ABSTRACT
Hemiconvulsion-hemiplegia-epilepsy syndrome is a rare consequence of a status epilepticus in the course of a febrile illness in children under the age of four years. Various degrees of hemiplegia and within a variable interval, subsequent epilepsia follows. Neuroimaging show unilateral cytotoxic oedema at the initial convulsive state, followed by severe chronic atrophy of the affected hemisphere. The aetiology remains unclear. Several mechanisms may contribute to this condition. To improve the outcome, further studies are needed and early diagnosis is essential.
Subject(s)
Epilepsy/diagnosis , Hemiplegia/diagnosis , Status Epilepticus/diagnosis , Brain/diagnostic imaging , Brain/physiopathology , Child, Preschool , Epilepsy/complications , Epilepsy/diagnostic imaging , Epilepsy/drug therapy , Fever/complications , Fever/diagnosis , Hemiplegia/complications , Hemiplegia/diagnostic imaging , Hemiplegia/drug therapy , Humans , Infant , Magnetic Resonance Imaging , Radiography , Rare Diseases , Status Epilepticus/complications , Status Epilepticus/diagnostic imaging , Status Epilepticus/drug therapy , SyndromeABSTRACT
Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a very rare condition caused by a fever-associated status epilepticus causing various degrees of hemiplegia and secondary epilepsy affecting children under the age of four. The aetiology is not fully understood. We report a case of a two-year-old girl with adrenogenital syndrome presenting with HHE syndrome in the course of a bacterial septicaemia.
