Primary Angiitis of the Central Nervous System

Case report


Mahfoud El Bashari, Mahmoud Askar, Hisham Hamdallah, Amani Al Zaabi, Abdallah Al Rumathi, Abdulmatlub Ben-Musa

Department of Neuroscience, Zayed Military Hospital, Abu Dhabi, UAE

JMJ 2009,Vol.9, No.1: 69-72


Primary angiitis of the central nervous system is a rare clinicopathological entity. It is a type of vasculitis of the central nervous system without other apparent systemic vasculitis. The neurological manifestations of primary angiitis are protean.
We report a 48-year-old patient, a known case of type 2 diabetes and asthma, presenting with painful asymmetrical paraparesis. Diagnosis of primary angiitis of central nervous system was made on the basis of an abnormal MRI, positive oligoclonal bands in the cerebrospinal fluid and histology. After an initial response to prednisolone alone the patient relapsed, however when combining cyclophosphamide and prednisolone, there was a good sustained clinical and radiological response, and clearance of cerebrospinal fluid with negative oligoclonal bands. The peculiarities of this case include the rare presentation of primary angiitis as a polyradiculomyelopathy, the oligoclonal band marker in the cerebrospinal fluid becoming negative during remission, and the need for combination therapy of cyclophosphamide and prednisolone for sustained remission.

Keywords: Primary angiitis of the central nervous system, Oligoclonal bands, Polyradiculomyelopathy, Combination therapy, Prednisolone, Cyclophosphamide.