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Headache. 2008 Apr;48(4):632-6. doi: 10.1111/j.1526-4610.2007.01033.x. Epub 2008 Jan 11.

Spontaneous intracranial hypotension syndrome in a patient with marfan syndrome and autosomal dominant polycystic kidney disease.

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  • 1Division of Neuroradiology, Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, and General Practice/Family Medicine, Haseki Education and Research Hospital, Istanbul, Turkey.


Intracranial hypotension is typically manifested by orthostatic headache. The most frequent underlying factor is cerebrospinal fluid leakage. It has been suggested that dural structural weakness in some connective tissue diseases may be responsible for dural tears and diverticula and consequently leakage. We present a case of spontaneous intracranial hypotension associated with Marfan syndrome and autosomal dominant polycystic kidney disease. The patient was treated successfully with epidural autologous blood patch. Dural involvements of these hereditary connective tissue diseases are also discussed.

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