Long-Term Cognitive Outcome of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in Adults.
Abstract number :
3.098
Submission category :
Year :
2001
Submission ID :
941
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
M.C. Harnadek, Ph.D., Clinical Neurological Sciences, London Health Sciences Centre, London, ON, Canada; S.M. Mirsattari, M.D., Clinical Neurological Sciences, London Health Sciences Centre, London, ON, Canada; W.T. Blume, M.D., Clinical Neurological Scie
RATIONALE: The long-term neuropsychological outcome of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome in adults is poorly studied.
METHODS: We describe detailed neuropsychological profiles of 3 female adult patients (mean age = 47 years) with HHE syndrome. All had their febrile seizures characteristic of HHE syndrome before age 2 years. Two out of 3 patients were left handed with right hemiparesis with the remaining patient being right handed with left hemiparesis. All patients had hemiatrophy and unilateral mesial temporal sclerosis (MTS) in the corresponding side of the brain and seizures originating from the related temporal lobe. The habitual seizures developed at age 1 and 12 years in the right hemiparetic patients. The left hemiparetic patient developed habitual seizures at age 7 years. Both right hemiparetic patients had right hemispheric speech representation. The right hemiparetic patient had left hemispheric speech. All patients underwent comprehensive neuropsychological evaluation of their verbal and visual memory, language, visual-spatial skills, and reasoning.
RESULTS: The right hemiparetic patients showed widespread neuropsychological impairment involving verbal and visual memory, language (especially naming), and visual-spatial functioning. One of the two also had impaired verbal and non-verbal reasoning. The left hemiparetic patient also demonstrated bihemispheric impairment characterized by language and visual-spatial dysfunction. In contrast to the right hemiparetic patients, the left hemiparetic patient had normal verbal and visual memory and reasoning. None of the 3 patients were mentally retarded.
CONCLUSIONS: The neuropsychological dysfunction in patients with HHE syndrome is widespread and not limited to the atrophic hemisphere. Right hemiparetic patients with HHE syndrome involving the left hemisphere may have broad range of neuropsychological impairments. This may be related, in part, to the shift of language to the right hemisphere and subsequent [dsquote]crowding-effect[dsquote]. Left hemiparetic patients with HHE syndrome involving the right hemisphere may have a more restricted range of neuropsychological impairments involving both hemispheres. Mental retardation need not be an essential feature of HHE syndrome in adults.