Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2017 Nov 14:4:17.
doi: 10.1186/s40734-017-0064-0. eCollection 2017.

Primary progressive apraxia: an unusual ideomotor syndrome

Yeva M Fernandez  1 , Steven J Frucht  1
Affiliations
Case Reports

Primary progressive apraxia: an unusual ideomotor syndrome

Yeva M Fernandez et al. J Clin Mov Disord. .

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; Ideomotor; Tauopathy.

PubMed Disclaimer

Conflict of interest statement

All patients that participated in this publication have signed consent for filming and publication.The patients have signed consent for filming and publication. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that we do not have any investments or competing interests. Steven J Frucht is Editor-in-Chief of Journal of Clinical Movement Disorders. He was not involved in handling this manuscript.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

References

    1. Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann Neurol. 1982;11(6):592–598. doi: 10.1002/ana.410110607. - DOI - PubMed
    1. Riley DE, et al. Cortical-basal ganglionic degeneration. Neurology. 1990;40(8):1203–1212. doi: 10.1212/WNL.40.8.1203. - DOI - PubMed
    1. Fukui T, et al. Primary progressive apraxia in Pick's disease: a clinicopathologic study. Neurology. 1996;47(2):467–473. doi: 10.1212/WNL.47.2.467. - DOI - PubMed
    1. Leiguarda RC, Marsden CD. Limb apraxias: higher-order disorders of sensorimotor integration. Brain. 2000;123(Pt 5):860–879. doi: 10.1093/brain/123.5.860. - DOI - PubMed
    1. Rapcsak SZ, et al. Progressive ideomotor apraxia: evidence for a selective impairment of the action production system. Brain Cogn. 1995;27(2):213–236. doi: 10.1006/brcg.1995.1018. - DOI - PubMed

Publication types

LinkOut - more resources