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Faciobrachial dystonic seizures result from fronto–temporo–basalganglial network involvement

• Faciobrachial dystonic seizures (FBDS) are caused by autoantibodies to leucine-rich glioma-inactivated1 proteins, a component of the voltage-gated potassium channel complex (VGKC-complex) and precede the clinical presentation of limbic encephalitis. • The exact pathophysiology of FBDS is not known...

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Detalles Bibliográficos
Autores principales: Iyer, Rajesh Shankar, Ramakrishnan, T.C.R., Karunakaran, Shinto, Ajit, Kamaleshwaran, Koramadai Karuppuswamy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5573798/
https://www.ncbi.nlm.nih.gov/pubmed/28879090
http://dx.doi.org/10.1016/j.ebcr.2017.06.001
Descripción
Sumario:• Faciobrachial dystonic seizures (FBDS) are caused by autoantibodies to leucine-rich glioma-inactivated1 proteins, a component of the voltage-gated potassium channel complex (VGKC-complex) and precede the clinical presentation of limbic encephalitis. • The exact pathophysiology of FBDS is not known and whether they are seizures or movement disorder is still debated. • We suggest the fronto-temporo-basal ganglia network involving the medial frontal and temporal regions along with the corpus striatum and substantia nigra being responsible for the clinical phenomenon of FBDS. • The varied clinical, electrical and imaging features of FBDS in our cases and in the literature are best explained by involvement of this network. • Entrainment from any part of this network will result in similar clinical expression of FBDS, whereas other electro-clinical associations and duration depends on the extent of involvement of the network.