Open AccessPilomotor seizures marked by infraslow activity and acetazolamide responsiveness
Author(s) -
Wennberg Richard,
Maurice Catherine,
Carlen Peter L.,
Garcia Dominguez Luis
Publication year - 2019
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.695
Subject(s) - medicine , acetazolamide , anesthesia , hyperventilation , ictal , electroencephalography , epilepsy , exacerbation , midbrain reticular formation , neuroscience , midbrain , psychology , central nervous system , psychiatry
A patient with pilomotor seizures post anti‐ LGI 1 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, CO 2 / 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.