Description

A 68-year-old woman was admitted with a 6-day history of sudden onset, throbbing headache associated with transient visual disturbance. On examination, she exhibited right facial numbness and right arm heaviness. Her blood pressure was 207/88 mm Hg. Of note, she had a history of migraines and was taking sumatriptan. She was treated with antihypertensives and her symptoms improved. Initial brain CT was unremarkable. No angiogram was performed at the time.

She further presented 12 days later with another episode of severe headache. Brain CT performed at the time demonstrated a focus of low attenuation in the left occipital lobe (figure 1A). No evidence of subarachnoid haemorrhage (SAH) was present. She subsequently had a brain MRI with MR angiogram (MRA). MRI confirmed left occipital subacute infarct (figure 1B). On MRA, there was definite focal narrowing of the left posterior cerebral artery (PCA) (figure 2A, B), although there was some further doubtful vascular narrowing present. The PCA narrowing was present at a non-branch point and thought to represent either vasculitis or vasospasm. The patient was treated with calcium channel blockers (CCB). She underwent follow-up MRI and MRA after 6 weeks, which demonstrated that the left PCA narrowing had resolved (figure 2C, D), while no new findings were present. A diagnosis of reversible cerebral vasoconstriction syndrome (RCVS) was made.

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Figure 1

Non-contrast CT (A) and MRI fluid-attenuated inversion recovery (B) showing an infarct in left occipital lobe (white arrow).

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Figure 2

Initial CT angiogram ((A) axial, (B) sagittal) showing focal narrowing of left posterior cerebral artery (white arrow). Subsequent CT angiogram 6 weeks later ((C) axial and (D) sagittal) showing that changes have resolved.

During this admission the patient underwent a lumbar puncture (LP), which was normal. Her platelet count was noted to be raised at 826×10⁹/L and she was found to be JAK-2 positive. Following review with the haematology team, she is now being treated for essential thrombocythaemia with hydroxycarbamide and clopidogrel.

At discharge, her symptoms resolved except for a minor right visual field deficit.

RCVS is a well-described entity that results in areas of vasospasm in one or more intracranial arteries, which usually resolve spontaneously.1 ,2 There are a range of causes described, including idiopathic causes, such as migraine due to certain drugs such as sumatriptan, which this patient was also taking. RCVS is an important differential in patients with sudden severe headaches, typically thunderclap headaches. Usually, such patients are primarily investigated for SAH and sinus thrombosis, after exclusion of which they tend to be treated conservatively.1 ,2 Some of these patients can develop complications such as infarcts or haemorrhage; these can happen even if headaches improve on symptomatic treatment. RCVS is specifically treated with CCB. Cerebral angiograms (CT/MR/digital subtraction angiography) if performed, can pick up several such patients and result in appropriate treatment, potentially preventing complications. It is important that more such patients have angiograms, even if SAH has been excluded, to reach the correct diagnosis. The usual differential on angiograms is vasculitis, with the usual helpful differentiating criteria being the reversible nature of RCVS on follow-up. The current case has been presented to emphasise that it is important to consider angiograms in more patients presenting with sudden onset headaches, even when SAH has been excluded, as they can potentially be treated appropriately, possibly preventing complications.