Abstract

A 51-year-old woman presented with a 10 day history of left shoulder pain which was managed with paracetamol and codeine. On straining the next day she developed a severe headache which necessitated assessment in hospital. After a normal CT scan and lumbar puncture she was sent home with a diagnosis of migraine. She subsequently had recurrent severe daily headaches for the next 5 days and was prescribed pizotifen and sumatriptan by her GP. Her headaches deteriorated and she developed sustained visual phenomena resulting in re-evaluation. Her examination revealed marked visuospatial disorientation including a partial Balint's syndrome in addition to signs indicative of more anterior cortical involvement. MRI demonstrated extensive altered signal intensity bilaterally in the occipital lobes extending to the right precentral gyrus. MR angiography and subsequent catheter angiogram showed diffuse abnormalities of the intracranial vessels of both the anterior and posterior circulation with short segments of narrowing involving both main and cortical branches. She was treated with steroids and calcium channel blockers. Brain biopsy did not show any evidence of cerebral vaculitis. Her headaches improved and she underwent rehabilitation. Repeat angiography at 3 months was normal consistent with a diagnosis of reversible cerebral vasoconstrictive syndrome. We present this rare syndrome along with a literature review and discussion of the difficulties of its management.