TY - JOUR
T1 - Speech and Language Presentations of FTLD-TDP Type B Neuropathology
AU - Lee, Daniel J.
AU - Bigio, Eileen H.
AU - Rogalski, Emily J.
AU - Mesulam, M. Marsel
N1 - Funding Information:
From the Mesulam Center for Cognitive Neurology and Alzheimer’s Disease (DJL, EHB, EJR, M-MM); Department of Neurology (DJL, M-MM); Department of Pathology (EHB); and Department of Psychiatry and Be-havioral Sciences (EJR), Northwestern University Feinberg School of Medicine, Chicago, Illinois Send correspondence to: Daniel Lee, MD, Department of Neurology, North-western University Feinberg School of Medicine, 300 E. Superior St., Tarry 8th Floor, Chicago, IL 60611; E-mail: daniel.lee2@northwestern. edu This study was supported by grants from the National Institute on Deafness and Other Communication Disorders (R01 008552) and the National In-stitute on Aging (AG13854), the Davee Foundation, and the Florane and Jerome Rosenstone Fellowship.
Publisher Copyright:
© 2020 American Association of Neuropathologists, Inc. All rights reserved.
PY - 2020/3/1
Y1 - 2020/3/1
N2 - 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.
AB - 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.
KW - Frontotemporal lobar degeneration
KW - Motor neuron disease
KW - Primary progressive aphasia
KW - Speech apraxia
KW - TAR DNA-binding protein
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U2 - 10.1093/jnen/nlz132
DO - 10.1093/jnen/nlz132
M3 - Article
C2 - 31995205
AN - SCOPUS:85080851615
VL - 79
SP - 277
EP - 283
JO - Journal of Neuropathology and Experimental Neurology
JF - Journal of Neuropathology and Experimental Neurology
SN - 0022-3069
IS - 3
ER -