Papulonecrotic tuberculid (PNT) is a rare hypersensitivity reaction to Mycobacterium tuberculosis, typically seen in immunocompetent individuals but occasionally reported in human immunodeficiency virus (HIV)-positive patients. We describe the case of a 30-year-old HIV-positive male with high-risk sexual behavior presenting with crusted papulopustular lesions and severe headache. Cerebrospinal fluid and venereal disease research laboratory (VDRL) were positive for VDRL and treponema pallidum hemagglutination confirming neurosyphilis, while skin biopsy revealed PNT. This case underscores the diagnostic challenge of differentiating co-infections and id reactions in HIV. The patient improved significantly with anti-tubercular therapy and benzylpenicillin.

Granulomatosis with polyangiitis (GPA) is a rare multisystem necrotizing vasculitis involving small to medium-sized vessels and is commonly associated with proteinase 3 antineutrophil cytoplasmic antibodies (PR3 ANCA). While pulmonary and renal manifestations are classically recognized, atypical presentations with predominant constitutional, sinonasal, ophthalmological, and endocrine manifestations may result in substantial diagnostic delay. We present the case of a 28-year-old woman with a two-year history of profound fatigue, generalized myalgia, anosmia, chronic sinonasal symptoms, headaches, painful eye movements, and secondary amenorrhea. Her symptoms progressed to significant impairment in activities of daily living because of severe fatigue and lower limb pain. The patient underwent extensive hematological and endocrine investigations, including lymph node biopsy and pituitary hormonal assessment, before the eventual diagnosis of PR3 ANCA-positive GPA. Computed tomography imaging demonstrated complete opacification of the right maxillary sinus with additional ethmoidal mucosal thickening, while autoimmune serology demonstrated strongly positive anti-PR3 antibodies with negative anti-myeloperoxidase (MPO) antibodies. The patient subsequently demonstrated marked symptomatic improvement following the initiation of oral prednisolone therapy. This case highlights the diagnostic challenges associated with ear, nose, and throat (ENT) predominant GPA in the absence of overt renal disease and emphasizes the importance of considering ANCA-associated vasculitis in patients with chronic inflammatory symptoms and refractory sinonasal disease.

Intravenous immunoglobulin (IVIG) is an established therapy for immune-mediated neurological disorders and is generally well-tolerated, although rare neurological adverse effects have been reported. We describe a woman in her 40s treated with IVIG for suspected acute inflammatory demyelinating polyneuropathy following Campylobacter infection. Within 48 hours of therapy, she developed a headache and fever without meningism. Neuroimaging was unremarkable. Cerebrospinal fluid (CSF) analysis demonstrated marked pleocytosis with neutrophilic predominance, initially suggestive of bacterial meningitis. However, inflammatory markers and microbiological investigations were negative. Symptoms resolved rapidly following the discontinuation of IVIG without antimicrobial therapy. A diagnosis of IVIG-induced aseptic meningitis was made. This case highlights that IVIG can produce marked CSF pleocytosis mimicking bacterial meningitis and underscores the importance of clinical correlation to avoid unnecessary antimicrobial treatment.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multiorgan damage mediated by multiple autoantibodies. The nervous system is one of the target organs of SLE, presenting as neuropsychiatric SLE (NPSLE), which includes central nervous system (CNS) involvement, peripheral neuropathy, and psychiatric symptoms. These symptoms are not necessarily associated with systemic disease activity and are often challenging to diagnose. We report an 11-year-old girl presenting with persistent fever and mild headache. Based on clinical and laboratory findings, she was diagnosed with SLE complicated by antiphospholipid antibody syndrome. Brain magnetic resonance imaging revealed multiple small lesions in the bilateral deep white matter of the frontal lobes, with high signal intensity on diffusion-weighted imaging and low signal intensity on apparent diffusion coefficient maps, consistent with multiple cerebral infarcts. Although multiple cerebral infarcts were present in this case, no localized clinical symptoms corresponding to the infarct locations were observed, and the case did not meet the definition of NPSLE. Conversely, electroencephalography (EEG) revealed diffuse high-amplitude slow waves in the occipital region, and short-latency somatosensory evoked potentials (SSEPs) showed absence of the N20 component at C3' following right median nerve stimulation. These neurophysiological findings supported a comprehensive diagnosis of CNS NPSLE. These abnormalities gradually improved during immunosuppressive treatment. NPSLE represents one of the most challenging organ-specific manifestations to diagnose in pediatric SLE due to its complex and heterogeneous clinical presentation and the absence of standardized diagnostic screening methods. Unlike lupus nephritis, complement consumption is not necessarily involved in the pathogenesis of NPSLE. Therefore, NPSLE may remain undiagnosed unless clinically suspected. In this case, abnormal EEG findings and absence of the N20 component on SSEP suggested ischemic changes due to cerebrovascular disease, enabling a comprehensive diagnosis of CNS NPSLE. Neurophysiological testing proved useful for early diagnosis of NPSLE and evaluation of treatment efficacy.

Gaps in preparedness for independent practice are common among recent graduates. Clinical experiences serve as the foundation for learning during residency, yet the breadth and variability of clinical exposure of child neurology residents remain poorly characterized. To inform programmatic curricular development and precision medical education (PME), we must better characterize learners' clinical exposure during training. We aimed to use electronic health record (EHR) data to catalog resident clinical experiences, linking diagnostic codes to educational benchmarks.

Central nervous system (CNS) tumors are the deadliest cancers in children, highlighting the need for new therapies. The tumor-associated antigens (TAAs) WT1, PRAME and survivin are widely expressed by these tumors, and a manufacturing technique has been developed to target these intracellular TAAs using autologous, nongenetically engineered T cells. Here we therefore conducted ReMIND, an open-label, phase 1 adaptive dose-finding study to determine the safety/feasibility of autologous, systemically administered trivalent T cells targeting WT1, PRAME and survivin in children with CNS tumors. Eligible patients had newly diagnosed diffuse intrinsic pontine glioma without lymphodepletion (arm A, n = 16 enrolled, n = 11 infused) and relapsed/recurrent nonbrainstem CNS malignancies without (arm B, n = 28 enrolled, n = 18 infused) or with (arm C, n = 7 enrolled, n = 4 infused) lymphodepletion. Primary end points were safety, feasibility and maximum tolerated dose determination; secondary end points included preliminary efficacy and immunobiological correlates, including in vivo TAA-T persistence and systemic immune activation. Dose level 3 (8 × 107 cells per m2 per dose) was determined as the maximum tolerated dose. Treatment was well tolerated with fatigue and headache being the most common adverse events, although two possibly related serious adverse events of tumor swelling occurred. One grade 5 event in a patient with diffuse intrinsic pontine glioma with hydrocephalus, tumor edema and respiratory failure was categorized as a dose-limiting toxicity. Median overall survival for arm A was 13.7 months from diagnosis (range, 6.2-32.0) and median progression-free survival for arms B/C was 5.0 months from infusion (range, 0.5-51.6). Three patients in arms B/C are alive without disease at 31.8, 41.2 and 51.6 months without further treatment, including one complete response. This trial met safety/feasibility primary end points with some preliminary signals of efficacy. ClinicalTrials.gov registration: NCT03652545 .