A Case of Isolated Central Nervous System Vasculitis in an Elderly Patient

Abstract

A 83 year old woman, with unremarkable family history, was admitted in 2013 to a Neurology Ward for lumbar pain and progressive motor impairment of right lower limb. BasedonMRIofthedorsal spinalcordatransversemyelitiswashypothesized. She was treated with prednisone and remained steady for 30 months. Two months after treatment cessation, a relapse occurred with reduction of lower limb strength and progressively impaired speech. A Cerebral MRI was performed, that showed multiple spot-like areas of leukoaraiosis. All laboratory blood tests, including C reactive protein, serum virology (HBV, HCV), retroviral tests, Lyme serology, ANA, ENA, ANCA, RA and antiphospholipid screening were normal. Cerebrospinal fluid showed normal protein, glucose and cell count, but oligoclonal bands were detected. Significant stenosis of medium-small cerebral vessels of both anterior and posterior circulation were detected with Magnetic resonance angiography (MRA), thus suggesting the diagnosis of primary angiitis of central nervous system (PACNS). We treated the patient with prednisone 50 mg/day with rapid clinical and subjective improvement. After one month we reduced prednisone to 25 mg/day. In a few days, the patient reported ageusia, fatigue, and depression. A new MRI showed a progression in number and size of white matter lesions (see figure). Prednisone was increased again to 50 mg/day and mycophenolate mofetil 2 g/day was added. Three months later the patient is stable.