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GAD65-Ab encephalitis and subtle focal status epilepticus
Mathilde Goudot1, Solène Frismand1, Lucie Hopes1
1Department of Neurology, University Hospital of Nancy.
Epileptic Disorders : International Epilepsy Journal with Videotape
|October 26, 2019
Summary
Glutamic acid decarboxylase autoantibody (GAD65-Ab) encephalitis can present with subtle epilepsy features, including focal status epilepticus. Early recognition is crucial as standard EEGs may be normal, potentially delaying diagnosis.
Area of Science:
- Neurology
- Immunology
- Epileptology
Background:
- Glutamic acid decarboxylase autoantibodies (GAD65-Ab) are associated with autoimmune encephalitis.
- Epilepsy is a recognized manifestation, but its subtle features in this context are not well-delineated.
Observation:
- Three patients with GAD65-Ab encephalitis exhibited subtle, new-onset ictal semiology.
- Symptoms included prolonged "déjà vu" states, "dreamy states," transient aphasia, and behavioral disturbances.
- All patients experienced clinical focal status epilepticus, confirmed by EEG.
Findings:
- Patients 1 and 2 had temporo-mesial seizures indicative of limbic encephalitis.
- Patient 3 presented with neocortical seizures, suggesting non-limbic encephalitis.
- High GAD65-Ab levels in CSF confirmed the diagnosis.
Implications:
- Encephalitis is a frequent neurological syndrome in GAD65-Ab disorders.
- Epilepsy may be more common and severe than previously thought.
- Subtle focal status epilepticus is a key semiology in GAD65-Ab encephalitis, necessitating advanced EEG for diagnosis.
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