Cerebral Palsy in Down's Syndrome Children: Another Two Cases

Chiara Crivelli, via M. Della Torre 37, 37031 Verona, Italy; Anna Donati; Renato Cocchi.
CIFFRE, Villa Renzi, S. Vito di Negrar, Verona, Italy
Italian Journal of Intellective Impairment 11 (1): 31-37 (1998 Jun)
  Reprinted with the permission of Renato Cocchi
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In a non-selected consecutive series of 551 home reared Down persons coming from all the Italian regions, only 5 of them presented CP. As for the origin of them, in 3 Ss CPs were surely postnatal, in another S it was almost surely the same, and the last CP cannot be decided if postnatal or other. Having previously reported the first three cases (Cocchi R. Riv. It. Disturbo Intellet. 1990, 3: 327-330), the two new cases are extensively described.
The prevalence of CP in this cohort is nearly four times higher than in NT children, but the prevalence of postnatal CPs is absolutely an extraordinary figure. Although the occurrence of CP following pre-, peri- and neo-natal insults did not have been denied, the present epidemiological data seem even more supporting the hypothesis that Down Ss are protected during the foetal age, at birth and in first days of life from the paralytic outcomes of anoxic-ischaemic damages.

Key words: Down' syndrome; cerebral palsy; epidemiological survey; 2 cases.

In a previous paper one of us dealt with the link between Cerebral Palsy (CP) and Down's Syndrome (DS) by complaining that such a link had not had the whole attention that it deserved (Cocchi 1990).
He suggested that a deeper study in this field would be able "to make more light on some mechanisms and results of the anoxic-ischemic damage due to pre-, peri-, neo- and postnatal troubles and a possible outcome in CP."
In first epidemiological research Cocchi (1987) noticed that 381 home-reared Downs did not include any case of CP due to prematurity, low birth weight or both, the two major risks factors for this motor disability. Indeed, no CP due to pre-, peri-, or neonatal factors was found there, despite Downs have higher incidence of these troubles, to which is attributed at least 75% of CP causes (Susser et al., 1985).
In a subsequent research on CP and squint (Cocchi and Branchesi, 1988) in 424 Down subjects - the preceding 381 + 43 new cases -, again no CP was found, other than three of postnatal origin. Only for the prevalence of the two major risk factors above mentioned, we would have found at least three cases more.
Cocchi (1990) reported these first three cases.
Now, eight years after, we can add another two cases largely described, giving a total of five cases within an non selected consecutive cohort of 551 Downs.
These two new cases came out from the Down population followed by CIFFRE, which as an organization specially involved in motor rehabilitation could more easily have encountered children with such a condition.

