Pilomotor seizures marked by infraslow activity and acetazolamide responsiveness

A patient with pilomotor seizures post anti‐LGI1 limbic encephalitis, refractory to immunotherapy and anti‐epileptic drugs, was investigated with electroencephalography and magnetoencephalography. Seizures occurred daily (14.9 ± 4.9/day), with catamenial exacerbation, inducible by hyperventilation....

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Bibliographic Details
Published in:Annals of clinical and translational neurology 2019-01, Vol.6 (1), p.167-173
Main Authors: Wennberg, Richard, Maurice, Catherine, Carlen, Peter L., Garcia Dominguez, Luis
Format: Article
Language:English
Subjects:
Acetazolamide - therapeutic use
Adult
Anticonvulsants - therapeutic use
Autoantibodies
Brain - drug effects
Brain - physiopathology
Brief Communication
Brief Communications
Convulsions & seizures
Electroencephalography
Female
Humans
Intracellular Signaling Peptides and Proteins - immunology
Limbic Encephalitis - complications
Limbic Encephalitis - immunology
Magnetoencephalography
Seizures - drug therapy
Seizures - etiology
Seizures - physiopathology
Treatment Outcome
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Summary:A patient with pilomotor seizures post anti‐LGI1 limbic encephalitis, refractory to immunotherapy and anti‐epileptic drugs, was investigated with electroencephalography and magnetoencephalography. Seizures occurred daily (14.9 ± 4.9/day), with catamenial exacerbation, inducible by hyperventilation. Anterior temporal ictal onsets were heralded (by ~15 sec) by high amplitude ipsilateral electromagnetic infraslow activity. The catamenial/ventilatory sensitivity and the infraslow activity (reflecting glial depolarization) suggested an ionic, CO2/pH‐related glioneuronal mechanism. Furosemide decreased seizure frequency by ~33%. Acetazolamide led to immediate seizure freedom, but lost efficacy with daily treatment. A cycling acetazolamide regimen (2 days on, 4 days off) plus low‐dose topiramate maintained >95% reduction (0.5 ± 0.9/day) in seizures.
ISSN:2328-9503
2328-9503
DOI:10.1002/acn3.695