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Clinical Spectrum of Cerebral Palsy in North India—An
Analysis of 1000 Cases
by Pratibha D. Singhi,a Munni Ray,a and Gunmala Surib
Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India
b University Business School, Punjab University, Chandigarh, India
a
Summary
One thousand children with cerebral palsy (CP) were reviewed to study their clinical profile, etiological factors and associated problems. Spastic quadriplegia constituted the predominant group (61
per cent), followed by spastic diplegia (22 per cent). Dyskinetic CP was present in 7.8 per cent of the
cases. Acquired CP, particularly secondary to nervous system infections, constituted a significant
proportion of cases. The clinical spectrum of CP is different in developing countries compared with
developed countries. Associated problems were present in a majority (75 per cent) of cases, of which
mental retardation was the commonest (72.5 per cent). Comprehensive assessment and early
management of these problems are emphasized, which can minimize the extent of disabilities.
Introduction
Changes in the epidemiology of cerebral palsy (CP)
have been reported from developed countries.1–3
Advances in perinatology have led to increasing
survival of preterms and a change in the distribution
of clinical types of CP.4,5 For the developing countries no accurate data regarding incidence, prevalence or clinical spectrum of CP is available. The
purpose of this study was to evaluate the clinical
profile of CP and to determine the associated risk
factors among North Indian children.
Materials and Methods
The study included 1000 consecutive children with
CP who were seen at The Rehabilitation Centre for
Disabled Children, Chandigarh from 1985 to 1993.
The Centre provides assessment and intervention
services to children with various types of disabilities
brought from all over North India after referral from
hospitals and doctors, or directly from the
community by the parents.
Cerebral palsy was defined as a non-progressive
disorder that manifests as abnormality of motion and
posture and results from a central nervous system
injury sustained in the early period of brain development, usually defined as the first 3 or 5 years of life.6
A detailed history was taken from the mother using
a prestructured proforma followed by a complete
physical, developmental and neurological examin-
Correspondence: Dr Pratibha D. Singhi, Department of Pediatrics,
Postgraduate Institute of Medical, Education and Research,
Chandigarh, India. Fax 91–0172–744401. E-mail <[email protected].
nic.in>.
162
Journal of Tropical Pediatrics
ation. For labeling a child as having birth asphyxia,
Apgar scores when available were considered. In the
absence of these, the following criteria were used: (i)
history of delayed cry > 5 min after birth; (ii) baby
turning blue and requiring oxygen therapy with baby
having difficulty in respiration, lethargy and/or
seizures within 72 h of birth. Protein energy malnutrition (PEM) was graded according to the criteria
proposed by the Nutrition Sub-Committee of Indian
Academy of Pediatrics (1972),7 by expressing the
weight of the child as a percentage of the expected
and classifying as follows: normal, > 80 per cent;
grade I, 71–80 per cent; grade II, 61–70 per cent;
grade III, 50–60 per cent; grade IV, < 50 per cent of
expected weight for age. Sociodemographic and
family structure details were recorded. Assessment
of intelligence/development quotient (IQ/DQ) and
an ENT and ophthalmologic evaluation was
performed in all children.
The cases of CP were classified, using the Swedish
classification, into spastic (tetraplegia, diplegia,
hemiplegia), ataxic and dyskinetic (including athetosis; dystonia) and mixed.8 The etiological classification was also used as: (i) congenital (prenatal,
perinatal and mixed pre- and perinatal causes); and
(ii) acquired CP occurring in a child born normally
and due to identifiable factors acquired after birth.
Clinical psychologists conducted psychometric
evaluations and children were categorized into mild
(IQ = 50–70), moderate (IQ = 30–50) and severe (IQ
< 30) mental retardation. All children were screened
for a hearing defect, and in those suspected of having
a hearing loss formal audiometry was done. Children
with active seizures or with a definite history of
recurrent seizures were considered to have epilepsy.
Behavioural problems were considered significant if
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P. D. SINGHI ET AL.
they were severe enough to be perceived as problems
by parents or interfered with therapy or education.
All children were also evaluated by a team of
therapists, provided appropriate rehabilitation, and
were periodically followed-up.
Data were entered in Foxpro and descriptive
statistics were calculated using SPSS software
package.
Results
The age at the time of presentation ranged from 2
months to 16 years (mean age: 36.4 ± 31.9 months). A
male preponderance was noted (67.5 per cent).
