Progressive agrammatic aphasia without apraxia of speech as a distinct syndrome

Katerina A. Tetzloff, Joseph R. Duffy, Heather Clark, Rene Utianski, Edythe A. Strand, Mary Margaret Machulda, Hugo Botha, Peter R. Martin, Christopher Schwarz, Matthew L. Senjem, Robert I. Reid, Jeffrey L. Gunter, Anthony J. Spychalla, David S Knopman, Ronald Carl Petersen, Clifford R Jr. Jack, Val Lowe, Keith Anthony Josephs, Jennifer Lynn Whitwell

Research output: Contribution to journalArticle

Abstract

Agrammatic aphasia affects grammatical language production and can result from a neurodegenerative disease. Although it typically presents with concomitant apraxia of speech, this is not always the case. Little is known about the clinical course and imaging features of patients that present with agrammatism in the absence of apraxia of speech, which we will refer to as progressive agrammatic aphasia. We aimed to make a detailed description of the longitudinal clinical, linguistic, and neuroimaging features of a cohort of 11 patients with progressive agrammatic aphasia to provide a complete picture of this syndrome. All patients underwent detailed speech and language, neurological and neuropsychological assessments, 3 T structural and diffusion tensor imaging MRI, 18F-fluorodeoxyglucose and Pittsburgh compound B PET. The 11 patients were matched by age and gender to 22 patients who had mixed apraxia of speech and agrammatism. The progressive agrammatic aphasia patients performed abnormally on tests of language, general cognition, executive function, and functional ability at baseline and declined in these measures over time. Only two patients eventually developed apraxia of speech, while parkinsonism was absent-to-mild throughout all visits for all patients. When compared to the patients with mixed apraxia of speech and agrammatism, the patients with progressive agrammatic aphasia performed better on tests of motor speech and parkinsonism but more poorly, and declined faster over time, on tests of general aphasia severity, agrammatism, and naming. The patients with progressive agrammatic aphasia also showed different neuroimaging abnormalities, with greater atrophy, hypometabolism and white matter tract degeneration in the prefrontal and anterior temporal lobes compared to patients with mixed apraxia of speech and agrammatism. These differences were more pronounced as the disease progressed. These results demonstrate that progressive agrammatic aphasia has a different clinical disease course and different underlying neuroanatomical abnormalities than patients with the more common syndrome of mixed agrammatism and apraxia of speech. This supports the distinction of progressive agrammatic aphasia and has implications for the classification of patients with agrammatic aphasia.

Original languageEnglish (US)
Pages (from-to)2466-2482
Number of pages17
JournalBrain : a journal of neurology
Volume142
Issue number8
DOIs
StatePublished - Aug 1 2019

Fingerprint

Apraxias
Aphasia
Broca Aphasia
Parkinsonian Disorders
Neuroimaging
Language
Language Tests
Diffusion Tensor Imaging
Aptitude
Neuropsychological Tests
Executive Function
Fluorodeoxyglucose F18
Temporal Lobe
Linguistics
Neurodegenerative Diseases
Cognition

Keywords

  • agrammatic
  • aphasia
  • longitudinal
  • MRI
  • positron emission tomography

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Progressive agrammatic aphasia without apraxia of speech as a distinct syndrome. / Tetzloff, Katerina A.; Duffy, Joseph R.; Clark, Heather; Utianski, Rene; Strand, Edythe A.; Machulda, Mary Margaret; Botha, Hugo; Martin, Peter R.; Schwarz, Christopher; Senjem, Matthew L.; Reid, Robert I.; Gunter, Jeffrey L.; Spychalla, Anthony J.; Knopman, David S; Petersen, Ronald Carl; Jack, Clifford R Jr.; Lowe, Val; Josephs, Keith Anthony; Whitwell, Jennifer Lynn.

In: Brain : a journal of neurology, Vol. 142, No. 8, 01.08.2019, p. 2466-2482.

Research output: Contribution to journalArticle

Tetzloff, Katerina A. ; Duffy, Joseph R. ; Clark, Heather ; Utianski, Rene ; Strand, Edythe A. ; Machulda, Mary Margaret ; Botha, Hugo ; Martin, Peter R. ; Schwarz, Christopher ; Senjem, Matthew L. ; Reid, Robert I. ; Gunter, Jeffrey L. ; Spychalla, Anthony J. ; Knopman, David S ; Petersen, Ronald Carl ; Jack, Clifford R Jr. ; Lowe, Val ; Josephs, Keith Anthony ; Whitwell, Jennifer Lynn. / Progressive agrammatic aphasia without apraxia of speech as a distinct syndrome. In: Brain : a journal of neurology. 2019 ; Vol. 142, No. 8. pp. 2466-2482.
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