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Published on: April 5, 2011
Status epilepticus in Wilson's disease.
Elia M Pestana Knight1, Sid Gilman, Linda Selwa
1Division of Pediatric Neurology, Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA.
Summary
Status epilepticus is a rare seizure complication in Wilson's disease (WD). This case study details a patient experiencing non-convulsive status epilepticus (NCSE) during Tetrathiomolybdate (TTM) therapy, highlighting seizures may stem from disease progression rather than treatment alone.
Area of Science:
- Neurology
- Neuroscience
- Medical Case Studies
Background:
- Wilson's disease (WD) is a genetic disorder causing copper accumulation.
- Seizures are an uncommon manifestation of WD, with status epilepticus (SE) being particularly rare.
- This study investigates a unique case of non-convulsive status epilepticus (NCSE) in a WD patient undergoing treatment.
Observation:
- A 55-year-old male with WD presented with seizures during week 4 of Tetrathiomolybdate (TTM) therapy.
- Electroencephalogram (EEG) revealed frequent left frontal seizures, progressing to NCSE.
- Brain MRI showed diffuse atrophy, basal ganglia mineralization, and left frontal lobe FLAIR hyperintensities.
Findings:
- The patient developed NCSE, characterized by electrographic seizures every 5-10 minutes.
- Seizures were successfully managed within 24 hours using fosphenytoin, midazolam, and levetiracetam.
- Literature review identified 6 prior cases of SE in WD patients, some before and some after copper-chelation therapy.
Implications:
- This is the first reported case of SE in a WD patient treated with TTM.
- The findings suggest that SE in WD may be multifactorial, potentially related to disease progression or treatment, rather than solely treatment-induced.
- Further research is needed to elucidate the precise mechanisms of seizures in WD and their relationship to various therapeutic interventions.
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