About half (50.2 per cent) of the patients were below
2 years of age at the time of their first visit, 34.2 per
cent were between 2 and 5 years, and 15.6 per cent
were above 5 years of age. The presenting problems
were delay in motor milestones (88.8 per cent),
delayed speech (47.6 per cent), seizures (28.1 per
cent), and behavior problems. The majority of the
cases were congenital (77 per cent). Antenatal
problems (Fig. 1) were reported in 25 per cent of the
mothers. In 33.6 per cent of cases the deliveries were
conducted at home; 85 per cent were normal vaginal
deliveries, 9.9 per cent were caesarian section. 1.5 per
cent were breech, 3.7 per cent required instrumentation; 86.8 per cent were term, and 13.2 per cent were
preterm. Delayed cry was reported in 49.2 per cent
cases. About half (49.6 per cent) of the children were
first borns; 27.6 per cent were second, and 22.8 per
cent were third in birth order. The predisposing
neonatal factors are shown in Table 1. Of the 23 per
cent cases of acquired CP, 63.5 per cent were due to
TABLE 1
Neonatal factors predisposing to cerebral palsy
Prematurity
Birth asphyxia
Neonatal convulsions
Neonatal septicemia
Neonatal jaundice
Low birthweight
Twins
CP cases
(n = 1000)
%
132
453
252
146
215
204
12
13.2
45.3
25.2
14.6
21.6
20.4
1.2
CNS infections such as meningitis and encephalitis,
and 30.1 per cent were due to bilirubin encephalopathy (secondary to Rh incompatibility or G6PD
deficiency) and 2.7 per cent due to head injury.
The distribution of cases of CP is shown in Table 2;
70 per cent were spastic and 61 per cent had quadriplegia. The associated problems as seen in the whole
group are shown in Table 3 and those in relation to
types of CP are shown in Table 4. About half (50.6
per cent) of cases were malnourished; 28.1 per cent
had grades III and IV malnutrition. Children with
mixed CP were most malnourished (68.35 per cent)
followed by those with athetoid (59.50 per cent),
hypotonic/ataxic (51.95 per cent), and spastic (42.86
per cent) CP. Children with spastic hemiplegia were
least malnourished (36.18 per cent). Most children
(88.6 per cent) had a head circumference less than a
2 SD of normal. Mental retardation was seen in 72.5
per cent cases; 42.7 per cent had severe, 26.0 per cent
moderate, and 31.3 per cent had mild mental retardation. All cases of mixed CP and athetoid CP had
mental retardation compared with 62.5 per cent of
cases of spastic and 75.3 per cent of cases of hypotonic CP. Convulsions were seen in 327 children,
most commonly in those with spastic hemiplegia
(42.0 per cent), diplegia (32.5 per cent), quadriplegia
(31.6 per cent), mixed (35.3 per cent), hypotonic
(25.9 per cent), and athetoid (27.4 per cent) CP. The
various types of seizures seen as depicted in Fig. 2.
TABLE 2
Distribution of types of cerebral palsy cases
Type of cerebral palsy
FIG. 1. Antenatal complications in mothers of
children with cerebral palsy. , Antepartum
hemorrhage; , fever; , pre-eclamptic toxemia;
, drugs; , other.
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Spastic
Quadriplegia
Diplegia
Hemiplegia
Right
Left
Dyskinetic/athetoid
Hypotonic/ataxic
Mixed
n
700
427
154
119
58
61
84
77
139
%
70
61
22
17
8.4
7.7
13.9
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P. D. SINGHI ET AL.
TABLE 3
Associated problems in children with cerebral palsy
Associated problems
Mental retardation
Speech disorder
Visual disorder
Hearing disorder
Convulsions
Behavior problems
Malnutrition
n
%
725
78
410
140
327
72
506
72.5
7.8
41.0
14.0
32.0
7.2
50.6
FIG. 2. Types of seizures in children with cerebral
palsy. , Generalized tonic clonic; , focal;
, atonic; , unclassified; , myoclonic.
Ocular defects were found in 41 per cent of children.
These were found more commonly in children with
dyskinetic CP (45.24 per cent) followed by mixed
(43.9 per cent), spastic (38.0 per cent), and hypotonic
(20.9 per cent) CP. Refractory errors were detected
in 48 per cent of cases. Hypermetropia was more
common than myopia. Strabismus was found in 24.5
per cent cases; 84 per cent had convergent and 16 per
cent had divergent squint. Optic atrophy (10.87 per
cent), nystagmus (4 per cent), and cataracts (3.9 per
cent) were also seen. Ptosis, corneal opacity, chorioretinitis and cortical blindness were seen in 2–4 per
cent cases. Speech delay was reported in 82 per cent
of cases of cerebral palsy. Hearing loss and speech
motor problems were most commonly seen in
dyskinetic CP.
Discussion
The preponderance of spastic cerebral palsy cases in
our study is similar to that reported by others.10
However, distribution of the clinical types of spastic
cases was very different. Whereas spastic diplegia is
generally the commonest form reported from developed countries,1 in our series spastic quadriplegia
was most commonly seen, as is also seen in other
developing countries. In developed countries, a
progressive decrease in spastic quadriplegia and a
relative increase in spastic diplegia has been attributed to the decrease in perinatal mortality rate, with
increasing survival rates of extremely premature
infants.4,5 This situation has not yet been achieved in
our country except in a few tertiary care centres.
Other studies from more developed countries have
found hemiplegia to be the commonest form of
spastic CP.10 In our study this was seen in only17 per
cent cases. While athetoid CP, particularly secondary
to neonatal hyperbilirubinemia, has virtually
disappeared from many parts of the world, it still
constitutes a significant proportion of CP cases in
India, and 41.6 per cent of our cases had a history of
significant neonatal jaundice.
