Myoclonic encephalopathy and diabetes mellitus in a boy

C. Lenti, E. Bognetti, R. Bonfanti, E. Bonifacio, F. Meschi

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)489-490
Number of pages2
JournalDevelopmental Medicine and Child Neurology
Volume41
Issue number7
DOIs
Publication statusPublished - 1999

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Neuroscience(all)
  • Developmental Neuroscience

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