Paralysis Research Today is a free monthly online journal that collates and summarizes the latest research about Paralysis, including details on treatment, diagnosis, facial paralysis, sleep paralysis.

Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia: a report with pathophysiological implications.

Auvin S, Devisme L, Maurage CA, Soto-Ares G, Cuisset JM, Leclerc F, Vallée L

Department of Pediatric Neurology, University Hospital, Lille, France. auvin@invivo.edu

The mechanisms underlying the hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) remain unclear. The current proposed pathogenic mechanism is a neuronal injury induced by venous thrombosis and/or hypoxia. Previous abnormalities of the brain were suggested as underlying mechanism. MATERIALS AND METHODS: We report a patient who presented acutely with hemiconvulsion-hemiplegia (HH) syndrome, but unfortunately died. We discuss the possible pathophysiology of the HH syndrome and possible therapeutic implications utilizing the data from neuroimaging and pathological studies. Post-mortem examination was performed including immunohistochemistry and electron microscopy of the brain tissue. RESULTS: The abnormalities in diffusion-weighted imaging indicate cytotoxic edema of the epileptic hemisphere. The pathological studies confirmed a right homogenous hemispheric edema without evidence of any malformation, inflammatory, infectious or metabolic disease. We found axonal damages in the right thalamus confirmed by anti-neurofilament staining. DISCUSSION: The pathological studies suggest that cytotoxic edema is responsible for neuronal damage. In HH syndrome, two mechanisms playing a role in the development of a later epilepsy could suggest delayed cell death induced by cytotoxic edema and/or thalamic dysfunction causing a disruption of thalamo-cortical circuit. In acute presentation, the use of anti-edema therapy should be discussed to prevent the cell injury.

Published 27 April 2007 in Seizure, 16(4): 371-6.
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