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To the Editor:
I read with interest the article by Scheid et al.,1 who describe 3 patients with chorea-acanthocytosis (ChAc) whose initial symptomatology included partial complex seizures (PCS). The brain MRI, in addition to caudate atrophy, showed mesial temporal sclerosis (MTS) in all 3 cases.
As the authors suggest, it is intriguing that a pathologic process with a predilection for the basal ganglia should cause focal abnormalities in the hippocampal region. The authors posit several theories but do not mention genetic abnormalities, which have been theorized as a contributing factor in MTS.
In addition, since MTS is the most common pathology in temporal lobe seizures, its occurrence in ChAc may be coincidental. The authors doubt the significance of the seizures themselves as a culprit behind MTS in their patients. However, seizures—particularly …
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