Abstract

A 52 year old man with a four year history of migraine with aura presented to hospital with a severe right sided headache and prolonged episode of left sided sensory and motor disturbance. Examination revealed left homonymous hemianopia, sensory inattention and hemiparesis (3/5). Reflexes were symmetrical and plantars downing. He had a facial port wine stain in a V1 distribution on the right. Unenhanced CT scan was normal. Migrainous infarction was the working diagnosis and he was commenced on aspirin. Two days later he experienced intermittent episodes of left face and arm twitching consistent with focal motor seizures, treated with clobazam and levetiracetam. EEG showed slowing of background rhythm over the right hemisphere but no epileptiform discharges. Lumbar puncture was unremarkable. MRI on day five revealed subtle, diffuse, abnormal cortical T2 high signal throughout the right cerebral hemisphere with asymmetry of signal change between diffusion weighted imaging and apparent diffusion coefficient sequences. Contrast examination demonstrated diffuse leptomeningeal enhancement over the right hemisphere. MRA was normal. He was diagnosed with Sturge–Weber syndrome on the basis of his V1 distribution port wine stain, stroke like episode and history of hemiplegic migraine. His symptoms fully resolved over the next nine days and he was discharged on levetiracetam and aspirin. Repeat MRI two months later showed improvement of the abnormal right cortical appearances and complete resolution of leptomeningeal enhancement.

Sturge–Weber syndrome is a spectrum disorder–this case highlights the possibility of late onset neurological symptoms and variability of imaging changes.