To describe the demographic, clinical and radiological characteristics of patients of Primary central nervous system vasculitis .

We retrospectively collected the demographic, clinical, radiological data of patients who had radiological or and histopathological findings fulfilling criteria of PCNSV after screening the discharge record of patients from 2011 to 2017. After going through the detailed records of patients, 53 patients had findings suggestive of PCNSV.

The mean age of patients at first presentation was 27.97 ± 12.3 years (range 15-50 years).Mean duration of illness before diagnosis ranged from 23 days to 4 years.Of the total of 53 patients, 47(88.6%) were males.The common symptoms were seizures (80%), headaches (60.3%),cognitive decline(32.1%),stroke like episode(30.2%), aphasia(22.6%), behavioral changes(20.1%), myelopathy like presentation(18.8%), fever(11.3%), vision or diplopia(18.8%), bulbar symptoms (9.4%),extrapyramidal features(3.7%).On reviewing the imaging the most common finding was presence of discrete to confluent white matter lesions of brain (99%), followed by presence of micro and macro-hemorrhages in Susceptibility weighted imaging sequence of MRI brain(98.7%) and linear or nodular gadolinium enhancement of meninges(79.7%). Cerebrospinal fluid examination was done in 25 patients of whom 20(80%) patients had abnormality in the form of pleocytosis and increased protein.

PCNSV usually affects young males and presents with a wide array of clinical features. Specific neuroimaging findings along with biopsy from the site of lesion help in confirming the diagnosis.