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Myoclonic encephalopathy and diabetes mellitus in a boy

Published online by Cambridge University Press:  01 July 1999

C Lenti ,
E Bognetti ,
R Bonfanti ,
E Bonifacio  and
F Meschi
C Lenti
Affiliation:
Institute of Neurological and Psychiatric Sciences of Childhood and Adolescence, University of Milan , Milan, Italy .
E Bognetti
Affiliation:
Paediatric Department, Scientific Institute HS Raffaele, University of Milan, Milan, Italy.
R Bonfanti
Affiliation:
Paediatric Department, Scientific Institute HS Raffaele, University of Milan, Milan, Italy.
E Bonifacio
Affiliation:
Department of Medicine, Scientific Institute HS Raffaele, University of Milan, Milan, Italy.
F Meschi
Affiliation:
Paediatric Department, Scientific Institute HS Raffaele, University of Milan, Milan, Italy.
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Abstract

We describe an 18-month-old boy with insulin-dependent diabetes mellitus who developed idiopathic myoclonic encephalopathy (dancing eye syndrome) at 26 months of age. The neurological symptomatology (multifocal myoclonus, opsoclonus, ataxia, behavioural disturbance) developed within 10 to 14 days after presentation. Biological, neuroradiological, and scintigraphic examination excluded CNS infectious diseases, intoxication, or tumours. At onset of diabetes mellitus, anti-glutamic-acid decarboxylase (GAD) antibodies were observed, and markedly increased in titre when myoclonic encephalopathy occurred. Corticosteroid treatment resulted in a decrease in anti-GAD autoantibody titres and the disappearance of neurological disturbances. As GAD is expressed both in pancreatic β-cells and cerebellar Purkinje cells, it is possible that a common autoimmune disorder in this patient may account for both the diabetes and myoclonic encephalopathy.

Type
Case Reports
Information
Developmental Medicine and Child Neurology , Volume 41 , Issue 7 , July 1999 , pp. 489 - 490
Copyright
© 1999 Mac Keith Press

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