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Atypical case of hemiconvulsions-hemiplegia-epilepsy syndrome revealing contralateral focal cortical dysplasia

Published online by Cambridge University Press:  16 November 2005

Nadia Bahi-Buisson
Affiliation:
Department of Neuropaediatrics and Metabolic Diseases, Necker Hospital for Sick Children, Paris, France.
Manoelle Kossorotoff
Affiliation:
Department of Neurophysiology, Necker Hospital for Sick Children, Paris, France.
Christine Barnerias
Affiliation:
Department of Neuropaediatrics and Metabolic Diseases, Necker Hospital for Sick Children, Paris, France.
Nathalie Boddaert
Affiliation:
Department of Paediatric Neurology, Necker Hospital for Sick Children, Paris, France.
Marie Bourgeois
Affiliation:
Department of Paediatric Neurosurgery, Necker Hospital for Sick Children, Paris, France.
Olivier Dulac
Affiliation:
Department of Neuropaediatrics and Metabolic Diseases, Necker Hospital for Sick Children, Paris, France.
Perrine Plouin
Affiliation:
Department of Neurophysiology, Necker Hospital for Sick Children, Paris, France.
Catherine Chiron
Affiliation:
Department of Neuropaediatrics and Metabolic Diseases, Necker Hospital for Sick Children, Paris, France.
Lucie Hertz-Pannier
Affiliation:
Department of Paediatric Radiology, Necker Hospital for Sick Children, Paris, France.
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Abstract

Hemiconvulsions-hemiplegia-epilepsy syndrome (HH/HHE) is a rare epileptic syndrome consisting of a prolonged unilateral convulsion producing a persisting hemiplegia, sometimes followed by epilepsy. We report on a 13-month-old male who presented with febrile left-sided HH syndrome with right hemispheric unilateral cytotoxic oedema followed by hemispheric atrophy on magnetic resonance imaging (MRI). Six months later the child progressively developed refractory focal epilepsy, including right hemiclonic seizures, and nearly continuous left frontal rhythmic spikes, suggesting the presence of a focal cortical dysplasia (FCD). A repeat MRI at 2 years of age showed left frontal FCD. This unusual case of dual pathology – right HH syndrome and left FCD – suggests that some other factor than the malformation determined the prolonged status and brain atrophy. The kinetics of regional cortical maturation could explain this unusual condition.

Type
Case Report
Copyright
© 2005 Mac Keith Press

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