Document Detail

Spectrum of epilepsy in Wilson's disease with electroencephalographic, MR imaging and pathological correlates.
MedLine Citation:
PMID:  20116809     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Seizures are uncommon in Wilson's disease. OBJECTIVE: To analyze profile of seizures in WD and to correlate with EEG and MR imaging observations. SUBJECTS AND METHODS: 41/490 patients (8.3%) of WD were documented to have seizures. Autopsy observations were available in 3 cases. RESULTS: The age at onset of seizures was 12.8+/-5.7years. Seizure - preceded the onset of characteristic features of WD (19.5%); occurred concurrently (46.3%); or, followed de-coppering therapy (29.2%) and occurred as terminal event (4.8%). The types of seizures were: generalized tonic-clonic - 29, simple partial - 8, complex partial - 6, partial seizures with secondary generalization - 2 and periodic myoclonus - 1. Six patients had multiple seizure types and 4 had status epilepticus. EEG abnormalities were frequent (19/24) consisting of background slowing and epileptiform discharges. MRI (n=20) revealed varying degree of atrophy and signal changes involving basal ganglia, brainstem and white matter. The duration of follow-up was 8.1+/-9.2years. The outcome of seizure was: no recurrence - 68.3%, breakthrough seizures - 17.1%, poor control - 9.7% and no follow-up - 4.9%. Two of them succumbed following cluster attacks. Autopsy revealed cavitatory lesions in white matter in frontal, temporal and parietal areas with varying involvement of cortical ribbon. Patients with seizures had more often white matter changes than those without. It was also noted that patients whose seizures were not controlled had MRI suggestive of cavitation of white matter, though the reverse was not true. CONCLUSIONS: This is the largest series regarding epilepsy in WD. Seizures are not uncommon and could occur at any stage. Deafferentation of white matter tracts from cortex may contribute for seizure in WD.
L K Prashanth; S Sinha; A B Taly; A Mahadevan; M K Vasudev; S K Shankar
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Publication Detail:
Type:  Journal Article     Date:  2010-02-08
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  291     ISSN:  1878-5883     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-03-08     Completed Date:  2010-06-17     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  44-51     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Elsevier B.V. All rights reserved.
Department of Neurology, NIMHANS, Bangalore, India.
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MeSH Terms
Brain / pathology*,  physiopathology*
Child, Preschool
Epilepsy / complications,  pathology*,  physiopathology*
Follow-Up Studies
Hepatolenticular Degeneration / complications,  pathology*,  physiopathology*
Magnetic Resonance Imaging
Retrospective Studies
Young Adult

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