Reversible Cerebral Vasoconstriction Syndrome with Atypical Subarachnoid Hemorrhage: A First Case Report

Case Report

Austin J Clin Neurol 2015;2(5): 1046.

Reversible Cerebral Vasoconstriction Syndrome with Atypical Subarachnoid Hemorrhage: A First Case Report

Gentile M¹*, De Vito A², Groppo E¹, Tola MR² and Azzini C²

¹Department of Biomedical Sciences and Medical Surgical Sciences, University of Ferrara, Italy

²Department of Neurology, Stroke Unit, Arcispedale S. Anna Ferrara, Italy

*Corresponding author: Mauro Gentile, Department of Biomedical Sciences and Medical Surgical Sciences, University of Ferrara, via Aldo Moro 8, 44123 Ferrara, Italy

Received: April 02, 2015; Accepted: May 05, 2015; Published: May 15, 2015

Abstract

Reversible cerebral vasoconstriction syndrome represents a group of syndromes characterised by prolonged, reversible, segmental vasoconstriction of the cerebral arteries, usually associated with acute, severe, “thunderclap” headache with or without additional focal neurological deficits and seizures. The major complications of this syndrome are localised cortical subarachnoid haemorrhages and ischaemic or haemorrhagic strokes. Subarachnoidhemorrhage represents the most frequent hemorrhagic complication and it is often mild, focal and superficial and involves few high sulci in the convexity. To our knowledge this report represents the first case of atypical subarachnoid hemorrhage localised in the basal cistern associated with reversible cerebral vasoconstriction syndrome.

We report a 52 year old woman presented severe sudden onset occipital headache episodes. The neurological examination revealed only horizontal nystagmus in left sight direction; vital signs were normal. Neuroimaging scans (CT and MRI) showed a mild diffuse cerebral swelling without focal lesions. The following days she a thunderclap type headache recurred acutely and a new brain CT showed subarachnoid hemorrhage in the basal cisterns. Non invasive angiography demonstrated a wide segmental vasoconstriction of the cerebral arteries with dilatations of the post-stenotic segments suggesting reversible cerebral vasoconstriction syndrome. New non invasive angiography obtained after 3 months showed a complete resolution of the previous segmental arterial vasoconstrictions.

Keywords: Reversible cerebral vasoconstriction syndrome; Call-Fleming syndrome; subarachnoid hemorrhage

Introduction

Reversible cerebral vasoconstriction syndrome (RCVS) was first recognized by Call and Fleming in 1988 [1]. RCVS represents a group of syndromes characterized by prolonged, reversible, segmental vasoconstriction of the cerebral arteries, usually associated with acute, severe, “thunderclap” headache with or without additional focal neurological deficits and seizures. It usually resolves in 1-3 months after onset [2].

The disease pathophysiological mechanism is still unknown. However it is possible to recognize a specific cause of RCVS only in 25- 60% of patients as postpartum vasospasm (with or without eclampsia or pre-eclampsia), exposure to vasoactive or immunosuppressant drugs, exposure to blood products, catecholamine secreting tumors, hypercalcemia, porphyria, head trauma, neurosurgery, subdural spinal haematoma, carotid endoarterectomy, cerebral venous thrombosis, CSF hypotension, autonomic dysreflexia, phenytoin intoxication [3], hormonal ovarian stimulation for intrauterine insemination [4], LES [5], tonsillectomy [6], high altitude [7]. Therefore there is still a high percentage of idiopathic cases.

The incidence of this condition is probably underestimated, particularly because of the undiagnosed benign forms without focal neurological deficits or seizures. Females seem to be more affected than males with a 2-10:1 ratio [2,8,9].

The RCVS major complications are localised cortical subarachnoid haemorrhages (SAH) and ischaemic or haemorrhagic strokes [8,10- 14]. The former represents the most frequent RCVS hemorrhagic manifestation [14].

We report a case of RCVS associated with atypical SAH.

Case Presentation

A 52 year old woman was admitted to our Emergency Department because of severe sudden onset occipital headache episodes associated to asthenia in the last ten days. She had a past history of short transient blurred vision episodes occurring in her half visual field (especially the left one) without any other associated symptoms. She did not use any drug.

At admission to our Neurologic Unit the examination revealed only horizontal nystagmus in left gaze. Vital signs, including blood pressure (120/80 mmHg), were normal. Brain computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a mild diffuse cerebral swelling without focal lesions.

In the evening of day 1, while headache was improving, she developed spatial disorientation and mild speech slowdown without other neurological deficits. EEG registration revealed diffuse slow abnormalities with epileptiform focal aspects in the left temporal region.

The following day a thunderclap type headache recurred acutely with pulsating severe occipital pain and mild neck stiffness. To rule out a SAH, a new cerebral CT was performed showing SAH in the basal cisterns with diffuse cerebral swelling. MRI with venous sequences confirmed SAH and excluded cerebral sinuses thrombosis (Figure 1). Non invasive angiography (MR angiography) and invasive angiography demonstrated a wide segmental vasoconstriction of the cerebral arteries with dilatations of the post-stenotic segments (“string of beads” appearance), without aneurysms or other vascular malformations (Figure 2,3).