The Japanese journal of neuropsychology

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ArticleTitle	An autopsied case of dementia with Lewy bodies and atrophy of the frontal and temporal lobes
Language	J
AuthorList	Kenji Ishihara¹⁾²⁾, Kiyomi Nagumo²⁾, Jun-ichi Shiota²⁾, Imaharu Nakano³⁾, Mitsuru Kawamura¹⁾
Affiliation	¹⁾Department of Neurology, Showa University School of Medicine ²⁾Department of Neurology, Ushioda General Hospital ³⁾Department of Neurology, Jichi Medical School
Publication	Japanese Journal of Neuropsychology: 27 (1), 64-70, 2011
Received	Jan 6, 2010
Accepted	Apr 6, 2010
Abstract	We report an autopsied case of dementia with Lewy bodies (DLB) and atrophy of the right and left frontal and temporal lobes. A 55-year-old Japanese man had memory impairment and an inability to draw a picture of houses. At approximately the same time, he became inactive and began drinking alcohol. He was admitted to a nursing home where he tended to wander and show signs of aggression. He was also referred to a psychiatrist. The tentative diagnosis was Alzheimer-type senile dementia. At the age of 63 years, he was transferred to our hospital for the treatment of convulsions. Neurological examination revealed severe dementia, mask-like facial expression, lead-pipe rigidity of the extremities, palilalia, pathological grasping of the both hands and myoclonus in the upper extremities. Magnetic resonance images showed apparent symmetrical atrophy of the right and left frontal and temporal lobes. His condition deteriorated gradually, leading to akinetic mutism. He died of pneumonia at the age of 66 years, after a clinical illness of 12 years. Based on the neurological and neuroradiological findings, final clinical diagnosis was frontotemporal dementia. Pathological examination revealed numerous alpha-synuclein-positive Lewy bodies and Lewy neurites in the cerebral cortex, limbic system and brainstem. Alzheimer pathology (neurofibrillary tangles and senile plaques) was observed in the entire cerebral cortex and limbic system; however, these deformities were few in number and did not satisfy the diagnostic criteria of Alzheimer disease. The pathological diagnosis was DLB with Alzheimer pathology. Another case of DLB with atrophy of the frontal and temporal lobes has been reported from Japan. However, the characteristic clinical symptoms of frontotemporal lobar degeneration (FTLD) are not observed in the initial stages. In such cases, DLB should be considered as a differential diagnosis.
Keywords	dementia with Lewy bodies, magnetic resonance images, frontotemporal dementia, brain atrophy