Broadening the clinical spectrum: unusual presentation of spontaneous cerebrospinal fluid hypovolemia

Case report

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✓ The syndrome of spontaneous intracranial hypotension is characterized by orthostatic headaches in conjunction with reduced cerebrospinal fluid (CSF) pressure or CSF volume, and characteristic magnetic resonance (MR) imaging findings.

A 50-year-old man presented with a 1-year history of paroxysmal ataxia of gait and short attacks of blurred vision when he stood up from a recumbent position and began to walk. Orthostatic headache was not a feature of his clinical presentation. Magnetic resonance images of the brain revealed diffuse enhancement of the dura mater and hygromas over both cerebral convexities. Magnetic resonance images of the spine demonstrated dilated cervical epidural veins and dilation of the perimedullary veins. Radionuclide cisternography identified a CSF leakage that was localized to the T12—L1 level on subsequent myelograms and on computerized tomography scans obtained after the myelograms. An epidural blood patch was administered and visualized with tungsten powder. The patient's clinical symptoms and sites of disease on imaging completely resolved.

The unusual clinical presentation in this case—paroxysmal ataxia of gait, lack of orthostatic headaches, and dilated epidural and perimedullary venous plexus—supports a recently noted broadening of both the clinical and imaging characteristics of spontaneous intracranial hypovolemia.

Article Information

Contributor Notes

Address reprint requests to: Helge Topka, M.D., Ph.D., Department of Neurology and Clinical Neurophysiology, Academic Hospital München—Bogenhausen, Technical University of Munich, Englschalkingerstrasse 77, D-81925 München, Germany. email: topka@extern.lrz-muenchen.de.
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