Right Hemisphere Involvement in Non-Fluent Primary Progressive Aphasia

We described a 56-years-old man with a diagnosis of "non-fluent primary progressive aphasia" (NfPPA). An accurate neuropsychological, neurological and neuroimaging evaluation was performed in order to assess clinical and behavioural features of the patient. From a neuropsychological point of view, the patient showed a typical cognitive profile of subjects affected by NfPPA: a prominent language deficit, associated with impairments in several cognitive domains after three years from the onset of the symptomatology. The most intriguing feature is that SPECT revealed hypoperfusion in the right frontal cortex, albeit the patient is right-handed. This unexpected finding shows that NfPPA may arise not only from cortical abnormalities in the language-dominant left hemisphere, but also from right hemisphere involvement in a right hander (crossed aphasia).