November 23, 2015: Immunotherapy responsive seizures
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
- Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Annals of Neurology 2011; 69: 892-900.
- 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.
- 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