Non-convulsive status epilepticus of frontal origin in mucopolysaccharidosis type II successfully treated with ethosuximide

Paolo Bonanni, Marco Gubernale, Federica Martinez, Giovanna Randazzo, Luca Milantoni, Andrea Martinuzzi, Clementina Boniver, Marilena Vecchi, Maurizio Scarpa

Research output: Contribution to journalArticlepeer-review

Abstract

At 7years of age, a female with mucopolysaccharidosis type II (MPS II) showed a sudden deterioration in neurological function, a sleep disorder, and progressive behavioural impairment. Electroencephalography was performed 1year and 8months after the onset of the neurological regression and revealed continuous ictal activity in the frontal regions. The female was diagnosed as having frontal non-convulsive status epilepticus. After 5weeks of therapy with ethosuximide, the ictal electroencephalographic activity disappeared. At the same time, her sleep and cognitive and behavioural functions were observed to improve. This is the first case of MPS type II reported in an individual with non-convulsive status epilepticus. Two main forms of MPS II can be recognized: attenuated and severe. Severe MPS II is characterized by neurodegeneration. No study has explored the relationship between epilepsy and neurological deterioration in MPS II. Our observation shows that epilepsy may be a treatable cause of neurological regression in individuals with MPS II.

Original languageEnglish
Pages (from-to)961-964
Number of pages4
JournalDevelopmental Medicine and Child Neurology
Volume54
Issue number10
DOIs
Publication statusPublished - Oct 2012

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience

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