Progressive Auditory Verbal Agnosia Secondary to Alzheimer Disease

A 70-year-old left-handed woman reported 2–3 years of word-finding difficulties, impaired verbal comprehension, and unimpaired hearing. She independently performed daily living activities and did not meet dementia criteria (Montreal Cognitive Assessment 23). Examination revealed hesitant speech, mild anomia without word/object knowledge loss, and a normal writing sample. Written command–following ability was preserved with verbal command–following impaired. Spared environmental sound recognition vs difficulty with spoken words indicated auditory verbal agnosia/pure-word deafness.¹ Neuroimaging revealed β-amyloid–positive PET, focal atrophy on MRI, and focal flortaucipir uptake indicating tau accumulation in primary auditory cortex (Brodmann areas 41/42) known as the Heschl gyrus (Figure).

Figure. Multimodal Neuroimaging in a Patient With Progressive Auditory Verbal Agnosia.

(A) Reduced [¹⁸F]-fluorodeoxyglucose uptake in the left superolateral temporal lobe. (B) Focal atrophy of the left Heschl gyrus on MRI (arrow). (C) Peak flortaucipir uptake in left Heschl gyrus with mild uptake in right Heschl gyrus. SUVR = standardized uptakevalueratio.

Appendix. Authors

Study Funding

NIH grant R01 AG50603.

Disclosure

M. Buciuc reports no competing interests. K.A. Josephs, D.T. Jones, J.F. Whitwell, and J. Graff-Radford receive research support from the NIH. J. Graff-Radford also serves on the editorial board of Neurology®. Go to Neurology.org/N for full disclosures.