years, all tests having been negative. Probably these patients should be treated like schizophrenics, which many of them probably are. What to do with patients who are less severely affected but who have an unshakable belief that they have Lyme disease or environmental “allergies” depends on the context, but the likelihood of dissuasion is almost as poor as for the worst hypochondriac patients.


Neurology_Adams

Neurology_Adams. The treatment of primary hypochondriasis is difficult unless the physician keeps in mind the personality of the patient and the therapeutic goals. A psychodynamic outlook would suggest that these patients need to retain their symptoms, so that the usual concept of “curing” is inapplicable. The presence of symptoms provides the context for a relationship with a physician and it is the continuation of this relationship, which is often the only dependable contact in the patient’s life, which is the motivation for some hypochondriac patients. Such patients are best managed by general physicians who realize that these are patients who do not necessarily want or expect a cure, and who are content with small gains and the avoidance of unnecessary surgery.


Neurology_Adams

Neurology_Adams. Of all the abnormal personality types listed in Table 47-1, the antisocial is the best defined and the one most likely to cause trouble in the family and community. It had been defined (in DSM) very well as a state in which the individual “is always in trouble, profiting not from experience or punishment, unable to empathize with family or friends or to maintain loyalties to any person, group, or code. He is likely to be shallow, callous, and hedonistic, showing marked emotional immaturity with lack of sense of responsibility, lack of judgment, and an ability to rationalize his behavior so that it appears warranted, reasonable and justified.”


Neurology_Adams

Neurology_Adams. Since Prichard, in 1835, first described this condition under the term immoral insanity, there have been many attempts to give it a more precise definition and to avoid using it as a psychiatric wastebasket. At the turn of the century, Koch introduced the term psychopathic inferiority, implying that it was a constitutionally determined deviation in personality. Later the term psychopathic personality came into common use. In the past, many authors used this last term indiscriminately to embrace all forms of deviant personality. Subsequently, the term came to be used in a more restricted sense to define a subgroup of antisocial or aggressive psychopaths (antisocial personality disorder in DSM-IV). Aubrey Lewis has given a lucid account of the history of the concept of sociopathy. By far the best modern study of sociopathy is that of L.N. Robins, based on a 30-year followup study of 524 cases from a child guidance clinic and 100 controls. Other investigations of note are those of


Neurology_Adams

Neurology_Adams. far the best modern study of sociopathy is that of L.N. Robins, based on a 30-year followup study of 524 cases from a child guidance clinic and 100 controls. Other investigations of note are those of Cleckley, of McCord and McCord, and of Guze and coworkers, who studied psychiatric illness in large numbers of felons and their first-degree relatives. More recently, the descriptor, “callous youth” has become part of the syndrome. The following material and the preceding quotation are taken largely from these writings and from those of Reid.


Neurology_Adams

Neurology_Adams. This condition, unlike the majority of psychiatric disorders, is manifest by the age of 12 to 15 years, and frequently earlier. The manifestations of sociopathic behavior in children and adults are 5 to 10 times more frequent in males than they are in females. It consists essentially of deviant behavior in which individuals seem driven to cause difficulty in everything they do or behave in a way that most societies identify as grossly criminal. Codes imposed by family, school, religion, and society are broken. Seemingly, the sociopath acts on impulse, but after committing the unsocial act, he shows no remorse. The most frequent antisocial activities are theft, truancy, running away, associating with undesirable characters, indiscriminate sexual relations, repeated fighting, recklessness and impulsivity, lying without cause, vandalism, abuse of drugs and alcohol, and, later, inability to work steadily or keep a job. Criminality is intimately associated. Fire setting and cruelty to


Neurology_Adams

Neurology_Adams. and impulsivity, lying without cause, vandalism, abuse of drugs and alcohol, and, later, inability to work steadily or keep a job. Criminality is intimately associated. Fire setting and cruelty to animals are particularly associated with future sociopathy according to several authors (the “callous youth” alluded to above). In the study by Robins et al, of children or adolescents who exhibited 10 or more antisocial symptoms, 43 percent were categorized as sociopaths in adulthood. If only 8 or 9 of these traits were present, 29 percent were so grouped; if 6 or 7, 25 percent; and 3 to 5, 15 percent. Conversely, not a single adult sociopath was observed who did not manifest antisocial symptoms in earlier life. Interestingly, a number of other problems of childhood and adolescence—such as enuresis, dirty appearance, sleepwalking, irritability, nail biting, oversensitivity, poor eating habits, nervousness, being withdrawn or seclusive, unhappiness, tics, and fears—were not predictive of


Neurology_Adams

Neurology_Adams. dirty appearance, sleepwalking, irritability, nail biting, oversensitivity, poor eating habits, nervousness, being withdrawn or seclusive, unhappiness, tics, and fears—were not predictive of adult sociopathy. None of Robins’ patients was mentally retarded.


