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July 1984

Familial Type IIa Hyperlipoproteinemia Associated With a Huge Intracranial Xanthoma

Author Affiliations

From the First Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan. Dr Akazawa is now at Northwestern University School of Medicine, Chicago.

Arch Neurol. 1984;41(7):793-794. doi:10.1001/archneur.1984.04050180119032

Hypercholesterolemia tends to be associated with xanthoma, commonly occurring in the extensor tendons. The present report deals with an unusual intracranial xanthoma in a patient with familial type IIa hyperlipoproteinemia, coexisting with curable hypertension.

REPORT OF A CASE  A 29-year-old man with homozygous familial type IIa hyperlipoproteinemia was admitted to the Nagasaki (Japan) University Hospital in May 1977 because of occipital headache and multiple xanthomas. Tendon xanthomas had been noted in the ankle region at the age of 2 years and subsequently appeared in the fingers, elbows, and knees. Cutaneous xanthomas also developed in the buttock region. At the age of 22 years, when the patient consulted an orthopedist for the removal of xanthomas, hypercholesterolemia and hypertension were initially diagnosed. Approximately four years later, he suffered a bout of occipital headache in the early morning.On admission, the BP was 180/100 mm Hg; temperature, pulse, and respirations were normal. His

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