Case report

A previously healthy 45-year-old man presented to the ENT clinic in 2008. He gave a history of subacute progressive bilateral sensorineural deafness, intermittent headaches and a brief episode of right-sided weakness. The ENT examination was normal apart from deafness.

Three weeks later, he was admitted with sudden-onset of left hemiplegia. He was mentally slow with bilateral sensorineural deafness, an incomplete left homonymous hemianopia and hemiparesis with an extensor left plantar response. Fundoscopy was normal, and his coordination and sensation were relatively spared. There were sparse erythematous macules.

MRI brain showed several areas of T2 high signal in the right occipital lobe and pons (figure 1), consistent with recent infarction. Several small areas in the left cerebrum were consistent with mature infarctions. There was no contrast enhancement.

Carotid Doppler study showed a 2 cm plaque causing 50% obstruction within the left internal carotid artery (ICA), just beyond its origin. Transthoracic echocardiogram with bubble-contrast showed a structurally normal heart without trans-septal shunt.

Routine bloods, including inflammatory markers, anti-neutrophil cytoplasmic antibodies and anti-nuclear antibodies …