CEREBRAL PALSY IN DOWN'S SYNDROME CHILDREN:
ANOTHER TWO CASES.
Chiara CRIVELLI (*) Anna
DONATI (**) Renato COCCHI (**)
(*) Rehabilitation therapist and
psychopedagigist; (**) Psychologist;
(***) Neurologist, CIFFRE consultant for drug therapies
CIFFRE, via D'Azeglio, 2 -Verona
Summary
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 llight 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 syntaxs.
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.
Discussion
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 nr. |
Sex |
CP type |
Origin |
Where reported |
|
|
|
|
|
|
|
|
|
1 |
f |
dx hemiparesis |
postnatal |
Cocchi 1990 |
|
|
2 |
f |
dx hemiparesis |
quite surely postnatal |
Cocchi 1990 |
|
|
3 |
m |
sn 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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Author’s
address: dr Chiara CRIVELLI, via M. Della Torre 37
37031 VERONA (Italy) / email: chia.sci@iol.it