Neurocysticercosis in childhood

Trc.t~s~azo~s

OF THE

ROYALSOCIETY OF TROPICALMEDICINEANDHYGIENE(1987)

Neurocysticercosis

81, 371-373

371

in childhood

V. KALRA, V. K. PAUL, R. K. MARWAH, G.S. KOCHHARAND S. BHARGAVA Department of Pediatrics, All-India Institute of Medical Sciences, New Delhi-110029, India Abstract Manifestations of cysticercosis in 11 children (mean age9.5 years) are presented. Features of raised intracranial pressure dominated the clinical picture (10 cases), followed by seizures (7 cases). Subretinal cysts occurred in three children. Focal signs were infrequent and often not attributable to anatomical lesions. Electra-encephalograms were invariably abnormal, but did not help to localize lesions. Plain roentgenograms of the skull showed sutural diastasis in the majority. Computerized tomography revealed white matter oedemawith throttled ventricles in 6 patients and single localized lesions in 3 others. None had hydrocephalus. Histopathology and indirect haemagglutination test aided diagnosis in 6 cases.Two children recovered completely, one after surgical excision of a parietal lobe cyst and the other following praziquantel therapy. Half of the remaining patients improved spontaneously. Introduction Cerebral cysticercosis is uncommon in childhood and appears in the literature as sporadic casereports (RAO et al., 1970; NAIR et al., 1976; TASKER & PLOTKIN, 1979; PERCYet aE., 1980). Patients below 10 years of age constitute 2.3 to 8.0% of all casesin large series, most casesbeing adults in the third or fourth decade (DIXON & LIPSCOMB, 1961; STEPIEN, 1962; TRELLES & TRELLES, 1978). The low incidence of diseaseamong children is attributable to the long incubation period which varies from a few months to 30 years, averaging 4.8 years (DIXON & LIPSCOMB, 1961).

Neurocysticercosis mimics many neurological syndromes. The usual manifestations include seizures, intracranial hypertension with or without focal symptomatology, and chronic meningitis (DIXON & LIPSCOMB, 1961.; STEPIEN, 1962; MC@MICK et al., 1982). A high index of suspicion 1s necessary to identify the cases;definitive diagnosis is possible only on histopathology. Computerized tomography (MERVIS & LOTZ, 1980; NASH & NEVA, 1984) and serological tests including indirect haemagglutination tests (MAHAJAN et al., 1975) and the recently developed radioimmunoassay and enzyme-linked immunosorbent assay (MILLER et al., 1983) are useful aids in diagnosis. Little is known of childhood neurocysticercosis. This paper presents clinical profiles of 11 children with protean manifestations of the infection. Material

and Methods

At the Pediatric Neurology Clinic of the All-India Institute of Medical Sciences? New Delhi, cerebralcysticercosiswasdiagnosedin I1 children during the years1979to 1983.A detailed clinical profile was noted and a careful examination made for subcutaneous nodules and ocular involvement. The investigations carried out in suspected children included lumbar puncture (unless contraindicated because of raised intracranial pressure), electroencephalogram (EEG) and skull X-ray. Thigh roentgenograms were done in 6 casesand computerized axial tomography (CAT) in all. Histopathological confirmation was obtained from subcutaneous nodules in 3 patients and from the excised brain cyst in another. Indirect haemagglutination (IHA) tests on blood, cerebrospinal fluid, or both (MAHAJAN et al., 1975)

were oerformed at another institute in 4 cases; a titre of 1:128 or more was considered positive. In all’ cases, the possibility of neurotuberculosis was excluded by Mantoux test, chest roentgenogram and family survey for tuberdOsis. Two cases had received adequate antituberculous therapy in the past without response. Symptomatic therapy for seizures and raised intracranial pressure was given when indicated. One patient received praziquantel in a dose of SOmg/kdday orally along with steroids for a period of two weeks. Craniotomy was performed to remove an approachable single cystic lesion in one case and to relieve gross intracranial hypertension in another. 2 patients were lost to follow up, the rest were followed for variable periods up to 5 years.

Observations Eleven out of a total of 2800 children enrolled in the Pediatric Neurology Clinic over a period of 4 years were documented to have neurocysticercosis. This amounts to an incidence of 0.4% among children with neurological problems. All our patients were males in the age group of 5 to 14 years (mean 9.5 years). All except one had one or more clinical features of raised intracranial pressure, namely headache, vomiting or papilloedema. Headache and vomiting were the presenting complaints in 2 cases respectively. Papilloedema was observed in 9 and seizures occurred in 8 cases. Prevalence of focal and generalized seizures was equal. Only 2 of the 4 patients with focal seizures had localizable lesions on CAT. Focal deficit included cerebellar signs in 3 and hemiparesis with parietal lobe signs in one. Abducens palsy was present in 3, of whom 2 had other clinical evidence of raised intracranial uressure. One Datient each had altered sensoriurn,. abnormal behaviour, and dementia. Visual svmotoms included didoDia in 2. Tvuical subretinal cy%c were present in 3, of whom 2 h&i complete loss

of vision in the affected eye. 3 patients had subcutaneous nodules. Only one patient gave a history of consuming pork, and one of passing tapeworm proglottids in the stools. Cerebrospinal fluid (CSF) was normal in one. Three had lymphocytic pleocytosis (up to 100 cells per mm3), with presence of globulins and normaI sugar.

