Primary progressive aphasia (PPA) is a heterogenous group of neurodegenerative disorders,  characterised by progressive language difficulties. The clinical classification criteria of non-fluent/agrammatic (nfvPPA) phenotype requires either agrammatism in language production or apraxia of speech. There may also be impaired understanding of complex syntax, but preserved object knowledge and preserved single word comprehension. This may be supported by radiological features of left posterior frontoinsular atrophy or hypometabolism. It may be confirmed by detecting a pathogenic mutation or a post-mortem diagnosis, of which Tauopathy is the most common pathology.

An 81-year-old right-handed retired journalist presented with a 1-year history of his speech “feeling broken” and every word being “hard to get out”. There were no other cognitive or behavioural symptoms. Clinical examination revealed a motor speech impairment characterised by loss of prosody and monotonous, effortful and hesitant speech. Naming, comprehension, repetition and writing were preserved. CSF for neurodegenerative biomarkers were not supportive of Alzheimer’s disease. MRI brain scan showed mild right-sided predominant frontal atrophy. 18-FDG-PET brain scan revealed mild bilateral frontal hypometabolism, greater on the right-side. This was suggestive of nfvPPA phenotype with atypical right-sided predominant imaging features. Treatment is supportive. Speech and language therapy advised reading aloud, writing articles, and continuing social interactions.

We present a case of an 81-year-old right-handed man with a nfvPPA phenotype and atypical right-sided predominant frontoinsular atrophy and hypometabolism. The language network is typically lateralised to the left-hemisphere in most individuals, especially right-handed people. This raises the question of a possible ‘crossed’ nfvPPA - described - whereby there is right-sided or bilateral language representation. Functional MRI would be needed to further assess this. A timely accurate diagnosis enables patient-specific supportive care and allows for future care planning decisions.