J Korean Neurol Assoc.  1998 Aug;16(4):557-562.

A Case of Frontotemporal Dementia with Motor Neuron Disease

• Choi SH
• Na DL
• Kim BJ
• Lee JI
• Suh YL
• Kim SE

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.

Keyword

frontotemporal dementia; motor neuron disease; PET

MeSH Terms

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
Glucose