Neurology_Adams

Neurology_Adams. At the same time, it should be noted that more than half the disturbed children in Robins’ study (even those with 10 or more antisocial manifestations) had lost most of their sociopathic traits by adulthood. This does not mean that they remained psychiatrically normal. Of those who did not become adult sociopaths, the large majority developed other adult psychiatric illnesses, particularly addiction to alcohol. Only in the group of children with fewer than 3 antisocial symptoms did a reasonable number (one-third) remain entirely well from a psychiatric point of view in adult life. Because sociopathic behavior in children may terminate spontaneously or evolve into other disorders, it is advised that the diagnosis of antisocial personality disorder be reserved for adults; the same behavior pattern in children is designated as conduct disorder.


Neurology_Adams

Neurology_Adams. Also of interest are Robins’ findings that sociopaths showed an unusually high incidence of “conversion” symptoms (we would substitute “malingering” as discussed further on), as well as depressive symptoms and anxiety, and that these symptoms are in proportion to the sociopathic ones. Among women with sociopathic behavior there was a high incidence of hysterical manifestations—evidence that female hysteria may be the counterpart of male sociopathy. In Robins’ series, a search for evidence of encephalitis, often postulated in the past as the basis of sociopathy, was not revealing, nor was there any proof of other brain damage. In the current era, head trauma is often imputed as the cause of troublesome behavior in adolescents and young adults, but there is no basis for this view.


Neurology_Adams

Neurology_Adams. EEG abnormalities, taking the form of mild to moderate bilateral slowing, are more frequent in criminals and sociopaths than they are in the normal population in some series, but the validity of this finding is uncertain.


Neurology_Adams

Neurology_Adams. Other findings suggest a genetic predisposition to antisocial personality. In a Danish study of criminals by Christiansen, “inappropriate nonpsychotic impulse-ridden behavior” was found 5 times more frequently in first-degree biologic relatives than it was in the general population. Criminality was 2 times more frequent in monozygotic twins than it was in dizygotic twins. His study also confirmed an association between hysteria and sociopathy. More direct evidence of a genetic factor was provided by Cadoret’s study of adoptees who were separated at birth from antisocial biologic parents. A higher incidence of antisocial behavior was present in the adoptees than were in controls. His study also suggested that excessive childhood hyperactivity and classic female hysteria were phenotypic manifestations of an antisocial personality genotype, but this is by no means confirmed.


Neurology_Adams

Neurology_Adams. There is no information as to the best methods of treatment and the role of the medical profession has never been clear. Treatment Most psychiatrists have been discouraged by the results of psychotherapy, but whether behavioral therapy, psychoanalysis, or drugs have more to offer cannot be determined from available data. Medical efforts should be directed to evaluating the patient’s neurologic status, assessing his intelligence, and explaining the nature of the disorder to parents and social agencies, tasks best performed by a psychiatrist.


Neurology_Adams

Neurology_Adams. This problem arises frequently in connection with both hysteria and sociopathy, and the physician should know how to deal with it. The term malingering refers to the conscious and deliberate feigning of illness or disability in order to attain a desired goal. It does not appear as an isolated phenomenon, and its occurrence must be interpreted as a sign of a serious personality disturbance, often one that prevents effective work or military service, as a means of obtaining recompense for an alleged injury, although noteworthy exceptions to this statement can be found.


Neurology_Adams

Neurology_Adams. In the malingerer one may observe pain, hyperesthesia, anesthesia, limping gait, tremor, contracture, paralysis, amaurosis, deafness, stuttering, mutism, amnesia, pseudoconvulsions and fugues, jumping of limbs with touch, and unexplained skin lesions—in short, the same array of symptoms and signs, singly or in combination, as in the patients with hysteria. Certainly there is a similarity between hysteria and malingering, but the nature of the relationship is nebulous and there may be great difficulty in establishing a clinical differentiation. As Jones and Llewellyn have observed:


Neurology_Adams

Neurology_Adams. Nothing…resembles malingering more than hysteria; nothing, hysteria more than malingering. In both alike we are confronted with the same discrepancy between fact and statement, objective sign and subjective symptom—the outward aspect of health seemingly giving the lie to all the alleged functional disabilities. We may examine the hysterical person and the malingerer, using the same tests, and get precisely the same results in one case as the other.


