Churg–Strauss Syndrome Presenting as Spontaneous Subarachnoid Haemorrhage

Abstract

Churg-Strauss syndrome (CSS) is a systemic small-vessel vasculitis characterised by the presence of asthma and eosinophilia. Central nervous system involvement (cerebral infarctions or intracerebral haemorrhage) is rare in CSS. Spontaneous subarachnoid hemorrhage (SAH) has been described in other systemic vasculitides. SAH is exceptional in CSS. We present a 47-year-old woman with CSS presenting as a spontaneous SAH with cerebral angiography findings consistent with vasculitis of the basilar artery and without aneurysms or arteriovenous malformations. She received treatment with prednisone and cyclophosphamide, and 2 months later the basilar artery was normal on magnetic resonance angiography.

Subarachnoid haemorrhage as the first clinical manifestation of Churg-Strauss syndrome

“…Intracerebral haemorrhage is rarely associated with CSS [4]. SAH in association with CSS is exceptional, with only six cases of CSS associated with SAH reported [1,2,5,7,8,10]. CNS involvement usually occurs later in the disease course [5], with asthma commonly preceding onset of neurological manifestations [9].…”

Demographic and Clinical Characteristics Associated with Central Nervous System Hemorrhage in Patients with Eosinophilic Granulomatosis with Polyangiitis: A Case Report and Review of the Literature

“…In these cases, cerebral ischemic infarction (~80%) and diffuse encephalopathy (~10%) have been by far the findings most frequently reported. Published data on EGPA with CNS hemorrhage are limited to anecdotal case reports [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]21,22,23,24,25,26 . We described a case of EGPA presenting with intracerebral hemorrhage (ICH) and did a comprehensive review of the literature.…”

Primary central nervous system vasculitis presenting with intracranial hemorrhage

“…In most of the reported cases, followup information was minimal, and it was unclear whether intracranial hemorrhage, in particular subarachnoid hemorrhage, was a manifestation of PCNSV or could be a complication of reversible cerebral vasoconstriction syndrome (RCVS), which is a major mimic of PCNSV (16, 20). Furthermore, cerebral vasculitis associated with other conditions, such as systemic vasculitides, connective tissue diseases, and viral and fungal infections has been reported to cause intracranial hemorrhage, and these conditions must be carefully considered in the differential diagnosis (21–32).…”