J Korean Neurol Assoc.  2018 Feb;36(1):35-39. 10.17340/jkna.2018.1.8.

An Autopsy Confirmed Case of Semantic Variant Primary Progressive Aphasia with Frontotemporal Lobar Degeneration-TDP type C

Affiliations
  • 1Department of Neurology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea.
  • 2Department of Neurology and Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea. eunjookim@pusan.ac.kr
  • 3Department of Psychiatry, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea.
  • 4Department of Rehabilitation Medicine, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea.
  • 5Department of Nuclear Medicine, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea.
  • 6Department of Pathology, Pusan National University School of Medicine, Yangsan, Korea.
  • 7Department of Anatomy, Pusan National University School of Medicine, Yangsan, Korea.
  • 8Department of Forensic Medicine, Pusan National University School of Medicine, Yangsan, Korea. gyhuh@pusan.ac.kr

Abstract

A 62-year-old man presented with a one-year history of word finding difficulty, impaired single word comprehension and personality changes including aggression, apathy and eating change. Brain MRIs showed severe atrophy in the left anterior temporal lobe. The clinical syndromic diagnosis was semantic variant primary progressive aphasia. He died at age 70 of pneumonia. At autopsy, transactive response DNA-binding protein (TDP) immunoreactive long dystrophic neurites were predominantly found in the cerebral cortices, which were compatible with frontotemporal lobar degeneration-TDP type C pathology.

Keyword

Semantic dementia; Frontotemporal lobar degeneration; TDP-43 proteinopathies

MeSH Terms

Aggression
Apathy
Aphasia, Primary Progressive*
Atrophy
Autopsy*
Brain
Cerebral Cortex
Comprehension
Diagnosis
Eating
Frontotemporal Dementia
Frontotemporal Lobar Degeneration
Humans
Magnetic Resonance Imaging
Middle Aged
Neurites
Pathology
Pneumonia
Semantics*
TDP-43 Proteinopathies
Temporal Lobe
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