Reversible cerebral vasoconstriction syndrome presenting with haemorrhage

Abstract

Reversible cerebral vasoconstriction syndromes are characterized by prolonged but reversible vasoconstriction of the cerebral arteries, presenting typically with thunderclap headache, with or without focal neurological signs or symptoms. They resemble primary central nervous system vasculitis but it is critical to differentiate these two entities. Here we draw attention to intracranial haemorrhage as an important and not uncommon clinical feature in reversible cerebral vasoconstriction syndrome. Four patients with reversible cerebral vasoconstriction syndrome, each presenting to a single unit with intracranial haemorrhage, are described. These descriptions of haemorrhage at presentation of RCVS extend the recognised clinical phenotype and so help to enhance recognition and diagnosis of this often unconsidered disorder.

Introduction

In 1988 Call and Fleming reported four patients with what appeared to be a reversible form of cerebral vasoconstriction, with no recognisable underlying cause, superficially resembling primary angiitis of the central nervous system (PACNS) in both its clinical and angiographic features[1]. Since then a number of authors have reported reversible cerebral vasoconstriction syndromes (RCVS), often in association with potential aetiological precipitants (such as sympathomimetic drugs; Table 1); in perhaps a third, no underlying cause is found[2], [3].

Reversibility and a benign outcome of course only become apparent in time, and initially there is commonly a tendency to presume a diagnosis of cerebral vasculitis, influenced principally by the neuroradiological appearances. RCVS is under-recognised, and yet distinction from PACNS is essential to avoid unnecessary and hazardous use of long-term immunosuppressant and cytotoxic agents. Subtle historical, clinical and investigational features – ‘pattern recognition’ – therefore assume considerable importance, and fundamental to this is a sound knowledge of the clinical spectrum. Haemorrhage as a common feature of this syndrome is not frequently reported. Here, we present four cases illustrating this condition which presented with haemorrhage, broadening the range of features of the unusual disorder.