Speech and Language Presentations of FTLD-TDP Type B Neuropathology

Daniel J. Lee*, Eileen H. Bigio, Emily J. Rogalski, M. Marsel Mesulam

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)277-283
Number of pages7
JournalJournal of neuropathology and experimental neurology
Volume79
Issue number3
DOIs
StatePublished - Mar 1 2020

Keywords

  • Frontotemporal lobar degeneration
  • Motor neuron disease
  • Primary progressive aphasia
  • Speech apraxia
  • TAR DNA-binding protein

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Fingerprint

Dive into the research topics of 'Speech and Language Presentations of FTLD-TDP Type B Neuropathology'. Together they form a unique fingerprint.

Cite this