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Klingler, Jan-Helge M.D.; Gläsker, Sven M.D.; Shah, Mukesch J. M.D.; Van Velthoven, Vera M.D.

doi: 10.1227/01.NEU.0000344002.21699.A3
Case Reports

OBJECTIVE: Presentation of a patient with acute subarachnoid hemorrhage from a ruptured spinal artery aneurysm attributable to exacerbated Sjögren syndrome.

CLINICAL PRESENTATION: A 46-year-old woman with symptoms of exacerbated Sjögren syndrome experienced the acute onset of extreme headache accompanied by nuchal rigidity.

INTERVENTION: A computed tomographic scan revealed subarachnoid hemorrhage. Angiography showed an isolated aneurysm of a branch of the right vertebral artery that was a feeding artery of the anterior spinal artery. Neither operative clipping nor endovascular coiling of the aneurysm was reasonable, owing to the high risk of occluding the anterior spinal artery during the intervention. Further diagnostic measures confirmed Sjögren syndrome and revealed cryoglobulinemic vasculitis, membranoproliferative glomerulonephritis with acute renal failure, Hashimoto thyroiditis, and acute hydrocephalus. In the course of conservative treatment, the patient recovered completely from the subarachnoid hemorrhage. One year after treatment with glucocorticoids and immunosuppressive agents, both the aneurysm and the vasculitis could no longer be detected on conventional angiography.

CONCLUSION: Generally, spinal artery aneurysms are exceptionally rare, and few cases of rupture with subsequent subarachnoid hemorrhage have been published. We report on a ruptured spinal aneurysm attributable to Sjögren syndrome–associated cryoglobulinemic vasculitis. Conservative treatment with glucocorticoids and immunosuppressive agents led to resolution of the vasculitic spinal aneurysm.

Department of Neurosurgery, Freiburg University Medical Center, Freiburg, Germany

Reprint requests: Jan-Helge Klingler, M.D., Department of Neurosurgery, Freiburg University Medical Center, Breisacher Strasse 64, D-79106 Freiburg, Germany. Email:

Received, October 21, 2008.

Accepted, December 11, 2008.

Copyright © by the Congress of Neurological Surgeons