A new case of idiopathic hemiplegia hemiconvulsion syndrome

作者：Franzoni Emilio; Garone Caterina^*; Marchiani Valentina; Brunetto Daniela; Tonon Caterina; Lodi Raffaele; Bernardi Bruno

来源：Neurological Sciences, 2010, 31(6): 799-805.

DOI：10.1007/s10072-010-0286-5

• Hemiplegia hemiconvulsion
• Epilepsy
• MRI
• Proton MR spectroscopy

We report a new case of infantile idiopathic hemiconvulsion-hemiplegia syndrome (HH). A prolonged right-sided febrile convulsion was followed 4 days later, by right hemiconvulsive status epilepticus, documented by video-electroencephalogram (EEG) recording. The child developed an ipsilateral hemiplegia, partially improved during the first month of follow-up. Sequential cerebral magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H-MRS) at 6, 15, 30 days of follow-up showed a cytotoxic edema in the left hemisphere and a subsequent necrosis. At 1-year of follow-up, we performed MRI control because of febrile convulsion lasting few minutes that confirmed a non-progressive left hemisphere atrophy. After 2 years, the patient was seizure-free, with a mild right hemiplegia and language skills deficit. We discuss the unclear pathogenesis of HH through sequential neuroradiological evaluation.

• 出版日期2010-12