2026-07-06 | [Successful treatment with rituximab in unilateral relapsing primary CNS vasculitis: a case report].
We report a case of unilateral relapsing primary CNS vasculitis (PCNSV) treated with Rituximab. A 52-year old man initially diagnosed as PCNSV by brain biopsy. He was treated with corticosteroids and no recurrence had been recognized for 4 years. However, after the reduction of corticosteroids to 9 mg, PCNSV recurred many times only in the right hemisphere. Mycophenolate mofetil and cyclophosphamide were not effective. We diagnosed this patient as unilateral relapsing PCNSV (UR-PCNSV), which is resistant to treatment and repeatedly relapses unilaterally. After rituximab was introduced, there was no clinical recurrence and abnormal lesions on head MRI improved. We emphasize that rituximab is a good candidate drug for treating the UR-PCNSV.
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2026-06-09 | MULTIPLE RECURRENT ISCHEMIC STROKES AS THE INITIAL MANIFESTATION OF PROBABLE PRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM: A CARE-COMPLIANT CASE REPORT
report a 57-year-old previously healthy man presenting with multiple recurrent ischemic strokes over a short period despite adequate antiplatelet therapy. Conventional imaging was inconclusive. Advanced neuroimaging with vessel wall imaging demonstrated concentric arterial wall enhancement suggestive of vasculitis. Cerebrospinal fluid and extensive laboratory tests were unremarkable. Brain biopsy revealed inflammatory changes without definitive granulomatous vasculitis. The diagnosis of probable PACNS was established based on clinicoradiological correlation and exclusion of secondary causes. Intervention and outcome: The patient was treated with high-dose corticosteroids followed by cyclophosphamide, evolving with clinical improvement, radiological stabilization, and functional recovery. Conclusion: This case highlights the importance of considering PACNS in patients with recurrent unexplained strokes and underscores the role of advanced imaging and multidisciplinary evaluation.
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2026-05-13 | Inside the Heterogeneity of Primary CNS Vasculitis: A Single-Center 40-Year Experience.
Primary CNS vasculitis (PCNSV) is a heterogeneous condition. This study examines a large cohort with long-term follow-up to identify potential disease subsets. We retrospectively analyzed 216 patients with PCNSV (Mayo Clinic, 1983-2023), using standardized diagnostic criteria, classifying by vessel size, histopathology, and outcomes. Subsets and predictors of functional and therapeutic outcomes were evaluated. Diagnosis was based on cerebral angiography in 142 patients and histologically confirmed in 74. Isolated small vessel involvement was positively associated with mass-lesion presentation (odds ratio [OR] 19.38, p = 0.02), meningeal-enhancing lesions (OR 39.10, p < 0.0001), elevated CSF protein (OR 4.04, p = 0.03), and β-amyloid vascular deposits (OR 23.43, p = 0.0001), but negatively with focal manifestations (OR 0.32, p = 0.04) and cerebral infarcts (OR 0.22, p = 0.003). Lymphocytic vasculitis was linked to younger age at diagnosis (p = 0.006), longer symptom-to-diagnosis interval (p = 0.05), more seizures (p = 0.04), and lower disability (p = 0.003) and mortality (p = 0.008). Necrotizing vasculitis was associated with intracranial hemorrhage (p = 0.008). Two or more relapses occurred in 12.7%, associated with histologic diagnosis (OR 3.15, p = 0.009) and inversely with gadolinium-enhanced lesions (OR 0.33, p = 0.01). Therapy response occurred in 82.9%, long-term remission in 23.6%. Cerebral infarcts, especially multiple, were associated with poor therapy response (OR 0.11, p = 0.03). Histologic diagnosis was inversely associated with long-term remission (OR 0.44, p = 0.03), whereas aspirin use was positively associated (OR 2.8, p = 0.002). A rapidly progressive course occurred in 13.4% of patients and was linked to increasing age (OR 1.34/10 years, p = 0.04), cognitive dysfunction (OR 5.59, p = 0.02), cerebral infarctions (OR 5.02, p = 0.004), and large vessel involvement (OR 3.51, p = 0.02). Gadolinium-enhanced lesions (OR 0.36, p = 0.04) and aspirin (OR 0.42, p = 0.08) were protective. Mortality (21.3%) was associated with older age (HR 1.42, p = 0.002), cognitive dysfunction (HR 3.93, p = 0.006), and cerebral infarctions (HR 1.94, p = 0.03). PCNSV heterogeneity, driven by vessel size and histology, affects presentation and outcomes; our findings offer insights to improve diagnosis and treatment.
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