Description of two cases
Case 1: Male, born in 1989 with standard trisomy 21. During pregnancy the mother suffered by colic pain with diarrhea, and vomiting throughout all over the time. She smoked 5/10 cigarettes per day. The fetus was very small.
At 36 weeks of pregnancy first contractions occurred and there was an attempt to stop labor. After two days hemorrhage occurred and she went through with delivery with a spontaneous breakup of the membranes, liquid dyed by the meconium. The placenta was small and with many infarcts. Birth weight of the baby was [?]. The first cry delayed some minutes, after aspiration, with slight cyanosis. A cardiopathy was discovered, a perimembranous IVD. Hospitalized in the incubator and then in isolette lasted two and half months.
The baby had maternal milk by tube feeding for 40 days, then bottle feeding with formula. His weight at discharge was 3500g. He needed digitalis therapy since first days of life. At the first month of age, a type of cyanosis attack (an epileptic fit?) occurred, treated with unreported therapy. During the hospitalization the child presented a gastrointestinal infection and bronchopneumonia treated with antibiotics.
In April 90 he had a second attack (an epileptic fit?); after staring and then revolving eyes, the child was rigid. This episode lasted five minutes; no therapy prescribed.
Heart surgery was acted in June 90 (at one year of age) for the congenital cardiopathy.
In November 90 it begins a progressive fall of hairs and then eyebrows and eyelashes until to complete alopecia.
The initial evaluation by CIFRRE in February 1991 (1 year and 8 months) as follows. He has good environment awareness. He presents saccadic eyes movements, localizes sounds in the environment, understands sentences, uses some words. The child can maintain (but he doesn't reach by himself) the sitting position with balance reactions, although he has much of the fear to sit alone on the floor. Babinski reflex evoked in both feet. He crawled on stomach with reciprocal movements (a cross pattern) and fair coordination. He did not maintain standing position. He grabs things with a hand or the other, and doesn't use two hands together; he has not reached cortical opposition yet. The grasping in left hand seemed better, but we notice that his mother is left-handed.
The CIFRRE team proposed a rehabilitation program involving sensory-motor and intellectual stimulation and suggested a more healthy and balanced diet.
During the first year of treatment the child got movement on all four done with a fair pattern and good coordination without underlining side differences. He reached the sitting position and standing with support, besides passages of position from on all four to kneeling position and then to standing position (with anterior support during the entire process).
The child used many words and he loved to repeat words that he did not know. Hand skills evolved and cortical opposition began but always better on the left.
Finally he used two hands to put together and pulled apart and in the attempt to unscrew: left hand was always in a prevailing position. The Babinski reflex became negative in the left side and remained positive in the right. In this period eyelashes and eyebrows showed some growth.
During the second year the motor evolution marked the footstep. Even if he could maintain the standing position for a while without support, the child was afraid to let go.
He could walk pushing the stroller, showing rigid extension of legs and the prevailing support on the left foot. He always asked for bilateral support to walk (Always both hands and not only one). In walking the right arm tended to be more rigid than the left one. The language evolution went on and the child communicates by using brief sentences with improving pronunciation.
He was using both hands together in simple games, but he did not get hands' independence from each other. Left hand was always preferred.
Overall, the child started to reveal a remarkable side difference that did not previously emerge.
During the second year of treatment he added the use of NTM - Neurotransmitter Modulator (Donati and Crivelli 1997). In the same period we observed a new growth to hair spots in different head points, but not in a stable way. In fact hair could fall after a growth period, and start over growing in another place of the head.
Only during the third year of treatment (four years of age) the child got autonomous walking but markedly in a hemiplegic way. With a larger feet' base, he was completing the footstep with his left foot, while the right one was reaching the left foot without overcoming it. He presented dexteral genus recurvatum, the right foot was in varus and supination, the right arm had no swinging and slight flexing of the elbow.
Fear in walking remained for a long time, also after he had gotten independent walking. The child did not walk alone in wide areas, did not go up and down stairs alone, even hanging to the handrail.
In September 93 (4 years 3 months) he started going to the kindergarten. Some difficulties emerged as for the relationship with the teacher, which was increasing during the following three years. The child felt uneasy this experience, since the teacher did not trust his cognitive potential and did not propose suitable activities.
Overall it was a stasis period. He did not succeed in overcoming his fears, had difficulties in the relationship with classmates, said often: I am not able to do anything.
In the same period the NTM use stopped. The growth of the hair continued in spots, with periods of fall and periods of growing again.
In the spring 95 (5 years and 9 months) he began drug therapy according to guidelines already stated by one of us (Cocchi, 1993). After one year he had full hairs' regrowth without the fall anymore. Gradually the fear in walking regressed. The use of the NTM started again.
Volunteers carried on a cognitive program at home with great success. In fact, in one year, before entry in elementary school, the child could recognize and to name all the letters of the alphabet; he read some syllables and simple words, and learned simple time and space ideas. He developed a richer language and better structured as for the syntax.
Currently (8 years and 9 months of age) the child has overcome all fears in movement. His walking has not modified in substantial way but generally motor functions are satisfactory: He runs, attempts to jump, goes in the bicycle with training wheels.
A moderate spasticity of the right gastrocnemious is underlined: Its ankle poorly overcomes 90 degrees when forced stretching is set up.
From September 1996 he is attending the elementary school and has begun to write in printed capital, being left-handed with poor handwriting.
Teachers planned a computer's help for writing. The teachers have well included the child in the classroom favouring his relationships between classmates. Child's learning levels are good. The language has had significant evolution. Verbalization is semantically and syntactically correct, and the complexity of the sentences produced is suitable to the class frequented (the 2nd elementary grade).
He has difficulties in the articulation of some phonemes. The activities related to autonomy (to undress and dress, to wash himself, etc.) does not run in satisfactory way because of both hemiplegia and substituting family attitude.

