Speech and Language Presentations of FTLD-TDP Type B Neuropathology

Abstract Four right-handed patients who presented with an isolated impairment of speech or language had transactive response DNA-binding protein of 43 kDa (TDP-43) type B pathology. Comportment and pyramidal motor function were preserved at presentation. Three of the cases developed axial rigidity a...

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Bibliographic Details
Published in:Journal of neuropathology and experimental neurology 2020-03, Vol.79 (3), p.277-283
Main Authors: Lee, Daniel J, Bigio, Eileen H, Rogalski, Emily J, Mesulam, M -Marsel
Format: Article
Language:English
Subjects:
Anomia - complications
Anomia - pathology
Aphasia, Broca - complications
Aphasia, Broca - pathology
Apraxia
Apraxias - complications
Apraxias - pathology
Brain - pathology
Diagnosis
DNA binding proteins
DNA-Binding Proteins - metabolism
Frontotemporal dementia
Frontotemporal Lobar Degeneration - complications
Frontotemporal Lobar Degeneration - pathology
Frontotemporal Lobar Degeneration - psychology
Health aspects
Humans
Inclusion Bodies - metabolism
Inclusion Bodies - pathology
Language disorders
Male
Middle Aged
Motor neurone disease
Original
Pathology
Speech disorders
TDP-43 Proteinopathies - complications
TDP-43 Proteinopathies - pathology
TDP-43 Proteinopathies - psychology
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Summary:Abstract Four right-handed patients who presented with an isolated impairment of speech or language had transactive response DNA-binding protein of 43 kDa (TDP-43) type B pathology. Comportment and pyramidal motor function were preserved at presentation. Three of the cases developed axial rigidity and oculomotor findings late in their course with no additional pyramidal or lower motor neuron impairments. However, in all 4 cases, postmortem examination disclosed some degree of upper and lower motor neuron disease (MND) pathology in motor cortex, brainstem, and spinal cord. Although TDP-43 type B pathology is commonly associated with MND and behavioral variant frontotemporal dementia, it is less recognized as a pathologic correlate of primary progressive aphasia and/or apraxia of speech as the presenting syndrome. These cases, taken together, contribute to the growing heterogeneity in clinical presentations associated with TDP pathology. Additionally, 2 cases demonstrated left anterior temporal lobe atrophy but without word comprehension impairments, shedding light on the relevance of the left temporal tip for single-word comprehension.
ISSN:0022-3069
1554-6578
DOI:10.1093/jnen/nlz132