J Korean Neurol Assoc.  2015 Aug;33(3):201-205. 10.17340/jkna.2015.3.12.

An Autopsy Case of Frontotemporal Dementia with Motor Neuron Disease

Affiliations
  • 1Department of Neurology, Pusan National University School of Medicine, Yangsan, Korea.
  • 2Department of Pathology, Pusan National University School of Medicine, Yangsan, Korea.
  • 3Department of Psychiatry, Pusan National University School of Medicine, Yangsan, Korea.
  • 4Department of Nuclear Medicine, Pusan National University School of Medicine, Yangsan, Korea.
  • 5Department of Rehabilitation Medicine, Pusan National University School of Medicine, Yangsan, Korea.
  • 6Department of Anatomy, Pusan National University School of Medicine, Yangsan, Korea.
  • 7Department of Forensic Medicine, Pusan National University School of Medicine, Yangsan, Korea. gyhuh@pusan.ac.kr
  • 8Memory and Aging Center, University of California San Francisco, CA, USA.

Abstract

Approximately 15% of patients with frontotemporal dementia (FTD) have co-occurring motor neuron disease (MND). FTD-MND cases have frontotemporal lobar degeneration (FTLD)-transactive response DNA-binding protein (TDP) pathology, which is divided into four subtypes (types A, B, C, and D) based on the morphological appearance, cellular location, and distribution of the abnormal TDP inclusions and dystrophic neurites. We report a patient with FTD-MND whose pathological diagnosis was FTLD-TDP type B. This is the first documented autopsy-confirmed case of FTD-MND in Korea.

Keyword

Frontotemporal dementia; Motor neuron disease; Transactive response DNA-binding protein

MeSH Terms

Autopsy*
Diagnosis
Frontotemporal Dementia*
Frontotemporal Lobar Degeneration
Humans
Korea
Motor Neuron Disease*
Motor Neurons*
Neurites
Pathology
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