Primary progressive apraxia: an unusual ideomotor syndrome
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CASE REPORT
Open Access
Primary progressive apraxia: an unusual ideomotor syndrome Yeva M. Fernandez* and Steven J. Frucht
Abstract Background: 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 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 presentation: We describe two patients with 2–5 years of progressive difficulty using their hands, despite having intact cognition and lack of correlating lesions on imaging. Conclusion: We 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. Keywords: Apraxia, Tauopathy, Ideomotor
Background Apraxia is a disruption of ability to perform skilled movements in the absence of sensory, motor or language deficits. Primary progressive apraxia is a rare form of apraxia which develops insidiously in the absence of dementia, and is slowly progressive. Personality, behavior and comprehension are preserved. Originally, 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]. Primary progressive apraxia is usually a presenting symptom of a neurodegenerative disease, as reported by Fukui et al. [3]. There are many types of limb apraxias, including ideational/conceptual apraxia, and limb kinetic apraxia, but this case report will focus mainly on the ideomotor apraxia seen in our patients [4]. Ideomotor apraxia refers to a disconnection between the motor system and stored information essential to coordinating, sequencing and organizing appropriate movements relative to space, sometimes referred to as an engram [5]. The concepts of tool utilization and learned skills are preserved, however there is failure to carry out specific goal oriented * Correspondence: [email protected] Mount Sinai, 5 East 98 street 1st floor, New York, NY 10029, USA
movements, or the goal is met with irregular movements [5, 6]. In addition, there is a dissociation between the ability to carry out tasks voluntarily versus involuntarily [6]. 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].
Case presentations Case 1
A 72- year- old right handed retired hairdresser presented with 5 years of progressive isolated difficulty with hand movements.
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