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An autopsy case of Nasu-Hakola's disease (membranous lipodystrophy) is reported. A 43-year-old Japanese man, whose parents were not consanguineous, had been suffering from frequent long bone fractures since the age of 10. Neuropsychiatric symptoms, which were characterized by euphoria, disturbance of attention and dementia, appeared at his thirties and generalized and/or localized seizures and apallial syndrome at the later stage. The neuropathology revealed diffuse leukoencephalopathy of the cerebrum. The peculiar aspects in this case were membranocystic changes in the lungs [Yagishita et al. Virchows Arch [A] 408:211-217 (1985)], diffuse degeneration of the cerebral cortex, chiefly in frontal and temporal lobes, and many axonal spheroids throughout the cerebral cortex. The ultrastructure of spheroids in the cerebral cortex demonstrated aggregations of mitochondria, dense bodies and minute concentric bodies and a small amount of neurofilaments.
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
January 1981,
Acta neuropathologica,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
April 1993,
Der Nervenarzt,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
October 1981,
Pathology, research and practice,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
October 1984,
No to shinkei = Brain and nerve,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
March 1982,
Neurology,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
December 1984,
Masui. The Japanese journal of anesthesiology,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
January 1981,
Seishin shinkeigaku zasshi = Psychiatria et neurologia Japonica,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
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J Kinoshita
January 1991,
Virchows Archiv. A, Pathological anatomy and histopathology,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
January 1985,
Clinical neuropathology,
N Amano, and
K Iwabuchi, and
H Sakai, and
S Yagishita, and
Y Itoh, and
E Iseki, and
S Yokoi, and
N Arai, and
J Kinoshita
January 2003,
Oncology reports,
