Bilateral temporal lobe epilepsy confirmed with intracranial EEG in chorea-acanthocytosis.
Chorea-acanthocytosis (ChAc) is an uncommon basal ganglia disorder, in which the movement disorder element may be obscured by the predominance of seizures. We report a pertinent case of a patient who had undergone extensive evaluation for epilepsy, including intracranial EEG before finally the diagnosis of ChAc was made and confirmed by Western blot. We suggest that in patients with epilepsy, particularly of temporal lobe origin and with onset in the third decade with inconclusive findings on clinical examination and neuroimaging such as dyskinesias, dystonia and basal ganglia involvement, ChAc should be considered.
Neurologische Klinik und Poliklinik, Ludwig-Maximilians-Universität, Marchioninistr. 15, 81377 München, Germany. benedikt.bader@med.uni-muenchen.de
Benedikt
B
Bader
Christian
C
Vollmar
Nibal
N
Ackl
Anne
A
Ebert
Christian
C
la Fougère
Soheyl
S
Noachtar
Adrian
A
Danek
D000328:Adult; D003937:Diagnosis, Differential; D004569:Electroencephalography; D020803:Encephalitis, Herpes Simplex / Q000473:pathology / Q000503:physiopathology; D004833:Epilepsy, Temporal Lobe / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D054546:Neuroacanthocytosis / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D049268:Positron-Emission Tomography; D015899:Tomography, Emission-Computed, Single-Photon