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Clinical and Pathological Studies

James R. Schimschock, MD; Ellsworth C. Alvord, Jr., MD; Phillip D. Swanson, MD

Arch Neurol. 1968;18(6):688-698.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

TENDINOUS and tuberous xanthomas have been almost exclusively associated with hyperlipidemic states. Such lesions have been studied intensively for the past 100 years, but only in very rare instances are they associated with similar xanthomatous lesions of the central nervous system (CNS). In 1937, van Bogaert et al¹ described two paternal cousins with cholesterinosis involving tendons, lung, and the CNS. The autopsy findings in the first patient were reported in the original monograph,¹ but the second patient did not die until 1949.² Biopsies of tendons in two additional cases were described by Epstein,^3,4 but no studies of brain or serum lipids were available.

We recently had an opportunity to study two individuals with symptoms and physical findings similar to those originally reported by van Bogaert et al.¹ One of our patients died, permitting pathological verification of this rare disease. In our cases, serum cholesterol levels . . . [Full Text PDF of this Article]

Author Affiliations
Seattle

From the Division of Neurology and Laboratory of Neuropathology, University of Washington School of Medicine, Seattle.

Footnotes
Submitted for publication Dec 29, 1967; accepted Feb 9, 1968.

Reprint requests to Division of Neurology, University of Washington School of Medicine, Seattle 98105 (Dr. Swanson).

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