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Author Spotlight: Insight Into Advances in Prion Diseases Research
Published on: August 11, 2023
691
Epilepsy and prion diseases: A narrative review
Gashirai K Mbizvo1, Besa Ziso1, Andrew J Larner1
1Cognitive Function Clinic, The Walton Centre NHS Foundation Trust, Liverpool, United Kingdom.
Epilepsy & Behavior : E&B
|December 14, 2020
Summary
Epileptic seizures are an unusual feature of prion diseases. This review highlights the limited evidence, suggesting seizures occur in about 10% of sporadic cases, with uncertain treatment efficacy.
Area of Science:
- Neurology
- Prion Diseases
- Epilepsy
Background:
- Epileptic seizures are an uncommon clinical manifestation of prion diseases.
- Current understanding is limited due to anecdotal evidence from case studies.
Purpose of the Study:
- To review and synthesize current clinical knowledge on epileptic seizures in various prion diseases.
- To identify gaps in the evidence base regarding seizure characteristics and management.
Main Methods:
- Narrative review of published literature.
- Analysis of case studies and small case series.
Main Results:
- Seizures occur in approximately 10% of sporadic prion disease cases, less in familial/iatrogenic forms (except E200K mutation).
- Focal motor seizures and nonconvulsive status epilepticus are common; EEG may show PLEDs/GPEDs.
- No effective antiepileptic drug therapies are consistently reported.
Conclusions:
- Significant uncertainty exists regarding seizure frequency, semiology, treatment, and pathogenesis in prion diseases.
- Potential mechanisms include loss of cellular prion protein (PrP^c) function and PrP^Sc aggregation.
- Systematic studies and clinical trials are crucial to improve understanding and management.
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