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Case Report With Autopsy Verification

Katalin Hegedüs, MD; György Németh, MD

Arch Neurol. 1984;41(4):440-442.

Abstract
• Seven histologically confirmed cases of intracranial fibromuscular dysplasia (FMD) have been published. A new case of intracranial FMD confined to the basilar artery occurred in a 32-year-old man. The proliferation of fibroblasts and smooth-muscle cells could be observed only in the intima detaching from the media. The neurologic symptoms corresponding to akinetic mutism occurred nine days after pulmonary lobectomy for aspergilloma. We believe that this pure intimal type of FMD consisting of cells without elastic and collagen fibers represents a more undifferentiated and therefore more progressive form of the disease. The FMD can be considered as an overwhelming reaction of so-called pluripotential smooth-muscle cells of the arterial wall to various stimuli.

Author Affiliations
From the Department of Neurology and Psychiatry, University Medical School, Debrecen, Hungary.

Footnotes
Accepted for publication June 16, 1983.

Reprint requests to Department of Neurology and Psychiatry, University Medical School, Debrecen H-4012, Hungary (Dr Hegedüs).

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ABSTRACT