Neurology_Adams

Neurology_Adams. The following have been cited as the main points of difference between the 2 conditions: (1) the conscious or unconscious quality of the motivation, which seems more unconscious in the hysteria patient and more conscious in the malingerer; (2) the influence of persuasion, which is usually effective in hysteria and not in the malingerer; and (3) the attitude of the patient. The patient with hysteria appears more genuinely ill and invites examination; the malingerer seems less ill and evades examination. Most of the more obvious cases of malingering seen by the present authors have been in sociopaths, for which reason discussions of the 2 conditions have been juxtaposed here.


Neurology_Adams

Neurology_Adams. A particular form of sociopathy or malingering, which consists essentially of systematically and specifically deceiving the medical profession, has been described as Munchausen’s syndrome—named (not altogether aptly) after a 17th-century German soldier, Baron von Munchausen, who invented incredible tales of adventure and daring. Ireland and colleagues, who analyzed 59 cases (45 men, 14 women), listed the following characteristic features, which will be recognized at once by all neurologists with extensive hospital experience: feigned severe illness of a dramatic and emergency nature; factitious evidence of disease, surreptitious interference with diagnostic procedures, or self-mutilation; a history of many hospitalizations (sometimes more than 100); extensive travel or visits to innumerable physicians; and, finally, regular departure from the hospital against medical advice. Unlike the usual forms of compensation hysteria, an ulterior motive is not readily discernible and the


Neurology_Adams

Neurology_Adams. physicians; and, finally, regular departure from the hospital against medical advice. Unlike the usual forms of compensation hysteria, an ulterior motive is not readily discernible and the psychopathology of this syndrome is obscure. It has been regarded by various experts as a form of sociopathy, malingering, or compensation hysteria, but the distinctions between them are too ambiguous to be of clinical value. Probably the medical profession has placed too great a reliance on degree of conscious awareness of deception. In such unstable and immature individuals, the terms conscious, unconscious, and deception are too uncertain to be useful.


Neurology_Adams

Neurology_Adams. Designated by this title is an uncommon disorder, the characteristic feature of which is the occurrence of repetitive, unpredictable outbursts of violent, aggressive behavior disproportionate to the provoking situation. This condition needs to be set apart from the uncontrollable outbursts that sometimes are associated with dementia, mental retardation, schizophrenia, drug addiction, or alcoholism, or those that follow serious head injuries or other brain diseases. Neurologic opinions have been solicited on our services for patients afflicted with this condition, the question usually being one of seizures as a cause of the aberrant behavior. Some persons with intermittent explosive disorder have, from early childhood, reacted to frustration with a loss of self-control, striking out in blind rage at anyone who crossed them (episodic dyscontrol syndrome); as adults, they may inflict serious injury or kill. Lesser degrees are recognized as expressions of “hot temper.” Sometimes such


Neurology_Adams

Neurology_Adams. blind rage at anyone who crossed them (episodic dyscontrol syndrome); as adults, they may inflict serious injury or kill. Lesser degrees are recognized as expressions of “hot temper.” Sometimes such behavior appears to be a continuation of the temper tantrums of earlier childhood. What is surprising in some of our patients has been a discrepancy between this episodic behavior and a pleasant and concerned demeanor at other times. Such patients are aware of the inappropriate nature of their behavior and its impact on others; they express remorse and may seek medical assistance to mute the outbursts. Others, of course, have no such insight and their anger episodes are simply an extension of their sociopathy.


Neurology_Adams

Neurology_Adams. The causes of aggressive violence are poorly understood. There appears to be a heritable tendency (Cadoret et al, 1997); males predominate and a sex-linked form extending over several generations has been described. Polymorphisms of the androgen receptor have been implicated in several preliminary studies. In only a very small number of patients, a seizure disorder can be identified, particularly temporal lobe epilepsy, but the majority still appear to be constitutional in nature. A state of adrenergic hyperactivity has been suggested and supported to some extent by these patients’ response to propranolol but this is not explanatory of the behavior (Elliott; Jenkins and Maruta; also see Chap. 25’s discussion of the limbic system). Most instances probably represent a variant of sociopathy. Intense outbursts of anger and physical violence are also features of the diagnostic category of borderline personality disorder, the other manifestations of which include “a pervasive pattern of