An Autopsy Case of Frontotemporal Dementia with Motor Neuron Disease.

Author: Eun Joo KIM ¹ ; Eun Hye OH ; Ki Tae KIM ; Yoori JUNG ; Jeong Hee LEE ; Jae Hyeok LEE ; Young Min LEE ; Seong Jang KIM ; Jin Hong SHIN ; Myung Jun SHIN ; Myung Jun LEE ; Jae Woo AHN ; Suk SUNG ; Kyung Un CHOI ; Dae Soo JUNG ; William W SEELEY ; Gi Yeong HUH
Author Information

1. Department of Neurology, Pusan National University School of Medicine, Yangsan, Korea.
Publication Type:Case Report
Keywords: Frontotemporal dementia; Motor neuron disease; Transactive response DNA-binding protein
MeSH: Autopsy*; Diagnosis; Frontotemporal Dementia*; Frontotemporal Lobar Degeneration; Humans; Korea; Motor Neuron Disease*; Motor Neurons*; Neurites; Pathology
From:Journal of the Korean Neurological Association 2015;33(3):201-205
CountryRepublic of Korea
Language:Korean
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.