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Amyotrophic lateral sclerosis (ALS) is a multi-system disorder. Mild cognitive deficits are present in a subgroup of non-demented patients with ALS. Detailed neuropsychological assessments reveal deficits of word retrieval including impairments on tests of verbal fluency and confrontation naming. The PET GABA(A) receptor ligand [11C]-flumazenil is a marker of neuronal dysfunction in ALS. This study used [11C]-flumazenil PET to identify correlations between cortical regions and impairments in word retrieval. Twelve patients with ALS underwent [11C]-flumazenil PET and neuropsychological assessment, including tests of written letter fluency and confrontation naming. Poorer performance on verbal fluency correlated with decreased [11C]-flumazenil binding in a region including the right inferior frontal gyrus, superior temporal gyrus, and anterior insula. Poorer performance on a test of confrontation naming correlated with decreased binding in the left middle frontal gyrus (extending to Broca's area) and left cuneus. This study indicates that [11C]-flumazenil PET can be used to help localize cortical regions associated with cognitive deficits in ALS.

Original publication




Journal article


Amyotroph Lateral Scler

Publication Date





43 - 49


Adult, Aged, Amyotrophic Lateral Sclerosis, Carbon Radioisotopes, Cognition Disorders, Female, Flumazenil, Humans, Male, Middle Aged, Neurons, Neuropsychological Tests, Positron-Emission Tomography