Case 2: Male, born in 83 and affected by trisomy 21. The mother's pregnancy lasted with regular controls but the obstetrician planned the C-Section due to a narrow pelvis.
C-Section was performed at the 38th week. Baby's Apgar Rate scored seven at one minute and eight at five minutes. He had birth weight of 2560g so he was placed in the isolette for two days.
Artificial feeding was set up, with slow suction. Ha was a carrier of a cardiopathy due to an interventricular defect (IVD). At 6 months of age he was suspected of an underdeveloped thyme; an immunologist suggested not intervening for the moment. A chronic therapy with gammaglobulins should be considered in the future.
At 9 months he had a right sided convulsion: it was considered a fever convulsion and administered diazepam. The child has had a light right paresis as the result of it. After two days again a right sided convulsion came out. The diagnosis was viral encephalitis from herpes simplex treated with sedatives, antivirals, antibiotics and immunoglobulins for the precedent suspicion of an immunological lack. Discharged with a right hemiplegia outcome, he had therapy with phenobarbital.
In the following the child showed a progressive organic deterioration (loss of weight, profound apathy) that lasted some months and from which he recovered very slowly.
The IVD closed by itself during the first two years of life.
The CIFRRE team carried out an initial evaluation in May 84 (1 year and 4 months). Alternate convergent squint (with prevalence of right squint) was found with poor ocular motion. The child perceived and localized sounds in the environment, but he did not answer to his name. He showed poor tactile perception, both exteroceptive and nociceptive, also poor propriception. The prehensile grasp was present in both hands but dysmetric on the right. He produced some vocalic sounds.
The child could roll from supine to prone, but he had poor head and trunk control and could not maintain the sitting position. On basic hypotonus, right limbs could show hypertonus especially to passive movement.
The CIFFRE team proposed a rehabilitation program with particular attention to sensory integration, with tactile and propriceptive stimuli, visual exercises, and a healthy diet with vitamins and mineral salts supplements.
During the first year of treatment the child greatly improved the tactile perception. His environment awareness showed a great improvement: He was understanding small orders, he began using sounds, he recognized images and complex symbols.
The alternate squint did not change. He improved grasping and started to pass objects from a hand to the other.
The child began to move crawling on the stomach with poor coordination, reached all four position. He moved forward in such position with poor coordination, pulled himself to stand with anterior support, and began a side walk with an anterior support.
During the second year of treatment the child continued improving sensory functions and understanding, used sounds or syllables (with the meaning of words), started some two-hands functions (pulling apart, unscrewing), used the spoon. He got coordination in crawling and creeping on all four, up standing, and walking with support.
He reached independent walking when he was near four, with a wider base, his footstep was with the left foot then parallelled by the right one. His right leg tended to extrarotation and at times he threw the side right foot. The child had the right arm slightly flexed to the elbow and tended to be more rigid, without swinging; right shoulder was lower than the left. During these years language improved. He was repeating many words with poor pronunciation, but he was unable to put together two words.
The child recognized different images and words, some times pronouncing them, some times pointing out to them.
He can eat alone with spoon and fork, but was unable to undress or to dress, and used both hands to manipulate, to throw the ball, etc.
He began a partial stay in the kindergarten in September 86 (3 years and 8 months) with good integration. His attendance, subsequently full time, continued for four years.
In the beginning of 1987 the use of the NTM - Neurotransmitter Modulator was introduced (Donati and Crivelli, 1997). In these years a moderate evolution of cognitive abilities took place.
The child was stimulated to use the left hand in graphic activities with poor results. For about one year a specific activity for the right hand was proposed, that involved also the attempt of use for graphic activities but often in such assignment he helped himself with left hand.
His walking became more sure; the right foot did not reach the left one anymore but overcame it. On the contrary, right arm tended to be always rigid along the body, without swinging. Motor coordination continued improving.
In September 90 he started the elementary school. After the first year, quite disappointing, the child had a very skilled support teacher who provided a diversified learning program.
Lots of tests showed his real cognitive abilities, at such a level nobody would expect according to his limited expressive abilities.
In November 92 the child began a drug therapy following the guidelines again expressed by one of us (Cocchi, 1993). During 93, the graphic abilities improved to the point that he wrote words and small sentences spontaneously in printed capitals.
He drew the human body and he was able to organize the graphic space on the sheet and on the blackboard. He used left hand, but sometimes he used the right hand to drive the left.
The language evolved and the child started two words' sentences, and increased new spontaneous words.
Currently (15 years) he walks with good balance always underlining a limp on the right side, his right arm is less rigid along the trunk, but it has not reached true swinging.
The right hand is used spontaneously in many situations, alone or with the left (to launch and to take the ball, to bring an object, to put together, to pull apart, to unscrew, to pull up pants, etc.).
It must be underlined that the boy has reached an awareness of his own body and the ability to manage his motion to reach his purposes despite the limitations due both to hemiplegia and DS.
Personal autonomies are fair, even if not yet completed. Moreover, the boy helps in preparing the table, and in other simple home activities. He communicates with many words, with couples of words and some longer sentences.

