Abstract
We report a sibling case of Nasu disease. A 35-year-old housewife, whose parents were consanguineous and whose sister died of the same disease, developed dementia, followed by bone fracture, incontinence and convulsions. She died at age 41. Pathologically, characteristic membranocystic changes of the fat cells in bone marrow and adipose tissues were observed. Neuropathologically, there was demyelination associated with intense gliosis and numerous spheroids in the cerebral white matter. At the electron microscope level, these spheroids were an accumulation of various cell organelles. In addition, some had Hirano bodies. Incontinence was correlated with reduction of the number of nerve cells in Onuf's nucleus of the sacral cord.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 38-45 |
| Number of pages | 8 |
| Journal | Clinical Neuropathology |
| Volume | 4 |
| Issue number | 1 |
| State | Published - 1985 |
| Externally published | Yes |
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ASJC Scopus subject areas
- Clinical Neurology
- Pathology and Forensic Medicine
- Neuroscience(all)
Cite this
Membranous lipodystrophy (Nasu disease) : Clinical and neuropathological study of a case. / Minagawa, M.; Maeshiro, H.; Shioda, K.; Hirano, A.
In: Clinical Neuropathology, Vol. 4, No. 1, 1985, p. 38-45.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Membranous lipodystrophy (Nasu disease)
T2 - Clinical and neuropathological study of a case
AU - Minagawa, M.
AU - Maeshiro, H.
AU - Shioda, K.
AU - Hirano, A.
PY - 1985
Y1 - 1985
N2 - We report a sibling case of Nasu disease. A 35-year-old housewife, whose parents were consanguineous and whose sister died of the same disease, developed dementia, followed by bone fracture, incontinence and convulsions. She died at age 41. Pathologically, characteristic membranocystic changes of the fat cells in bone marrow and adipose tissues were observed. Neuropathologically, there was demyelination associated with intense gliosis and numerous spheroids in the cerebral white matter. At the electron microscope level, these spheroids were an accumulation of various cell organelles. In addition, some had Hirano bodies. Incontinence was correlated with reduction of the number of nerve cells in Onuf's nucleus of the sacral cord.
AB - We report a sibling case of Nasu disease. A 35-year-old housewife, whose parents were consanguineous and whose sister died of the same disease, developed dementia, followed by bone fracture, incontinence and convulsions. She died at age 41. Pathologically, characteristic membranocystic changes of the fat cells in bone marrow and adipose tissues were observed. Neuropathologically, there was demyelination associated with intense gliosis and numerous spheroids in the cerebral white matter. At the electron microscope level, these spheroids were an accumulation of various cell organelles. In addition, some had Hirano bodies. Incontinence was correlated with reduction of the number of nerve cells in Onuf's nucleus of the sacral cord.
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UR - http://www.scopus.com/inward/citedby.url?scp=0021961496&partnerID=8YFLogxK
M3 - Article
C2 - 2983919
AN - SCOPUS:0021961496
VL - 4
SP - 38
EP - 45
JO - Clinical Neuropathology
JF - Clinical Neuropathology
SN - 0722-5091
IS - 1
ER -