372

NEUROCYSTICERCOSIS IN CHILDHOOD

Skull roentgenograms revealed sutural diastasis suggesting raised intracranial tension in 6 cases;none showed intracranial calcification. CAT contributed a great deal to the diagnosis of neurocysticercosis. The commonest pattern was diffuse white matter oedema with throttled ventricles in 6 cases.Focal lesions were documented in 3; they were parietal in 2 and frontal in one. 2 had low attenuation cystic lesions, while the third had a high attenuation ring-like space-occupying lesion with an extensive surrounding hypodense lesion indicating oedema.In one patient multiple high attenuation lesions measuring lmm were seen scattered bilaterally in cerebral cortices and basal ganglia. Meningobasal or ventricular cysticercosis was not observed. No case had hydrocephalus. Initial CT scans were normal in 2 case with typical subretinal cysts; in one of them a repeat study after 2 years revealed bilateral multinle hvnodense lesions. EEG was abnormal in-all c&is. Only in one did the lateralization in EEG correlate with the nature of focal seizures and the location of the lesion on CAT. Thigh roentgenograms done in 6 patients showed tissue calcification in none. IHA test (MAHAJANet al., 1973) corroborated the diagnosis in 2 cases.Histopathology of subcutaneous nodules in 3, and that of the excised parietal lobe lesion in one, confirmed the diagnosis of cysticercosis. The child who underwent surgical decompression died 6 months later. One patient, from whose parietal lobe a cyst was removed,-recovered completely. The repeat CT of the natient treated with nraziauantel was normal 6 weeks after treatment and ho symptom has recurred for one year. Of the remaining 6 who were followed up, 3 showed partial improvement, 2 deteriorated and one remained unchanged. Discussion Not unexpectedly, almost all our caseshad symptoms and signs of intracranial hypertension. The reported prevalence of these features has varied from 6 to 98% (ARSENI & SAMTICA, 1957; DIXON 81 LIPSCOMB,1961; STEPIEN,1962; MCCORMICKet al., 1982). The second commonest manifestation was seizures, previously reported in 37 to 90% of cases(ARSENI& SAMTICA,1957; DIXON & LIPSCOMB,1961; STEPIEN, 1962; MCCORMICK et al., 1982). In south India, MAN1 et al. (1974) reported neurocysticercosis as the aetiology in 2.2% of 631 epileptic patients. Focal seizures related poorly to the anatomical localization of the lesion on CAT in our experience, occurring in fewer than half of our cases;the reported incidence varies from 3 to 97% (STEPIEN, 1962; TRELLES & TRELLES. 1978’1. Evidence of taeniasis and wrkeating was scarce in our patients, being preseit in only 2. This was similar to the incidence of 22% among 450 cases reported by DIXON i? LIPSCOMB (1961). Subcutaneous nodules provide the most useful clinical clue to the diagnosis of cysticercosis in a patient with neurological complaints. Their prevalence is variably reported to be 4% (ARSENI & SAMITCA, 1975), 28% (DINAKAR et al., 1970) and 54% (DIXON & LIPSCOMB, 1961). The presence of accessiblesubcutaneous nodules helped in clinching a histopathological diagnosis in 3 of our cases. Children are more prone to localize cysticerci in the

