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1064. 2. Gupta AK, Anand NK, Lamba IMS.

Ultrasound evaluation of kidney dimensions in neonates, hzdian Pediatr 1993; 30 : 319-324.

3. Lyons EA, Murphy AV, Arneil FC. So- nar and its uses in kidney diseases in children. Arch Dis Child 1972; 47 : 777-786.

4. Blane CE, Bookstein FL, Dipietro MA, Kelsch RC. Sonographic standards for normal infant kidney length. Amer J Radio11985; 145 : 1289-1291.

5. Laboratory medicine and reference tables. In : Nelson Textbook of Pediatrics 14th Ed. Behrman RE, Klelgman RM (Assoc. Ed), Nelson WE, Vaughan III VC (Sen Eds). 14th Edn. Philadelphia, WB Saunders & Co. 1992, p 1827.

6. Hernandex RJ, Pozanski AK, Kuhn LR,

Mccormick TL. Factors affecting measurement of renal lengthL Radiology 1979; 130 : 653-656.

7. Han BK, Babcock DS. Sonographic measurements and appearance of normal kidneys in children. Amer J Radiol 1985; 145 : 611-616.

8. Rosenbaum DM, Korngold E, Teele RL. Sonographic assessment of renal length in normal children. Amer J Radio/1984; 142 : 467-469.

9. Hederstrom E, Forsberg L. Kidney size in children assessed by ultrasonography and urography. Acta Radiologicai Diagnos~s 1985; 26 : 85-91.

10. Haugstvedt S, Lundberg J. Kidney size in normal children measured by sonogra- phy. Scand J Ural Nephrol 1980; 14 : 251- 255.

Bilateral Basal Ganglia Lucencies Following Acute Febrile Illness

S. Bhaumik, M. Behari and G.K. Ahuja

Department o f Neurology, Al l India Insti tute o f Medical Sciences, N e w Delhi

Abstract. Bilateral striatal necrosis in children without damage elsewhere in the brain can present as an acute neurological disorder or as a progressive disorder. Three children of 6, 7 and 12 years age developed dystonic posture of limbs without any cranial nerve involvement or alteration of sensorium Soon after recovery from .acute high grade febrile illness of 3-4 days duration. Computerized tomographic scan of head showed bilateral necrosis of basal ganglia. We think that these patients probably constitute a clinically and radiologically distinct subgroup of disorder that prouce bilateral striatal necrosis in children. The cause of the syndrome is unknown. (Indian J Pediatr 1996; 63 : 557-560)

Key words : Strfatal necrosis; Encephafitis; Dystonia; Children.

Reprint requests: Dr. M. Behari, Additional Professor, Department of Neurology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029.

Bilateral striatal necrosis in chi ldren with- out damage e lsewhere in the brain m a y present as acute neu ro log ica l d i so rde r or an a progressive disorder . A large var ie ty

558 THE INDIAN JOURNAL OF PEDIATRICS 1996; Vol. 63. No. 4

of familial, infect ive and metabol ic disor- ders are known to produce lucencies of ba- sal ganglionL Here we r epo r t 3 cases in which file patients deve loped dystonia, fol- lowing a brief febrile illness wi th lucencies in basal gangha.

CxsE I

A 6-year-old boy, p r o d u c t of non- consangu inous mar r iage wi th full- term- normal birth and mile stones, had a febrile illness lasting for 4-5 days wi thout any lo- cal iz ing symptoms. There was no head- ache, vomiting, seizures or al tered sensori- urn. Three days af ter feel ing feverish, he was noted to have st iffening and twist ing of both lower limbs and left u p p e r limb re- suit ing in abnormal posture with difficulty in walking and abil i ty to use left u p p e r limb. These abnormal m o v e m e n t s used to alleviate during sleep. On recovery from fe- ver, the child started walking on toes with inversion of feet and flexion deformities at ~rrkles and knees. The left a rm was hyper- sc~pinated and flexed at e t lbow and wrist. There were no symptoms pertaining to sen- sory system, cranial nerves and sphincters. There was no past h is tory of j aundice or neurologica l illness or fami ly his tory of similar or other neurological diseases.

