An immunocompromised patient with non-specific neurological symptoms
and signs, along with rapid cognitive decline evolving over three to four weeks, can
present a diagnostic challenge. Here we report rapidly progressive dementia in a
patient with systemic lupus erythematosus, who was subsequently diagnosed with
sporadic Creutzfeldt-Jakob disease (sCJD). This case illustrates the need for prompt
investigations to consider an alternative diagnosis where significant history fails to
yield an explanation. A diagnosis of sCJD drastically alters the prognosis in a subject
with a well-controlled connective tissue disease who is otherwise fit and well.
