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Pilomotor seizures marked by infraslow activity and acetazolamide responsiveness

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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,… Click to show full abstract

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.

Keywords: marked infraslow; infraslow activity; pilomotor seizures; seizures marked; activity

Journal Title: Annals of Clinical and Translational Neurology
Year Published: 2019

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