On examinat ion, h igher menta l func- t ions and cranial nerves were fqund nor- real. No KF ring was observed. Flexion con- f racture were noticed in ankles and knees. Sustained twisting pos ture was noticed in I~.{~ fingers and wrist. Feet were p lantar f lexed and inverted. Sensory examinat ion was normal. Deep t endon reflexes could not be elicited due to contractures. Plantar response was flexor bi lateral ly. Rout ine haematotogical and biochemical investiga- tions were normal. Detailed ophthalmolog-

ical check up, including examinat ion for KF r ing on slit l amp was nega t ive . Non-con- trast CT scan sh o w ed bi la tera l basal gan- glia lucencies.

CASE II

A 7-year-old boy with an his tory of febrile illness for 4-5 days in July 1993 without any local iz ing s y m p t o m or associa ted head- ache, vomiting, seizure, a l tered sensorium, jaundice or skin rash. Three to four days af- ter recovery from fever, the pat ient started toe walking on left side and deve loped sus- ta ined twist ing m o v e m e n t s of left foot causing inversion and plantar flexion. Sub- sequently, he developed contracture of left tendo-achlillis. There was no sensory, blad- der or bowel symptoms. No history of simi- lar illness in past or in the family. He was first seen after one year of illness.

On examinat ion, the pa t ien t was found to h a v e no rma l menta l funct ions . Cranial nerves were normal. He had wasting of left leg muslces below knee with contracture in left tendo-achitl is . There was p lan tar flex- ion and inversion of left foot. Other neuro- logical examinat ion , inc lud ing ref lexes were found to be normal (Fig. 1).

Routine haematological and biochemical inves t iga t ions p r o d u c e d n o rm a l results. Ophtha lmologica l check up, inc luding slit l amp examina t ion for KF ring, was nega- tive. N o n contras t CT d o n e in July 1993 showed bilateral symmetrical basal ganglia lucencies. Repeat CT scan done dur ing ad- miss ion showed the same lesion which had become smal ler m size (Fig. 2).

CASE III

A 12-year-old boy had high grade fever for

1996; Vol. 63. No. 4 THE INDIAN JOURNAL OF PEDIATRICS 559

Fig. 1.

Fig. 2. CT scan of the patients showing lucen- cies in the basal ganglion

1-2 days wi thout headache , vomit ing, sei- zu r e or a l tered sensor ium. One day after r e cove ry of fever, he was not iced to have diff icul ty in walking. Within 3 months, he d e v e l o p e d st i ffening and twisting move- m e n t of left foot. After ano ther 3 months , similar symptoms started in right foot also. S y m p t o m s p rog re s sed for ano ther 2-3 m o n t h s and became Static thereafter . No associated sensory symptoms or sphincter- ic involvement was present. There was no pas t h is tory of jaundice, similar illness or other neurological disease. No family histo-

ry of similar illness or o ther neurologica l disease. No family his tory of similar illness was present . He was seen by us 9 months after the start of illness.

On examinat ion, his h igher mental functions was normal. Cranial nerves were normal. Dystonic pos tur ing was noticed in both lower limbs, more on left side causing invers ion, p lantar f lexion of feet and extension of great toes. There were minimal chor ie fo rm m o v e m e n t s not iced in all 4 limbs. Reflexes and plantar responses were normal.

Routine haematological alld biochelnical invest igat ions were normal . Serum cer ru lop lasmin and c o p p e r levels were within normal limits. Slit lamp examination for KF ring was negative. Non-contrast CT scan showed bi lateral lucencies in basal ganglia.

DzSCUSSION

The three patients descr ibed here suffered f rom dystonia , i nvo lv ing one or more limbs, following, short febrile illness with- out neurological s y m p t o m s such as altered consciousness , h e a d a c h e or seizures and associated with basal ganglia lucencies on CT scan without any other lesion elsewhere in the brain.