First we need to say that a research on last 10 years of MEDLINE and PsychINFO, and on the last 12 years of Developmental Medicine and Child Neurology did not get at new papers on this topic.
As for the two further cases here reported, both with dexteral hemiparesis, the causal factor acted in postnatal time in case two, but cannot be decided when, in case one.
These two cases too were from a non-selected consecutive series of 551 home reared Down subjects, and with the first three cases already reported (Cocchi, 1990), show a CP prevalence = 0.90%.
In the Table 1 we summarize some features of all these five cases.

Table 1: Summary of some data referring to 5 CP out of 551 Down Ss.
Case no. Sex CP type Origen Where reported
1 f dx hemiparesis postnatal Cocchi 1990
2 f dx hemiparesis quite surely postnatal Cocchi 1990
3 m sx hemiparesis postnatal Cocchi 1990
4 m dx hemiparesis not to be decided Crivelli et al., 1998
5 m dx hemiparesis postnatal Crivelli et al., 1998

It is worthy to note that we have here five hamiparesis, four of which on the right side.
A 90% prevalence is about 4 times more than that found by Kundriavcev et al., 1985. In a statistic referred to a series of about 28.000 [normal] live borne babies in the Rochester area (USA), they found 64 CP with an .23% prevalence
In Finland, the prevalence of CP in DS has found 5.1% out of the DS inpatients of a specialized institute (Iivanainen and Hongell, 1986). Even if the Finnish sample could not be considered as representative, since it comes out from a population of hospitalized DS, however it states for an increased prevalence of CP in DS.
In Italy, out of 70 cases seen during 10 years in a specialized hospital division, Bertamino (1984) has found 11.4% "signs of damage at the neurological examination including "spasticity, etc." Also n this paper, although the sample has very debatable as for representability, and CPs did not have a specific mention, we cannot deny a trend of an increased CP prevalence in DS.
In the epidemiological research made by Kundriavcev et al., 1985, there is however another element of notable interest. The diagnosis of CP was made within 16th day from birth, and therefore all the 64 CPs had their causes in troubles occurred in pre- peri- and neonatal periods.
Taking in consideration that, according to Susser et al., 1985, CPs postnatal origin cannot be more of the 5-10% of all CPs, what we found is remarkably anomalous, if compared with non Down subjects.
In facts, it is anomalous for two reasons: The already noticed prevalence, nearly four times increased, but overall the rate of postanatal origin (three sure cases out of five, one nearly the same but we cannot decide about the last one).
If we agree with Susser et al., 1985, given our four cases = 10%, we would have at least others 30 CP from pre-, peri- and neonatal troubles, as responsible troubles of 75% CPs in non Down subjects.
If evidence of the case two at 8 months refers to a non-postnatal CP, among the first three cases already reported, three cases out of five with sure and documented postnatal cause are anyhow too many. We would have found at least other 22 CPs from pre-, peri- and neonatal factors.
We did not get these figures, although an increased prevalence of prematurity, low birth weight or both on our sample (Cocchi and Branchesi, 1988). Being these troubles claimed as the major risk factors for the CP outcome, it leads us to suppose that compensating mechanisms are set up (Cocchi, 1987; Cocchi et al., 1988).
As one of us supposed in the first paper he devoted to this matter, there is therefore an undeniable excess of CP of postnatal origin in our Downs subject. This could be explained through greater capacity of the Down fetus and of Down newborn to oppose negativing effects of anoxia and consequent toxic action of oxygen's free radicals.
This increased protection, since from first months of life, should be cut down or lost, for different reasons (Cocchi, 1990). If subsequently confirmed, we should have found a datum that could give more knowledge both on Down's syndrome and on causal mechanisms of CP in non Down children.

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