Abstract
BackgroundPrimary progressive apraxia is a rare form of apraxia in the absence of dementia which develops insidiously and is slowly progressive. Most reports of patients with apraxia also describe coexisting aphasias or involve additional apraxias with affected speech, usually in the setting of neurodegenerative diseases such as corticobasal degeneration, Alzheimer’s disease or frontotemporal dementia. The aim of this report is to describe and demonstrate by video two cases of isolated primary progressive ideomotor apraxia seen in our clinic.Case presentationWe describe two patients with 2–5 years of progressive difficulty using their hands, despite having intact cognition and lack of correlating lesions on imaging.ConclusionWe report two cases of primary progressive apraxia that may be early presentations of taupathic disease in both patients. In both cases, there is isolated profound ideomotor apraxia of the hands, with preserved cognition, language skills, muscle power and tone, and gait. There are no correlating lesions on imaging.
Highlights
Primary progressive apraxia is a rare form of apraxia in the absence of dementia which develops insidiously and is slowly progressive
Primary progressive apraxia is a rare form of apraxia which develops insidiously in the absence of dementia, and is slowly progressive
The concept was described by Mesulam in 1982 in patients who demonstrated development and slow progression of aphasia in the absence of dementia [1, 2]
Summary
Primary progressive apraxia is a rare form of apraxia in the absence of dementia which develops insidiously and is slowly progressive. Most reports of patients with apraxia describe coexisting aphasias or involve additional apraxias with affected speech, usually in the setting of neurodegenerative diseases such as corticobasal degeneration, Alzheimer’s disease or frontotemporal dementia. The aim of this report is to describe and demonstrate by video two cases of isolated primary progressive ideomotor apraxia seen in our clinic. Primary progressive apraxia is a rare form of apraxia which develops insidiously in the absence of dementia, and is slowly progressive. The concept was described by Mesulam in 1982 in patients who demonstrated development and slow progression of aphasia in the absence of dementia [1, 2]. Radiologic investigations in the few reported cases of primary progressive apraxias and in cases of primary progressive aphasias generally show left hemispheric atrophy and involvement of the dominant parietal lobe [5, 7, 8]
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