Non‐fluent/agrammatic variant of primary progressive aphasia with progressive supranuclear palsy

Abstract

AbstractBackgroundNon‐fluent/agrammatic variante of primary progressive aphasia (nfvPPA) is a neurodegenerative disorder characterized by speech production impairment (1). The overlap of nfvPPA and progressive supranuclear palsy (PSP) and corticobasal syndrome cases have been reported (2). As nfvPPA progresses, a parkinsonian disorder often co‐occurs (3). There are different kinds of language impairment including articulatory deficits, agrammatism, reduced speech rate, and anomia (1). Here we present a Peruvian woman with nfvPPA with single‐photon emission computed tomography (SPECT) and parkinsonian syndrome.MethodWe describe a 73‐year‐old woman with progressive speech production impairment and parkinsonian syndrome. She completed standard neurological, neuropsychological, and speech‐language evaluations, as well as brain MRI and SPECT scan.ResultThe patient is a right‐handed woman with 16 years of education. No family history of dementia, parkinsonism or motor neuron disease was reported. At age 71, she presented progressive difficulty in speaking, anomia and verbal hesitancy; but she can understand conversations, send and read text messages. She also had depressive symptoms. MRI showed mild left frontal and perisylvian > right atrophy (fig 1).By age 73, she developed a prominent reduction in spontaneous speech, mild difficulties in reading and writing, occasional paraphasia, less empathy, dietary change, moria and, mild parkinsonism (she expresses less emotion, tremor, slowly walk and falls). Her episodic memory starts to decline thereafter.On examination, she had mild‐moderate telegraphic speech, imitation behavior, mild facial masking, bradykinesia and mild postural instability but no abnormal extraocular movement. Speech‐language assessment showed non‐fluent aphasia and apraxia of speech. Impaired comprehension of syntactically complex sentences. Naming was mildly impaired. Syntactic errors were present. Executive function was also impaired, but visuoperceptual skills were preserved.Serological analyses were unrevealing. Brain SPECT scan showed left frontal and perisylvian > right cortical hypoperfusion (fig 2).ConclusionWe report a Peruvian case of an elderly woman presenting with a 3‐year history of nfvPPA features and PSP. Her SPECT scan showed left frontal and perisylvian > right cortical hypoperfusion. Consider early evaluating motor function in nfvPPA patients in light of the possibility of CBS or PSP overlap.