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November 23, 2015: Immunotherapy responsive seizures

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November 23, 2015

Immunotherapy responsive seizures

Faciobrachial dystonic seizures are observed in a type of limbic encephalitis that is associated with non-paraneoplastic antibodies against leucine-rich glioma inactivated-1 protein, or LGI1, which is a component of the voltage-gated potassium channel complex. The seizures are of adult-onset, brief, and with frequent stereotyped posturing movements that typically involve the arm and ipsilateral face1-3. MRI is generally unremarkable, but T2 hyperintensities in the medial temporal lobe and basal ganglia have been described1-3. CSF is also generally nonspecific1-3, and intra-ictal focal slowing and epileptiform discharges can be seen on scalp EEG; however, many of these episodes are not associated with electrographic abnormalities1. These seizures are refractory to antiepileptics but are dramatically responsive to immunotherapy1-3. Some studies have suggested that early treatment of faciobrachial dystonic seizures can prevent future cognitive dysfunction by preventing its progression to limbic encephalitis.3

  1. Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Annals of Neurology 2011; 69: 892-900.
  2. Boesebeck F, Schwarz O, Dohmen B, et al. Faciobrachial dystonic seizures arise from cortico-subcortical abnormal brain areas. Journal of Neurology 2013; 260: 1684-1686.
  3. Irani SR, Stagg CJ, Schott JM, et al. Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Brain 2013: 136: 3151-3162.

Submitted by Ahmed Z Obeidat, MD, Ph.D, PGY-3, Department of Neurology, University of Cincinnati.

Disclosures: Dr. Obeidat reports no disclosures

For more clinical pearls and other articles of interest to neurology trainees, visit Neurology. Listen to this week's Neurology Podcast.

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