A 73-year-old woman developed cognitive decline over 1 year. MR scan of the brain showed a focal asymmetrical leukoencephalopathy involving the right frontal, temporal, parietal and occipital lobes. Extensive laboratory investigations found no cause but brain biopsy identified amyloid-beta-related angiitis (ABRA), a potentially treatable cause of rapid-onset dementia. We gave intravenous methylprednisolone and then two courses of intravenous cyclophosphamide, after which her cognitive skills gradually but significantly improved over several months.

A previously well and independent 84-year-old man presented with subacute behavioural disturbance and cognitive decline, progressing over one month to a bedbound encephalopathic state with myoclonus. Examination at that time revealed global cognitive dysfunction, dysarthria, dysphasia, myoclonus and motor pyramidal signs. MRI study revealed widespread high signal on T2-weighted imaging in the hemi- spheric deep white matter, as well as in the right caudate nucleus. EEG revealed frequent periodic triphasic complexes. CSF examination was positive for 14–3–3 and RTQuIC, with S100b 1.2mg/ml, confirm- ing a diagnosis of sporadic Creutzfeldt-Jacob Disease (CJD).During his admission, we noted the emergence of a vasculitic skin rash over his torso spreading bilater- ally over three days. Punch biopsy confirmed the presence of an acute leucocytoclastic vasculitis not suggestive of a drug-associated rash. Further immunological staining did not detect any abnormal prion protein. The rash persisted until he sadly died 4 weeks after admission.In the literature, there are only 3 reported cases of cutaneous manifestations of CJD. The emergence of an acute vasculitic rash in the context of confirmed CJD in our case may suggest immune dysregulation associated with the latter’s underlying pathophysiology, a direct manifestation of prion protein (despite negative staining) or an unrelated concurrent presentation.katieyoganathan@gmail.com