Primary Angiitis of the CNS: The Elvis Presley of Neurology

Dr. David Hellmann of Johns Hopkins, in the current Clinical Neurology News, is quoted as saying that primary angiitis of the central nervous system (PACNS) “is the Elvis Presley of neurology… CNS vasculitis-like illness is more often thought to be present than actually present”. In fact, he says, CNS vasculitis accounts for only 1% of all biopsy-proven cases of vasculitis. Interestingly, very few patients with PACNS describe a stroke-like presentation. Rather, the most common presentation is insidious cognitive decline with headache. A biopsy is required for diagnosis, and cyclophosphamide without a definitive biopsy-proven diagnosis is not recommended. Yet again, the neuropathologist is the key!