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Case Reports
. 2017 Jun 8:8:47-50.
doi: 10.1016/j.ebcr.2017.06.001. eCollection 2017.

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

Rajesh Shankar Iyer  1 , T C R Ramakrishnan  1 , Karunakaran  2 , Ajit Shinto  3 , Koramadai Karuppuswamy Kamaleshwaran  3
Affiliations
Case Reports

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

Rajesh Shankar Iyer et al. Epilepsy Behav Case Rep. .

Abstract

•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.

Keywords: Basalganglial hypermetabolism; Faciobrachial dystonic seizures; Fronto–temporo–basalganglial network; LGI1antibody; Network hypothesis; VGKC antibody encephalitis.

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Figures

Fig. 1
Fig. 1
MRI Brain FLAIR images axial (A) and sagittal (B) of patient 1 showed bilateral medial temporal hyperintensities. MRI FLAIR images axial (C) in two months, follow-up showed diffuse brain atrophy and persistence of medial temporal hyperintensities. PET CT Brain at presentation (D) showed bilateral basal ganglial and amygdalar hypermetabolism. PET CT Brain at two-month follow-up (E) showed bilateral basal ganglial hypermetabolism and disappearance of medial temporal hypermetabolism. Ictal EEG (F,G) showed movement artifacts with the jerk followed by brief attenuation and build-up of right temporal spikes. MRI Brain axial images T2 (H), FLAIR (I) and T1 (J) in patient 2 showed basal ganglia hyperintensities. T2 axial images (K) also showed left sided substantia nigra hyperintensity (white arrow).
Fig. 2
Fig. 2
Schematic diagram showing the fronto–temporo–basal ganglia network (dotted line).
See this image and copyright information in PMC

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