A new case of idiopathic hemiplegia hemiconvulsion syndrome.
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.
Franzoni
Emilio
E
Child Neuropsychiatry Unit, Paediatric Department, University of Bologna, Via Massarenti 11, Bologna, Italy.
Garone
Caterina
C
Marchiani
Valentina
V
Brunetto
Daniela
D
Tonon
Caterina
C
Lodi
Raffaele
R
Bernardi
Bruno
B
D001284:Atrophy; D001929:Brain Edema / Q000150:complications / Q000378:metabolism / Q000473:pathology; D020938:Epilepsy, Partial, Motor / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D006429:Hemiplegia / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D003294:Seizures, Febrile / Q000150:complications / Q000378:metabolism / Q000473:pathology; D013577:Syndrome
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