A 30-year-old man with Sturge–Weber syndrome (SWS) diagnosed 10 years previously was admitted with a 3 day history of headache, photophobia, drowsiness and progressive left hemiplegia. There was a history of a similar episode in 2004, complicated by a period of generalised tonic–clonic status epilepticus. He had remained seizurefree for some years on levetiracetam 500 mg bd. Examination revealed a port-wine stain in a right trigeminal V1 distribution. He was drowsy and partially oriented, with left lower facial weakness. There was complete left hemiplegia with brisk left-sided deep tendon reflexes, left extensor plantar response and left-sided sensory extinction. There was no acute haemorrhage or infarct on CT brain. CSF white cell count and protein were normal. A T1weighted MRI brain showed dilatation of the deep venous system (Fig. 1a) and pial enhancement following gadolinium, with normal opacification of the superficial venous sinuses (Fig. 1b). There was high diffusion-weighted imaging (DWI) signal limited to the right cerebral cortex (Fig. 1c). EEG showed right-sided slowing but no epileptiform activity. He was treated with aspirin 300 mg/day and tinzaparin 17,000 u/day. Levetiracetam was increased to 1,000 mg bd. The left hemiplegia completely resolved over the next 10 days, and no seizures were observed. He complained of intermittent non-threatening formed visual hallucinations which gradually resolved. Repeat MR showed a resolution in the cortical DWI signal (Fig. 1d). Recurrent stroke-like episodes due to ischaemia or seizures occur frequently in children with SWS [3, 4, 9], but are less well-described in adults. Headaches, mostly with migrainous features, are common and have been associated with stroke-like episodes in one study [6]. Post-ictal Todd’s paresis was also considered as a potential cause of the patient’s symptoms, but the absence of definite seizures and the progressive nature of the deficit were against this, although levetiracetam was increased as a precaution against further deterioration [2]. The deep venous anomalies and pial enhancement we describe are consistent with longstanding vascular changes in SWS [2, 9]. The increased DWI signal over the right cerebral cortex likely represents cortical ischaemia due to microcirculatory stasis within the aberrant leptomeningeal vasculature, a mechanism which has been proposed for neurological complications of SWS [3, 9]. Acute hemiparesis due to chronic venous sinus occlusion is reported in SWS, albeit without DWI changes suggestive of ischaemia [5]. A recent report described increased cortical DWI signal in a patient with SWS with a subacute history of headache and hemiparesis [7], although the DWI changes only occurred after a series of prolonged seizures. Increases in cortical DWI signal have been reported in patients with SWS and chronic neurological deficit, and may reflect gliotic changes in this context [1]. Aspirin is thought to reduce the risk of stroke-like episodes due to microcirculatory stasis [8], and its use in adults with SWS is described anecdotally [7] although there are no large clinical trials to support its use. Given the imaging changes suggestive of C. Kobylecki (&) M. Jones A. Gerhard Department of Neurology, Greater Manchester Neurosciences Centre, Stott Lane, Salford M6 8HD, UK e-mail: christopher.kobylecki@manchester.ac.uk