Convusions and homonymous hemianopsia as initial manifestations of Sturge-Weber syndrome in a 64-year-old male.

Sturge-Weber syndrome (SWS), or encephalotrigeminal angiomatosis, is the most frequent neurocutaneous syndrome, characterised by vascular malformations which manifest sporadically. The full presentation includes brain vascular malformations (pial or leptomeningeal angioma), cutaneous abnormalities (facial angioma) and ocular abnormalities (choroidal angioma). We present the case of a patient with very-late-onset SWS who experienced acute confusional syndrome, convulsions, and homonymous hemianopsia. Our patient is a 64-year-old man, a former smoker, who drinks 80 g ethanol/day and has a personal history of diabetes. In the 48 hours prior to his admission, he presented confusional symptoms and altered conduct/behaviour. He arrived at the emergency department due to a generalised tonic-clonic seizure. Computed tomography in the emergency department did not show acute intracranial disease and lumbar puncture obtained acellular cerebrospinal fluid with a protein level of 73.3 mg/dL. He was conscious, disoriented, and anxious at admission, presenting sensory aphasia and coprolalia. Doctors began treatment with intravenous levetiracetam. After 48 hours, confusional symptoms resolved and the patient was oriented during the neurological examination, which revealed with bilateral horizontal nystagmus, right homonymous hemianopsia, right upper limb apraxia, and unsteady gait with tendency to drift to the right. The patient presented a small cutaneous angioma on the left frontal region (territory of the ophthalmic branch of the fifth cranial nerve) (Fig. 1). We consulted the ophthalmology service, which ruled out the presence of