Coccidioidal vasculitis is a serious complication of coccidioidal infection. Prior studies suggest that steroids may reduce the risk of secondary vasculitis in coccidioidal meningitis (CM), but data on optimal dosing are limited. This study compared the characteristics and outcomes of CM patients with vasculitis who received different steroid doses. The Stanford Research Data Repository was used to identify adult patients diagnosed with coccidioidal meningitis from 1992 to 2024. Patients were divided into those who received no steroids, low or medium dose steroids, and high dose steroids. Characteristics at admission were compared by steroid use category, and their association with clinical outcomes were assessed. A total of 65 patients with CM were identified, with mean (standard deviation) age 45 (17) and 35% female. A higher percentage of patients not treated with steroids had a better modified Rankin score (mRS) of 2 or lower on admission compared to patients who were treated with steroids (standardized mean difference [SMD] = 0.67). Compared to patients who received a low/medium dose, those who received a high dose were 32% (95% CI 0.12, 3.61; P = 0.65) less likely to experience death or vasculitis. There is a complex relationship between the use of steroids and outcomes among patients with CM. Among steroid recipients, those who received high dose steroids may achieve better long-term outcomes. A larger study is needed to validate these findings.

Central nervous system (CNS) vasculitis is a rare, diagnostically challenging disorder involving inflammation of cerebral arteries. Differentiating infectious from autoimmune etiologies is difficult due to nonspecific presentations and limited tools. We characterized CNS vasculitis cases at a tertiary care center to identify distinguishing features by etiology. Patients admitted to Columbia University Irving Medical Center between 2020-2024 with an ICD diagnosis of CNS vasculitis were reviewed. Cases were classified as infectious, autoimmune, or cryptogenic based on clinical, laboratory, radiographic, and pathologic data. Demographics, symptoms, cerebrospinal fluid (CSF) findings, neuroimaging, treatments, and outcomes were analyzed with nonparametric testing. Of 43 included cases, the mean age was 45.7 years; 51.2 % male; 44.2 % immunocompromised; median symptom duration 7 days. Etiologies were infectious (14 %), autoimmune (30 %), and cryptogenic (56 %), with definitive diagnosis in 23.3 %. Headache, cognitive dysfunction, and motor symptoms were common. CSF parameters did not significantly differ across groups (median protein 53 mg/dL; glucose 79 mg/dL; RBC 60/µL; WBC 3/µL). Vessel narrowing occurred in 81.4 %; involvement of >2 vessels was more frequent in infectious (50 %) and cryptogenic (41.7 %) vs autoimmune (0 %) cases (p = 0.016). Cryptogenic cases more often showed ischemia (75 %), hemorrhage (25 %), and FLAIR hyperintensities (75 %) (p < 0.010). Biopsy was performed in 27.9 %. Steroids were given in 97.4 % and disease-modifying therapies in 25.6 %. Despite extensive testing, definitive diagnosis of CNS vasculitis remains difficult. While clinical and CSF features lacked discriminatory value, vascular and imaging patterns differed by etiology, highlighting the need for improved biomarkers and imaging strategies.

Vasculitides are a heterogeneous group of immune-mediated inflammatory disorders that compromise the vascuar wall, leading to luminal narrowing and tissue ischemia. When inflammation selectively affects the vasa nervorum without systemic involvement, it results in nonsystemic vasculitic neuropathy (NSVN), an underrecognized condition. NSVN presents diagnostic challenges due to its variable clinical manifestations and reliance on nerve biopsy for definitive diagnosis. This study aimed to characterize the clinical, neurophysiological, and histopathological features of NSVN in a Brazilian cohort. We conducted a cross-sectional, ambispective cohort study combining retrospective chart review and prospective patient assessments. Inclusion required histopathological confirmation of isolated peripheral nerve vasculitis; cases with systemic or secondary vasculitis were excluded. Data collection included clinical evaluation, neurophysiology, and nerve biopsy. A total of 14 patients were included (9 female, 64%; mean age: 61). Most (n = 8, 57%) had subacute onset of painful sensory or sensorimotor deficits. Multiple mononeuropathies predominated (n = 11, 78%), but a subset exhibited chronic progression (n = 5, 35%) and axonal polyneuropathy (n = 3, 21%). Electrophysiological studies revealed a consistent axonal pattern. Biopsies confirmed possible vasculitis in six (43%), and probable vasculitis in six (42%), with only two (14%) fulfilling criteria for definite vasculitis. Serologies were nonspecific. Treatment involved corticosteroid pulse therapy, with immunosuppression in refractory cases. These findings highlight that NSVN often presents with painful sensorimotor symptoms and may clinically mimic progressive axonal polyneuropathies. Given its potential for significant morbidity if left untreated, early recognition and consideration of nerve biopsy remain critical. The diagnostic complexity and variability in presentation suggest that NSVN may be underrecognized. We hope this cohort contributes to a broader understanding of its clinical spectrum and informs future diagnostic strategies.

Vasculitic neuropathies are a group of disorders characterized by inflammation and destruction of vessel walls, resulting in nerve ischemic injury that leads to acute-to-subacute sensory and motor deficits. This can arise from systemic inflammation or be confined to the peripheral nervous system. Causes include primary systemic vasculitis, secondary vasculitis associated with connective tissue disorders, viral infections, paraneoplastic and drug-induced reactions, and nonsystemic vasculitic neuropathy. Early recognition and prompt treatment are essential to mitigate the morbidity and mortality associated with these disorders. This review provides an update on the classification, diagnosis, and treatment of vasculitic neuropathies.

Acute ischemic stroke is a rare condition in the pediatric population. This case highlights the importance of considering neuroborreliosis as a potential cause of stroke in children, emphasizing the role of early diagnosis and appropriate treatment in preventing long-term sequelae. We present the case of a 13-year-old girl who was admitted with left-sided central facial nerve paresis. She had a six-month history of recurrent tension headaches and unintentional weight loss. Brain MRI revealed an ischemic lesion in the right thalamus and internal capsule, with additional findings in the left thalamus and cerebellar hemispheres on follow-up imaging. The diagnostic workup revealed positive Borrelia burgdorferi antibodies in both the cerebrospinal fluid and serum, confirming neuroborreliosis. Causal treatment was initiated with a third-generation cephalosporin, resulting in significant clinical improvement. Pediatric acute ischemic stroke in the course of secondary vasculitis on an infectious background appears to be the leading cause of stroke in children, which underscores the need for a thorough diagnostic evaluation targeting treatable infectious etiologies in all pediatric stroke cases. Early identification and causal treatment of such conditions, particularly neuroborreliosis, significantly improve neurological outcomes and increase the likelihood of full recovery. Therefore, potential infectious causes should not only be actively investigated but also considered when initiating empirical treatment. There is a necessity of maintaining high clinical vigilance in symptomatic patients from endemic regions presenting solely with positive Borrelia IgG serology, as such a profile does not exclude the presence of active and potentially severe neuroborreliosis.