The major et iologies of bilateral basal ganglia lucencies are subacute necrot is ing e n c e p h a l o m y e l o p a t h y (Leigh 's Disease). Bra ins tem is affected in abou t 98% of pa- tients with subacute necrolising encephalo- myelopa thy . Among all the repor ted cases of Leigh's disease, absence of brainstem in- v o l v e m e n t has been seen in only two cas- es 2'3. The diagnosis of Leigh 's disease was unl ikely in our pat ients as no abnormal i ty outside the striatum clinically or radiologi- cally was apparent. Also, there was only a

560 THE INDIAN JOURNAL OF PEDIATRICS 1996; VoL 63. No. 4

single episode, there be ing no recurrences on follow up. The tempora l relationship be- tween an acute febrile illness and non pro- gressive, non relapsing illness excluded the l?ossibilities of d e g e n e r a t i v e or metabol ic etiologies.

Preceding febrile illness and the onset of neurological signs is cons is ten t with some k ind of encephal i t ic process . Ten cases of bilateral basal ganglia lucencies, following febrile illness have been described m litera- ture. In some of these cases viral serology for rattraps, in f luenza , Echo 25 virus are positiveL

Virological s tud ies on CSF or se rum were not p e r f o r m e d in the pat ients descr ibed here as t hey all p r e sen t ed 5-12 months, following acute febrile illness. The possibility of striatal necrosis being a result of an encephali t ic p rocess is suppor ted by the descr ip t ion of a f ew cases of encephali t is of basal ganglia in monkeys ~,6 and by a r epo r t of a h u m a n case of unilateral s t r iated i n v o l v e m e n t associated ,:Tith an Echo 25 virus infectionL Goutieres and Aicardi (1982) r epo r t ed three patients with acute neuro log ica l dys func t ion e:ssociated with b i la te ra l basal ganglia destruct ion, fo l lowing febri le illness 8. The impor t an t fea tures of these pat ients was acute febrile illness, non- local izable to CNS, tot lowed wi th in a week, by dystonia involving all four l imbs, morn f requent ly the lower limbs. The dys ton ia p rogressed over a few days to few m o n t h s and then became static. The CT scan showed character is t ic lucencies in basal ganglion. Thot~gh the size of the CT lesion became

smaller, as seen in one patient, there was no clinical improvement .

The three patients described above prob- ably constitute a clinically and radiological- ly d is t inc t subgroup of d i so rde r that pro- duces bilateral striatal necrosis in children. The cause of the syndrome is unknown.

REFERENCES

1. Friede RL. Developmental Neuropatholo- gy. Vienna, Springer-Verlag, 1975; 88-89.

2. Bargeton-Farkas E, Cochard AM, Bris- saud HE et al; Encephalopathic infantile famiiiate avec necrose bilateral et sym- metrique des corps stries. I Neurol Sci 1961; 1 : 429-445.

3. Reye RDK. Subacute necrotising encephalomyelopathy. ] Pathol Bacterial 1960; 79 : 165-173.

4. Harike WA, Donohue WK. Bilateral symmetrical necrosis of corpora striata, report of a fatal case and reference to a possible syndrome of the corpora striata. ] Nero ment Dis 1951; 113 : 20-39.

5. Richter R. Encephalitis affecting the basal ganglia in Monkeys.-] Neuropathol Exp Neurol 1945; 4 : 16-26.

6. Van Boagert L. Studies on spontaneous primary encehalitides in the monkey. 1 t encephalitis with a corticostriate predilec- tion in the baboon (Papio bamadryas). ] Neuropatho Exp Neurol 1952; 18 : 306-312.

7. Peters ACB, Vielvoye GJ, Versteeg Jet aL Echo 25 focal encephalitis and subacute hemichorea. Neurology (NY), 1979; 29 : 676-681.

8. Goutieres F, Aicardi J. Acute Neurological Dysfunction associated with destructive lesions of the basal Ganglia in children. Ann of NeurohNy 1982; 12 (No. 4) : 328- 332.