AUTHOR=Sheikh Tahani Saker , Rozenberg Ayal , Merhav Goni , Shifrin Alla , Stein Polina , Shelly Shahar 

TITLE=Primary CNS vasculitis: insights into clinical, neuropathological, and neuroradiological characteristics

JOURNAL=Frontiers in Neurology

VOLUME=15

YEAR=2024

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1363985

DOI=10.3389/fneur.2024.1363985

ISSN=1664-2295

ABSTRACT=<sec id="sec1"><title>Background and objectives</title><p>Primary CNS vasculitis (PCNSV) is a rare inflammatory disorder that affects the blood vessels of the central nervous system (CNS). We aimed to analyze the neurological presentations, clinical follow-up, and long-term outcomes of patients with primary central nervous system vasculitis.</p></sec><sec id="sec2"><title>Methods</title><p>We conducted a retrospective analysis of medical records to assess the neurological presentation, rate of remission, and functional status at the last follow-up in patients with primary central nervous system vasculitis seen in our center in the last 13 years (2010–2023).</p></sec><sec id="sec3"><title>Results</title><p>We identified five patients, whose median age at symptom onset was 31 years (range: 15–41 years), including four male individuals (80%) from Muslim Arab (<italic>n</italic> = 4) and Ashkenazi Jewish (<italic>n</italic> = 1) backgrounds. Symptoms persisted for a median of 36 weeks (range: 3 weeks to 4 years) before diagnosis, with one case exceeding 3 years. Follow-up lasted a median of 56 months (range: 20–161 months). Clinical symptoms varied, presenting unilateral weaknesses (<italic>n</italic> = 2), cognitive and gait abnormality (<italic>n</italic> = 1), headaches (<italic>n</italic> = 1), and epileptic seizures (<italic>n</italic> = 1). MRI scans revealed abnormalities in the basal ganglia, corona radiata, parietal, and frontal lobes, showing hemorrhage, vasogenic edema, restricted diffusion, and enhancement post-gadolinium. All patients reported progressive holocephalic headaches and cognitive changes with overall progressive symptoms. Initial neurological examinations revealed abnormalities in all patients and included one or more of the following: cognitive or visual impairment (<italic>n</italic> = 2), seizures (<italic>n</italic> = 1), and unilateral UMN signs (<italic>n</italic> = 2) at the initial neurological examination, all but one patient required walking aids including (cane 2, wheelchair, bedridden 1). Patients were stable (<italic>n</italic> = 2), deteriorated (<italic>n</italic> = 1), or improved (<italic>n</italic> = 2). Following treatment, two patients still required ambulatory aids, with one using a cane and the other using a wheelchair, while the remaining three did not require any ambulatory aids.</p></sec><sec id="sec4"><title>Discussion</title><p>The study on PCNSV highlights varied symptoms and diagnostic challenges, including delayed diagnosis and a spectrum of neurological issues from cognitive impairments to seizures. Brain biopsies showed lymphocytic infiltration, thrombi, and necrosis. Immunotherapy significantly improved clinical and radiological outcomes. Over 56 months of follow-up, outcomes varied from stability and deterioration to improvement.</p></sec>