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Jeavons Syndrome: Clinical Features and Response to Treatment
Kelsey M Smith1, Paul E Youssef1, Elaine C Wirrell1
1Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Pediatric Neurology
|August 8, 2018
Summary
Jeavons syndrome, characterized by eyelid myoclonia and closure-induced seizures, often leads to drug-resistant epilepsy. Early diagnosis and identifying seizure types like generalized tonic-clonic seizures are crucial for better management.
Area of Science:
- Neurology
- Epileptology
Background:
- Jeavons syndrome is an underreported epilepsy syndrome.
- It presents with eyelid myoclonia, eye-closure-induced seizures/EEG changes, and photosensitivity.
- Prognostic factors for drug resistance in Jeavons syndrome are not well understood.
Purpose of the Study:
- To characterize the clinical course and prognostic factors of Jeavons syndrome.
- To identify factors associated with drug-resistant epilepsy in this population.
Main Methods:
- Retrospective chart review of 30 Jeavons syndrome patients (2000-2016).
- Analysis of epilepsy history, antiepileptic drug (AED) trials, and treatment responses.
- Evaluation of diagnostic delay and seizure types.
Main Results:
- Mean age at onset was 7.3 years; 80% were female.
- Diagnosis was delayed by an average of 9.6 years.
- 80% had drug-resistant epilepsy; generalized tonic-clonic seizures (GTCS) and non-absence seizures predicted drug resistance (P=0.049, P=0.03).
- Valproic acid, lamotrigine, ethosuximide, and levetiracetam showed >50% seizure reduction.
Conclusions:
- Delayed diagnosis is common in Jeavons syndrome.
- GTCS and other non-absence seizure types are predictors of drug-resistant epilepsy.
- Certain AEDs demonstrate efficacy in managing Jeavons syndrome seizures.
Keywords:
Drug-resistant epilepsyEpilepsyEyelid myoclonia with or without absenceGeneralized epilepsyJeavons syndromeMore Related Videos
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