The role of perinatal complications, in particular
TABLE 4
Associated problems in relation to types of cerebral palsy
Types of CP
Spastic
quadriplegia
Spastic
hemiplegia
Spastic
diplegia
Mixed
Hypotonic/
ataxic
Athetoid
dyskinetic
164
Total
Seizures
Mental
retardation
Speech
defects
Visual
defects
Hearing
defects
Microcephaly
Malnutrition
Behavior
problems
427
135
(31.6%)
50
(32.5%)
50
(42.0%)
49
(35.3%)
20
(25.9%)
23
(27.4%)
276
(64.6%)
73
(61.3%)
95
(61.7%)
139
(100%)
58
(75.3%)
84
(100%)
31
(7.3%)
8
(6.7%)
10
(6.5%)
14
(10.1%)
6
(7.8%)
9
(10.7%)
181
(42.4%)
32
(26.9%)
69
(44.8%)
61
(43.9%)
29
(20.9%)
38
(45.2%)
58
(13.6%)
12
(10.1%)
16
(10.4%)
14
(10.1%)
12
(15.6%)
28
(33.3%)
399
(93.3%)
106
(89.1%)
119
(77.3%)
124
(89.2%)
72
(95.3%)
66
(78.6%)
212
(49.7%)
43
(36.1%)
66
(42.9%)
95
(68.4%)
40
(52.0%)
50
(59.5%)
28
(6.55%)
11
(9.2%)
10
(6.5%)
8
(5.8%)
7
(9.1%)
8
(9.5%)
119
154
139
77
84
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P. D. SINGHI ET AL.
birth asphyxia, in the causation of CP has been challenged.11,12 However, we found history indicative of
birth asphyxia in a large number of cases. This is
similar to other studies from Nigeria, Malta, and
other developing countries.13–15 Occurrence of
severe birth asphyxia, which is rarely seen in developed countries, continues to be a major problem in
many developing countries where obstetric facilities
are virtually non-existent for a vast majority of
women in rural areas.
Several studies have reported a significant association between low birthweight and cerebral
palsy.11,12,16,17 Higher survival rate of preterm, low
birthweight infants, usually attributed to more
advanced obstetric care, is associated with a higher
rate of CP in these infants.18 However, unlike
western figures, most children in our study were term
babies.
Acquired cases of CP, particularly secondary to
CNS infections and bilirubin encephalopathy, constitute a significant proportion of CP in our country as
well as other developing countries.19,20
Although a direct causative role of antenatal
problems cannot be commented on with certainty,
they were reported in a third of cases. A significant
association between antepartum hemorrhage and
cerebral palsy has been reported11,16 and refuted.12,18
Toxemia in the mother as a risk factor for cerebral
palsy in the baby has been found by several
authors.11,12 A large proportion of mothers gave
history of fever in the early antenatal period;
however, tests for intrauterine infections in their
babies were negative.
Most of the babies in our study were born by
normal vaginal delivery. Instrument-assisted
delivery18 and caesarian section10,21 have been
associated with cerebral palsy,18 but others have
differed on this.10,22 Multiple pregnancy has been
considered an important prenatal risk factor for
CP.10 However, in our study only four pairs of twins
were identified.
Almost all children with cerebral palsy have at
least one additional disability associated with
damage to CNS.23 Approximately two-thirds of
children with cerebral palsy have mental retardation.6 Our findings are in accordance with this.
Children with spastic quadriplegia and mixed type
of cerebral palsy have the worst intellectual
outcome.
Epilepsy in children with cerebral palsy occurs
frequently.24 Seizures are reported to be least
common in children with choreoathetoid and ataxic
types of CP,25 as was also found in our study. Most
epileptic patients with cerebral palsy have locationrelated epilepsies; focal and generalized seizures
dominate the clinical picture.24 Although generalized seizures were commonest, 32 per cent of our
children had myoclonic seizures – this figure is higher
than that described in literature.26 Epilepsy was
Journal of Tropical Pediatrics
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June 2002
significantly more common in those children with
cerebral palsy who had mental retardation (85.4 per
cent) compared with those without mental retardation (14.6 per cent).
The poor nutritional status of children in the study
is explained by feeding problems, gastroesophageal
reflux, inability to independently access food or
communicate hunger and constipation.27,28 Hearing
loss and dysarthria, seen in our children with dyskinetic or mixed CP, were similar to that reported in
the literature.29 Ocular problems and difficult
behavior were other problems requiring intervention.
In conclusion the spectrum of CP in North India
differs from that seen in the West. The shift to
predominant involvement of preterm survivors is
still not apparent. Severe birth asphyxia is an important predisposing factor for CP. Acquired CP, particularly secondary to CNS infections and kernicterus,
constitutes a significant proportion of CP cases.
Targeting the preventable causes of CP may help
reduce, to some extent, the enormous problem of
childhood disability in the country.
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