Unexpected cerebrovascular catastrophe in Guillain–Barré syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is an uncommon neurovascular complication reported with Guillain-Barré syndrome (GBS). A woman in her 30s developed rapidly progressive quadriparesis and areflexia consistent with GBS. Two plasma-exchange procedures were performed but discontinued because of haemodynamic intolerance, after which intravenous immunoglobulin was administered and progression of weakness subsequently ceased, although residual limb weakness persisted at the time of transfer to neurorehabilitation. Approximately 1 week later, she returned with right-leg weakness and new pyramidal signs. MRI showed multifocal acute infarcts in bilateral distal anterior cerebral artery and right cerebellar territories, diffuse postcontrast leptomeningeal/sulcal enhancement and multifocal narrowing of the anterior and posterior intracranial circulation. No thunderclap headache was reported. She subsequently developed further multifocal infarction, diffuse cerebral oedema, herniation and cerebral circulatory arrest. Because angiographic reversibility could not be demonstrated before death, we describe this as probable fulminant RCVS. The case highlights a catastrophic, diagnostically challenging RCVS phenotype in GBS and the need for urgent cerebrovascular imaging when new central neurological signs appear.