Idiopathic hemiconvulsion-hemiplegia syndrome

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Idiopathic hemiconvulsion-hemiplegia syndrome

Synonyms: HHE syndrome | Hemiconvulsion-hemiplegia-epilepsy syndrome | IHHS

A rare acute encephalopathy with inflammation-mediated status epilepticus characterized by infancy-onset of refractory unilateral mainly clonic status epilepticus during or shortly after a febrile episode without evidence of central nervous system infection followed by permanent or transient hemiplegia with a minimum duration of one week. The majority of children develop pharmaco-resistant epilepsy a few months later. Brain imaging shows edematous swelling of the affected hemisphere at the time of the initial status followed by hemiatrophy that does not correlate with any vascular territory.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: // Data version October 2023.

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