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Pranshu Sharma, Aseem Sharma and Ari G. Chacko

✓ The authors describe the presence of a syrinx in a patient with spontaneous intracranial hypotension. The likely pathophysiological basis of this hitherto unreported association is also presented. It is believed that chronic obstruction at the foramen magnum may be the most important factor for the development of syringes.

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Daniel Refai, James A. Botros, Russell G. Strom, Colin P. Derdeyn, Aseem Sharma and Gregory J. Zipfel

Object

The clinical characteristics and overall outcome in patients with spontaneous isolated convexity subarachnoid hemorrhage (SAH) are not well described in the literature. The purpose of this study was to examine the mode of presentation, common origins, radiographic findings, and clinical course in a large case series of such patients.

Methods

A retrospective single-center chart review of all patients in whom nontraumatic primary convexity SAH was diagnosed between 2002 and 2007 was performed. Twenty patients were identified and analyzed for presenting symptoms, radiological and laboratory findings, hospital course, and outcome.

Results

There were 15 women and 5 men in our series, and the mean age was 52 years (range 18–86 years). The most common presenting symptom was headache, with 15 patients experiencing it as a chief complaint. Other frequent manifestations included altered mental status (8 patients), focal neurological deficits (7), and seizure (4 patients). An underlying cause of the hemorrhage was identified in 13 cases, whereas the remainder went unresolved. Of the known causes, 5 were due to posterior reversible encephalopathy syndrome, 3 were caused by thrombocytopenia or anticoagulation, and the remainder were isolated cases of lupus vasculitis, drug-induced vasculopathy, postpartum cerebral angiopathy, hypertensive microangiopathy, and Call–Fleming syndrome. All patients with unknown disease origins had favorable outcomes, whereas 8 of 13 patients with an identifiable underlying disorder experienced favorable outcomes.

Conclusions

Spontaneous isolated convexity SAH is rarely caused by aneurysm rupture, has a distinct mode of presentation, and generally carries a more favorable prognosis than that of aneurysmal SAH.