作者: T. A. Wilson , B. A. Alford , L. Morris
DOI: 10.1001/ARCHNEUR.1982.00510230059019
关键词:
摘要: Lipodystrophy is characterized by a partial or complete absence of body fat in association with propensity to develop insulin-resistant, nonketotic diabetes mellitus. At least three clinical entities share this phenotype: (1) total congenital lipodystrophy, (2) acquired and (3) lipodystrophy. The first inherited as an autosomal recessive trait, while the latter two forms occur sporadically. 1,2 Although etiology lipodystrophy unknown, presence lipolytic factor pituitary hypothalamic origin has been suspected. 2-6 similarities between diencephalic syndrome, which also decreased adipose tissue usually associated CNS tumor located diencephalon, 7 suggest possibility that structural lesion may be involved. In initial report generalized pneumonencephalograms showed dilated venticles enlarged basal cisterns patients. 8 A postmortem examination on another