Teaching NeuroImages: Nonfluent variant primary progressive aphasia

A 66-year-old woman presented with 4 years of progressive speech difficulty. She had nonfluent speech with phonemic errors but intact single-word comprehension and object knowledge. Her grammar was impaired in both speech and writing, and she exhibited orofacial apraxia. A clinico-radiologic (see figure) diagnosis of nonfluent variant primary progressive aphasia was made.

Figure. Magnetic resonance image.

Coronal volumetric T1-weighted MRI showing asymmetric atrophy of left insula and opercular inferior frontal gyrus (arrow), a pattern typical of nonfluent variant primary progressive aphasia.

Nonfluent variant primary progressive aphasia is a neurodegenerative disease within the spectrum of frontotemporal dementia, characterized by the typical language and brain atrophy patterns seen here.¹ It is most frequently due to tau pathology, and clinicians should be alert to the potential development of progressive supranuclear palsy or corticobasal syndrome.²

AUTHOR CONTRIBUTIONS

C.R.M.: image selection and drafting of the manuscript. C.J.D.H., M.N.R., J.D.W.: critical revisions of the manuscript.

STUDY FUNDING

The Dementia Research Centre is grateful for the support of the NIHR Queen Square Dementia Biomedical Research Unit. The Article Processing Charge was paid by Wellcome Trust.

DISCLOSURE

C. Marshall is funded by a clinical fellowship from the Leonard Wolfson Experimental Neurology Centre at UCL. C. Hardy holds an MRC PhD studentship. M. Rossor is an NIHR investigator. J. Warren is a Wellcome Trust Senior Fellow. Go to Neurology.org for full disclosures.