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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


a Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India
b University Business School, Punjab University, Chandigarh, India

Summary
One thousand children with cerebral palsy (CP) were reviewed to study their clinical profile, etio-
logical 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 ation. For labeling a child as having birth asphyxia,


Changes in the epidemiology of cerebral palsy (CP) Apgar scores when available were considered. In the
have been reported from developed countries.1–3 absence of these, the following criteria were used: (i)
Advances in perinatology have led to increasing history of delayed cry > 5 min after birth; (ii) baby
survival of preterms and a change in the distribution turning blue and requiring oxygen therapy with baby
of clinical types of CP.4,5 For the developing coun- having difficulty in respiration, lethargy and/or
tries no accurate data regarding incidence, preva- seizures within 72 h of birth. Protein energy malnu-
lence or clinical spectrum of CP is available. The trition (PEM) was graded according to the criteria
purpose of this study was to evaluate the clinical proposed by the Nutrition Sub-Committee of Indian
profile of CP and to determine the associated risk Academy of Pediatrics (1972),7 by expressing the
factors among North Indian children. 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;
Materials and Methods grade III, 50–60 per cent; grade IV, < 50 per cent of
The study included 1000 consecutive children with expected weight for age. Sociodemographic and
CP who were seen at The Rehabilitation Centre for family structure details were recorded. Assessment
Disabled Children, Chandigarh from 1985 to 1993. of intelligence/development quotient (IQ/DQ) and
The Centre provides assessment and intervention an ENT and ophthalmologic evaluation was
services to children with various types of disabilities performed in all children.
brought from all over North India after referral from The cases of CP were classified, using the Swedish
hospitals and doctors, or directly from the classification, into spastic (tetraplegia, diplegia,
community by the parents. hemiplegia), ataxic and dyskinetic (including atheto-
Cerebral palsy was defined as a non-progressive sis; dystonia) and mixed.8 The etiological classifi-
disorder that manifests as abnormality of motion and cation was also used as: (i) congenital (prenatal,
posture and results from a central nervous system perinatal and mixed pre- and perinatal causes); and
injury sustained in the early period of brain develop- (ii) acquired CP occurring in a child born normally
ment, usually defined as the first 3 or 5 years of life.6 and due to identifiable factors acquired after birth.
A detailed history was taken from the mother using Clinical psychologists conducted psychometric
a prestructured proforma followed by a complete evaluations and children were categorized into mild
physical, developmental and neurological examin- (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
Correspondence: Dr Pratibha D. Singhi, Department of Pediatrics, a hearing loss formal audiometry was done. Children
Postgraduate Institute of Medical, Education and Research, with active seizures or with a definite history of
Chandigarh, India. Fax 91–0172–744401. E-mail <medinst@pgi.chd. recurrent seizures were considered to have epilepsy.
nic.in>. Behavioural problems were considered significant if

162 Journal of Tropical Pediatrics Vol. 48 June 2002  Oxford University Press 2002
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P. D. SINGHI ET AL.

they were severe enough to be perceived as problems TABLE 1


by parents or interfered with therapy or education. Neonatal factors predisposing to cerebral palsy
All children were also evaluated by a team of
therapists, provided appropriate rehabilitation, and CP cases %
were periodically followed-up. (n = 1000)
Data were entered in Foxpro and descriptive
Prematurity 132 13.2
statistics were calculated using SPSS software
Birth asphyxia 453 45.3
package. Neonatal convulsions 252 25.2
Neonatal septicemia 146 14.6
Neonatal jaundice 215 21.6
Results Low birthweight 204 20.4
The age at the time of presentation ranged from 2 Twins 12 1.2
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 CNS infections such as meningitis and encephalitis,
cent were between 2 and 5 years, and 15.6 per cent and 30.1 per cent were due to bilirubin encephalopa-
were above 5 years of age. The presenting problems thy (secondary to Rh incompatibility or G6PD
were delay in motor milestones (88.8 per cent), deficiency) and 2.7 per cent due to head injury.
delayed speech (47.6 per cent), seizures (28.1 per The distribution of cases of CP is shown in Table 2;
cent), and behavior problems. The majority of the 70 per cent were spastic and 61 per cent had quadri-
cases were congenital (77 per cent). Antenatal plegia. The associated problems as seen in the whole
problems (Fig. 1) were reported in 25 per cent of the group are shown in Table 3 and those in relation to
mothers. In 33.6 per cent of cases the deliveries were types of CP are shown in Table 4. About half (50.6
conducted at home; 85 per cent were normal vaginal per cent) of cases were malnourished; 28.1 per cent
deliveries, 9.9 per cent were caesarian section. 1.5 per had grades III and IV malnutrition. Children with
cent were breech, 3.7 per cent required instrumenta- mixed CP were most malnourished (68.35 per cent)
tion; 86.8 per cent were term, and 13.2 per cent were followed by those with athetoid (59.50 per cent),
preterm. Delayed cry was reported in 49.2 per cent hypotonic/ataxic (51.95 per cent), and spastic (42.86
cases. About half (49.6 per cent) of the children were per cent) CP. Children with spastic hemiplegia were
first borns; 27.6 per cent were second, and 22.8 per least malnourished (36.18 per cent). Most children
cent were third in birth order. The predisposing (88.6 per cent) had a head circumference less than a
neonatal factors are shown in Table 1. Of the 23 per 2 SD of normal. Mental retardation was seen in 72.5
cent cases of acquired CP, 63.5 per cent were due to per cent cases; 42.7 per cent had severe, 26.0 per cent
moderate, and 31.3 per cent had mild mental retar-
dation. 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 hypo-
tonic 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 n %

Spastic 700 70
Quadriplegia 427 61
Diplegia 154 22
Hemiplegia 119 17
Right 58
FIG. 1. Antenatal complications in mothers of Left 61
children with cerebral palsy. , Antepartum Dyskinetic/athetoid 84 8.4
Hypotonic/ataxic 77 7.7
hemorrhage; , fever; , pre-eclamptic toxemia;
Mixed 139 13.9
, drugs; , other.

