Atraumatic nonaneurysmal sulcal subarachnoid hemorrhages: a diagnostic workup based on a case series

Cerebrovasc Dis. 2012;34(2):147-52. doi: 10.1159/000339685. Epub 2012 Aug 8.

Abstract

Introduction: Atraumatic and nonaneurysmal sulcal subarachnoid hemorrhage (sSAH) is a rare type of cerebrovascular disease with various etiologies previously reported in small case reports. In this study, we propose to analyze clinical presentations, imaging patterns and etiologies in a large case series of such patients in order to propose a diagnostic workup.

Methods: We retrospectively analyzed clinical and radiological data of consecutive patients with a diagnosis of atraumatic and nonaneurysmal sSAH, admitted to our institution between 2008 and 2011. All patients had both computed tomography (CT) and magnetic resonance imaging (MRI) as a part of their initial evaluation.

Results: 30 patients (18 women and 12 men, mean age: 60 years) were identified. The main clinical symptoms at presentation were focal and transient neurological deficit (n = 22) and thunderclap headache (n = 10). Four patients had progressive headache and 4 other had partial or generalized epileptic seizures. MRI abnormalities associated with sSAH were prior hemorrhages, microbleeds, severe leukoencephalopathy and hemosiderosis suggesting cerebral amyloid angiopathy (CAA; n = 9), vasogenic edema in parieto-occipital areas compatible with a posterior reversible encephalopathy syndrome (PRES; n = 3), cortical venous thrombosis (n = 2) and concomitant acute cortical stroke (n = 3). Other underlying causes of sSAH, not diagnosed on MRI, were reversible cerebral vasoconstriction syndrome (RCVS) based on clinical criteria and conventional angiography (n = 4), angiitis diagnosed by skin biopsy (n = 1), vascular malformation diagnosed on CT and digital subtraction angiographies (n = 3), and overanticoagulation (n = 1). Four cases remained unresolved.

Conclusion: This study confirmed that sSAH is a rare condition related to a wide spectrum of etiologies. Combination of brain MRI and magnetic resonance angiography and eventually digital subtraction angiography allowed the identification of an underlying etiology for 87% of patients. CAA, RCVS and PRES represented more than 50% of the etiological mechanisms. Among older patients, sSAH was mainly related to CAA while in younger patients, RCVS represented the most frequent etiology.

MeSH terms

  • Aged
  • Cerebral Amyloid Angiopathy / complications
  • Cerebral Amyloid Angiopathy / diagnosis
  • Cerebral Hemorrhage / complications
  • Epilepsy, Generalized / etiology
  • Female
  • France / epidemiology
  • Headache / etiology
  • Hemosiderosis / etiology
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Middle Aged
  • Posterior Leukoencephalopathy Syndrome / complications
  • Posterior Leukoencephalopathy Syndrome / diagnosis
  • Recurrence
  • Retrospective Studies
  • Subarachnoid Hemorrhage / diagnosis*
  • Subarachnoid Hemorrhage / diagnostic imaging
  • Subarachnoid Hemorrhage / epidemiology
  • Subarachnoid Hemorrhage / etiology
  • Subarachnoid Hemorrhage / pathology
  • Tomography, X-Ray Computed*
  • Vasospasm, Intracranial / complications
  • Venous Thrombosis / complications