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Journal of Neurology

07-04-2024 | Apraxia | Original Communication

Progression to corticobasal syndrome: a longitudinal study of patients with nonfluent primary progressive aphasia and primary progressive apraxia of speech

Authors: Danna P. Garcia-Guaqueta, Hugo Botha, Rene L. Utianski, Joseph R. Duffy, Heather M. Clark, Austin W. Goodrich, Nha Trang Thu Pham, Mary M. Machulda, Matt Baker, Rosa Rademakers, Jennifer L. Whitwell, Keith A. Josephs

Published in: Journal of Neurology

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Abstract

Background and objectives

Nonfluent variant primary progressive aphasia (nfvPPA) and primary progressive apraxia of speech (PPAOS) can be precursors to corticobasal syndrome (CBS). Details on their progression remain unclear. We aimed to examine the clinical and neuroimaging evolution of nfvPPA and PPAOS into CBS.

Methods

We conducted a retrospective longitudinal study in 140 nfvPPA or PPAOS patients and applied the consensus criteria for possible and probable CBS for every visit, evaluating limb rigidity, akinesia, limb dystonia, myoclonus, ideomotor apraxia, alien limb phenomenon, and nonverbal oral apraxia (NVOA). Given the association of NVOA with AOS, we also modified the CBS criteria by excluding NVOA and assigned every patient to either a progressors or non-progressors group. We evaluated the frequency of every CBS feature by year from disease onset, and assessed gray and white matter volume loss using SPM12.

Results

Asymmetric akinesia, NVOA, and limb apraxia were the most common CBS features that developed; while limb dystonia, myoclonus, and alien limb were rare. Eighty-two patients progressed to possible CBS; only four to probable CBS. nfvPPA and PPAOS had a similar proportion of progressors, although nfvPPA progressed to CBS earlier (p-value = 0.046), driven by an early appearance of limb apraxia (p-value = 0.0041). The non-progressors and progressors both showed premotor/motor cortex involvement at baseline, with spread into prefrontal cortex over time.

Discussion

An important proportion of patients with nfvPPA and PPAOS progress to possible CBS, while they rarely develop features of probable CBS even after long follow-up.
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Metadata
Title
Progression to corticobasal syndrome: a longitudinal study of patients with nonfluent primary progressive aphasia and primary progressive apraxia of speech
Authors
Danna P. Garcia-Guaqueta
Hugo Botha
Rene L. Utianski
Joseph R. Duffy
Heather M. Clark
Austin W. Goodrich
Nha Trang Thu Pham
Mary M. Machulda
Matt Baker
Rosa Rademakers
Jennifer L. Whitwell
Keith A. Josephs
Publication date
07-04-2024
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-024-12344-x