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P. D. SINGHI ET AL.

TABLE 3 (43.9 per cent), spastic (38.0 per cent), and hypotonic
Associated problems in children with cerebral palsy (20.9 per cent) CP. Refractory errors were detected
in 48 per cent of cases. Hypermetropia was more
Associated problems n % common than myopia. Strabismus was found in 24.5
per cent cases; 84 per cent had convergent and 16 per
Mental retardation 725 72.5 cent had divergent squint. Optic atrophy (10.87 per
Speech disorder 78 7.8
cent), nystagmus (4 per cent), and cataracts (3.9 per
Visual disorder 410 41.0
Hearing disorder 140 14.0
cent) were also seen. Ptosis, corneal opacity, chori-
Convulsions 327 32.0 oretinitis and cortical blindness were seen in 2–4 per
Behavior problems 72 7.2 cent cases. Speech delay was reported in 82 per cent
Malnutrition 506 50.6 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 devel-
oped 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 attrib-
uted 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
FIG. 2. Types of seizures in children with cerebral
cent cases. While athetoid CP, particularly secondary
palsy. , Generalized tonic clonic; , focal;
to neonatal hyperbilirubinemia, has virtually
, atonic; , unclassified; , myoclonic.
disappeared from many parts of the world, it still
constitutes a significant proportion of CP cases in
Ocular defects were found in 41 per cent of children. India, and 41.6 per cent of our cases had a history of
These were found more commonly in children with significant neonatal jaundice.
dyskinetic CP (45.24 per cent) followed by mixed The role of perinatal complications, in particular

TABLE 4
Associated problems in relation to types of cerebral palsy

Types of CP Total Seizures Mental Speech Visual Hearing Microcephaly Malnutrition Behavior
retardation defects defects defects problems

Spastic 427 135 276 31 181 58 399 212 28


quadriplegia (31.6%) (64.6%) (7.3%) (42.4%) (13.6%) (93.3%) (49.7%) (6.55%)
Spastic 119 50 73 8 32 12 106 43 11
hemiplegia (32.5%) (61.3%) (6.7%) (26.9%) (10.1%) (89.1%) (36.1%) (9.2%)
Spastic 154 50 95 10 69 16 119 66 10
diplegia (42.0%) (61.7%) (6.5%) (44.8%) (10.4%) (77.3%) (42.9%) (6.5%)
Mixed 139 49 139 14 61 14 124 95 8
(35.3%) (100%) (10.1%) (43.9%) (10.1%) (89.2%) (68.4%) (5.8%)
Hypotonic/ 77 20 58 6 29 12 72 40 7
ataxic (25.9%) (75.3%) (7.8%) (20.9%) (15.6%) (95.3%) (52.0%) (9.1%)
Athetoid 84 23 84 9 38 28 66 50 8
dyskinetic (27.4%) (100%) (10.7%) (45.2%) (33.3%) (78.6%) (59.5%) (9.5%)

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P. D. SINGHI ET AL.

birth asphyxia, in the causation of CP has been chal- significantly more common in those children with
lenged.11,12 However, we found history indicative of cerebral palsy who had mental retardation (85.4 per
birth asphyxia in a large number of cases. This is cent) compared with those without mental retarda-
similar to other studies from Nigeria, Malta, and tion (14.6 per cent).
other developing countries.13–15 Occurrence of The poor nutritional status of children in the study
severe birth asphyxia, which is rarely seen in devel- is explained by feeding problems, gastroesophageal
oped countries, continues to be a major problem in reflux, inability to independently access food or
many developing countries where obstetric facilities communicate hunger and constipation.27,28 Hearing
are virtually non-existent for a vast majority of loss and dysarthria, seen in our children with dyski-
women in rural areas. netic or mixed CP, were similar to that reported in
Several studies have reported a significant associ- the literature.29 Ocular problems and difficult
ation between low birthweight and cerebral behavior were other problems requiring inter-
palsy.11,12,16,17 Higher survival rate of preterm, low vention.
birthweight infants, usually attributed to more In conclusion the spectrum of CP in North India
advanced obstetric care, is associated with a higher differs from that seen in the West. The shift to
rate of CP in these infants.18 However, unlike predominant involvement of preterm survivors is
western figures, most children in our study were term still not apparent. Severe birth asphyxia is an import-
babies. ant predisposing factor for CP. Acquired CP, particu-
Acquired cases of CP, particularly secondary to larly secondary to CNS infections and kernicterus,
CNS infections and bilirubin encephalopathy, consti- constitutes a significant proportion of CP cases.
tute a significant proportion of CP in our country as Targeting the preventable causes of CP may help
well as other developing countries.19,20 reduce, to some extent, the enormous problem of
Although a direct causative role of antenatal childhood disability in the country.
problems cannot be commented on with certainty,
they were reported in a third of cases. A significant References
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