Frontal impairment and hypoperfusion in neuroacanthocytosis
F. Delecluse, J. Deleval, J. M. Gerard, A. Michotte and D. Zegers de Beyl
Neurology Service, Erasme Hospital, University of Brussels, Belgium.
Cerebral blood flow tomography, by xenon 133 inhalation or HMPAO (99mTc-d,
l-hexamethyl-propylene amine oxime) technetium Tc 99m injection, revealed a
severe hypoperfusion in both frontal lobes of a 40-year-old woman with
confirmed neuroacanthocytosis. This finding occurred in conjunction with
neuropsychological deficits consistent with selective frontal lobe
dysfunction. This observation is the first documentation of this type of
dementia in neuroacanthocytosis.