Lipodystrophy is characterized by a partial or complete absence of body fat in association with a propensity to develop insulin-resistant, nonketotic diabetes mellitus. At least three clinical entities share this phenotype: (1) total congenital lipodystrophy, (2) total acquired lipodystrophy, and (3) partial acquired lipodystrophy. The first is inherited as an autosomal recessive trait, while the latter two forms occur sporadically.1,2
Although the etiology of lipodystrophy is unknown, the presence of a lipolytic factor of pituitary or hypothalamic origin has been suspected.2-6 The clinical similarities between lipodystrophy and the diencephalic syndrome, which is also characterized by decreased adipose tissue and is usually associated with a CNS tumor located in the diencephalon,7 suggest the possibility that a structural hypothalamic lesion may be involved. In the initial report of congenital generalized lipodystrophy, pneumonencephalograms showed dilated venticles and enlarged basal cisterns in three patients.8 A postmortem examination on another
Wilson TA, Alford BA, Morris L. Cerebral Computed Tomography in Lipodystrophy. Arch Neurol. 1982;39(11):733. doi:10.1001/archneur.1982.00510230059019
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