A Case of Frontotemporal Dementia with Motor Neuron Disease.
- Author:
Seong Hye CHOI
1
;
Duk L NA
;
Byoung Joon KIM
;
Jung Il LEE
;
Yeon Lim SUH
;
Sang Eun KIM
Author Information
1. Department of Neurology, Samsung Medical Center, College of Medicine, Sung Kyun Kwan University.
- Publication Type:Case Report
- Keywords:
frontotemporal dementia;
motor neuron disease;
PET
- MeSH:
Akinetic Mutism;
Amnesia;
Amyotrophic Lateral Sclerosis;
Aphasia;
Atrophy;
Biopsy;
Brain;
Deglutition Disorders;
Delusions;
Dementia;
Extremities;
Female;
Frontal Lobe;
Frontotemporal Dementia*;
Glucose;
Humans;
Impulsive Behavior;
Magnetic Resonance Imaging;
Middle Aged;
Motor Neuron Disease*;
Motor Neurons*;
Neurons;
Neuropsychological Tests;
Pneumonia;
Temporal Lobe
- From:Journal of the Korean Neurological Association
1998;16(4):557-562
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Dementia with motor neuron disease is a rare subtype of frontotemporal dementia. A 59-year-old woman presented with a twenty month history of abnormal behaviors and progressive cognitive decline. Abnormal behaviors consisted of emotional unconcern, persecutory delusion, distractibility, impulsivity, wondering and excessive laughing. Neuropsychological tests showed profound frontal lobe dysfunction out of proportion to amnesia, aphasia, and visuospatial dysfunction. Brain MRI showed subtle atrophy of both frontal lobes. However, brain FDG-PET showed glucose hypometabolism of bilateral frontal and anterior temporal lobes. An open brain biopsy of right frontal lobe demonstrated nonspecific findings including neuronal loss and microvacuolation with reactive astrogliosis in the absence of Alzheimer changes or Pick body. Four months later she developed amyotrophic lateral sclerosis starting with dysphagia. Her condition had continued to deteriorate, resulting in akinetic mutism and marked limb atrophy by the time she died of pneumonia three years postonset.