eyes than adults. One-fifth of the patients with ocular cysticercosis are reported to be below 10 years, and sub-retinal cysts were observed in 40% of a large series (LECH, 1949). In India, ocular cysticercosis is more frequently reported from the south, (REDDY i? SATYENDRAN, 1964; JAIN et al., 1979). The commonest radiological abnormality on plain films in adults is intracranial calcification (SANTIN & VARGAS,1966). In childhood neurocysticercosis this finding is rare becauseit usually takes almost 10 years for intracranial calcification to develop (DIXON & LIPSCOMB, 1961). This may explain the absence of this finding in our subjects..In contrast, PERCYet al. (1980) renorted calcifications in 7 of 9 children with neurocys&ercosis. Radiological evidence of raised intracranial pressure has been reported in 10 to 72% (ARSENI & SAMTICA, 1957; DORFSMAN, 1963). The usual picture of paediatric neurocysticercosis on CAT is the presence of diffuse low attenuation areas predominantly in the white matter with small throttled ventricles (MERVIS & LOTZ, 1980; DANZIGER & BLOCH, 1975), indicating severe white matter oedema. Low attenuation cystic masses or dense nodular lesions are uncommon (CARBAJALet al:, 1977). Lesions in the subarachnoid spaceand ventrtcles may be difficult to identify but-the consequent hvdrocenhalus is usuallv evident. This relativelv common form in adults (SHANLEY & JORDON, 1986; MCCORMICK et al., 1980)was conspicuous by its total absencefrom- our cases,in whom intracranial hypertension was generally related to the white matter oedema. In the 9 paediatric patients reported by PERCY et al. (1980), who had apparentlv contracted diseasein Mexico or Guatemala-,-focalcystic lesions dominated the CAT nrofile: none had white matter oedema or hydroceph’alus. It appears that the profile of brain involvement in cysticercosis may vary not only in different age groups but also in different geographical areas (BI-&RG~vA, 1983). EEG is almost invariablv abnormal but has verv little diagnostic value (TREBLES& TRELLES, 1978)“. Similarly, routine cerebrospinal fluid examination is usually abnormal but not diagnostic. The indirect haemagglutination test is considered reasonably reliable in diagnosing cysticercosis (Loo & BRAUDE, 1982; MCCORMICK et al., 1982). The positivity rate of this test at the centre where our samples were processed is 87.5% (MAHAJAN et al., 1975). Until recently the only definitive therapy was surgical removal of an accessiblelesion. The rest of the- patients had an unpredictable course, most remaining unchanged (60%) or deteriorating (19%) on long-term follow up (DIXON & LIPSCOMB, 1962). The introduction of praziquantel has changed the outlook of the disease.With it, clinical imnrovement has been reported in 87.5% (G~oLL, 1982)io 100% (SOTELO et ul., 1984) of patients. The usual therapeutic regimen is 50mg/kg/day for 15 days. A strong inflammatory response due to the dying larvae may result in increased intracranial pressure during treatment. The manifestations can be controlled or, better, prevented by concomitant use of corticosteroids (NASH & NEVA, 1984). Use of praziquantel in one casereported here led to disappearanceof CAT abnormalities within a period of 6 weeks and clinical quiescence for over a year. Another drug, metrifonate, an organophosphorus compound, has been shown to be effective in

v.

KALKA

cutaneous (TSCHEN et al., 1981) and cerebral cysticercosis (TRUJILLO-VALDES,1981). The evidence is still preliminary and more studies are needed to justify its recommendation. Acknowledgements The authors are grateful to Dr R. C. Mahajan of the Postgraduate Institute of Medical Education and Research, Chandigarh, India for conducting the indirect haemagglutination tests. References Arseni, C. & Samtica, D. C. (1957). Cysticercosis of the brain. British Medical Journal, ii, 494-497. Bhargava, S. (1983). Radiology - including computed tomography - of parasitic diseasesof the central nervous system. Neurosurgical Review, 6, 129-137. Carbajal, J. R., Palacios, E., Axar-Kia, B. & Churchill, R. (1977). Radiology of cysticercosis of the central nervous system including computed tomography. Radiology,lZS, 127-131. Danziger, J. & Bloch, S. (1975). Tapeworm cyst infestations of brain. Clinical Radiology, 26, 141-148. Dinaker, I., Mathai, K. V. & Chandy, J. (1970). Cysticerco& of brain. Neurology India, 18, 165-170. Dixon, H. B. F. & Lipscomb, F. M. (1961). Cysticercosir:an analysis and follow up of 450 cases. Medical Research Council Special Report Series No. 299. London: Her Majesty’s Stationery Office. Dorfsman, J. (1963). The radiologic aspects of cerebral cysticercosis. Acta Radiologica, l,, 836-842. Groll, E. (1981). Human cysticercosts and praxiquantel: a survey of first clinical experience. Boletin ChileM de Parasitologfa, 36, 29-37. (Quoted from Biological Abstracts, 1982, 73, 5387. Ref. No. 52017.) Jain, I. S., Dhir, S. P., Chattopadhyaya, P. R. & Kumar, P. (1979). Ocular cysticercosis in North India. Indian Knnnal of Obhthalmolow.

27. 54-58.

Lech, J. (1949): Ocular cy&ercosis. American Journal of Ophthalmology, 32, 523-547.

Loo, L. & Braude, A. (1982). Cerebral cvsticercosis in San Diego: a report of .23 cases and review of literature. Medicine (Baltimore), 61, 341-359. Mahajan, R. C., Chopra, J. S. & Chitkara N. L. (1975). Comparative evaluation of indirect haemagglutination and complement fixation tests in serodiagnosis of cysticercosis. Indian 3oumal of Medical Research, 63, 121-125. Mani, A. J., Ramesh, C. K., Ahuja G. K. & Mani, K. S. (1974). Cerebral cysticercosis presenting as epilepsy. Neurology India, 22, 30-34.

et al.

373

McCormick, G. F., Zee, C.-S. & Heiden, J. (1982). Cysticercosis cerebri: review of 127 cases. Archives of Neurology, 39, 534-539.

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for publication

9 December

1985