Primary progressive apraxia: a syndrome difficult to categorize

Souza, Ricardo Krause Martinez de; Rocha, Samanta Fabricio Blattes da; Cordellini, Marcela Ferreira; Vosgerau, Ronaldo; Kowacs, Pedro André

doi:10.1590/0004-282x20130016

Cited by 1 publication

(3 citation statements)

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“…Of the 35 patients, 22 of these cases had isolated involvement of the hands as a presenting symptom 7 of the 35 cases reported had involvement of upper and lower limbs as a presenting symptom. In rare cases, patients have been reported to initially present with gait abnormality [ 8 ], speech difficulty [ 3 ], and orofacial apraxia [ 12 ]. Interestingly, many of the patients with ideomotor apraxia, including our patients, had an asymmetry in the handedness of their symptoms, with the left hand being reported as the more severely affected hand [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

“…In the cases presented by Fukui et al [ 3 ], the patients had focal atrophy of the left parietal lobe and in some cases, diffuse cortical atrophy. Another case, reported by Martinez de Souza et al [ 8 ], described a woman with both ideational and ideomotor apraxia that presented with apraxic gait and progressive inability to dress or to use her hands. This patient’s brain MRI demonstrated atrophy of the left superior parietal lobe and supplemental motor area.…”

Section: Discussionmentioning

confidence: 99%

“…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 ].…”

Section: Introductionmentioning

confidence: 99%

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Primary progressive apraxia: an unusual ideomotor syndrome

Fernandez

Frucht

2017

J Clin Mov Disord

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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.Electronic supplementary materialThe online version of this article (10.1186/s40734-017-0064-0) contains supplementary material, which is available to authorized users.

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