Cerebrospinal fluid eosinophils in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease.

BACKGROUND: Eosinophils in cerebrospinal fluid (CSF) are an uncommon finding most often associated with parasitic infections, but have also been described in some neuroinflammatory disorders. Eosinophilic infiltration is not thought to be a typical feature of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). We aim to describe the rate of CSF eosinophil positivity in a cohort of pediatric MOGAD patients. METHODS: Single-center retrospective chart review of pediatric MOGAD patients. Clinical and laboratory data was collected from the electronic medical record and analyzed. RESULTS: Of 46 pediatric patients with positive serum myelin oligodendrocyte glycoprotein antibody (MOG-IgG) identified, 38 patients fulfilling internationally proposed MOGAD diagnostic criteria were included for analysis. 6 patients with MOGAD were excluded as no CSF data was available, and 2 patients with positive MOG-IgG but diagnosis more consistent with MS were excluded. Median age was 7.3 years, and 19/38 (50 %) were female. Acute disseminated encephalomyelitis (ADEM) was the most common presenting phenotype (23/38, 61 %), and other phenotypes included optic neuritis (10/38, 26 %), transverse myelitis (3/38, 8 %), and neuromyelitis optica spectrum disorder (NMOSD) (2/38, 5 %). 12 of 36 (33 %) patients with all lumbar puncture (LP) data available had CSF eosinophils present, with eosinophil mean of 3 % and range from 1 % to 18 % of CSF while blood cells. CONCLUSION: CSF eosinophils were present in one third of pediatric MOGAD patients, which is a higher rate than previously reported in either MOGAD or aquaporin-4 antibody positive NMOSD cohorts. Understanding the CSF composition of pediatric MOGAD patients helps to facilitate more prompt diagnosis and treatment and may shed light onto underlying pathologic mechanisms of disease with the goal to inform future therapeutic targets.

Pediatric neuroinflammatory diseases in the intensive care unit.

Inflammatory disorders of the central nervous system (CNS) include a wide spectrum of autoimmune, autoinflammatory, and paraneoplastic diseases. While many affected patients require acute hospital admission, a subset may present with severe neurological symptoms requiring intensive care unit (ICU) escalation due to disordered consciousness, respiratory failure, status epilepticus, intracranial hypertension, and/or severe autonomic dysregulation.

Case report: a co-occurring case of severe Mycoplasma pneumoniae pneumonia and Anti-IgLON5 antibody-associated encephalitis in a pediatric patient.

This case report details the clinical course of a 16-year-old female student with Mycoplasma pneumoniae infection complicated by autoimmune encephalitis, spanning from 6 February 2022, to 12 April 2022, with a one-year follow-up. The patient presented with a two-week history of cough and fever, followed by altered consciousness and neuropsychiatric symptoms, including hyperactivity and incoherent speech. Despite normal brain MRI findings, cerebrospinal fluid (CSF) analysis confirmed Mycoplasma pneumoniae with titers of, and positive IgLON5 antibodies. Initial treatment included azithromycin, ceftriaxone, and acyclovir, followed by mechanical ventilation and ECMO due to respiratory failure. The antibiotic regimen was switched to intravenous omadacycline based on genetic testing results. Autoimmune encephalitis was managed with intravenous methylprednisolone, intravenous immunoglobulin (IVIG), and plasma exchange. The patient's condition improved, and she was discharged on 12 March 2022, with normal cognitive and behavioral functions. However, she was readmitted one month later due to cognitive decline and sleep disturbances, with a Mini-Mental State Examination (MMSE) score of 20/30 and a modified Rankin Scale (mRS) score of 3. At the one-year follow-up, her MMSE score had improved to 28/30, and her mRS score was 1. This case underscores the importance of comprehensive diagnostic approaches and personalized treatment strategies in managing complex cases of mycoplasma-related infections and associated autoimmune conditions.

Inflammatory Diseases of the Central Nervous System.

Pediatric neuroinflammatory conditions are a complex group of disorders with a wide range of clinical presentations. Patients can present with a combination of focal neurologic deficits, encephalopathy, seizures, movement disorders, or psychiatric manifestations. There are several ways that pediatric neuroinflammatory conditions can be classified, including clinical presentation, pathophysiologic mechanism, and imaging and laboratory findings. In this article, we group these conditions into acquired demyelinating diseases, immune-mediated epilepsies/encephalopathies, primary rheumatologic conditions with central nervous system (CNS) manifestations, CNS vasculitis, and neurodegenerative/genetic conditions with immune-mediated pathophysiology and discuss epidemiology, pathophysiology, clinical presentation, treatment, and prognosis of each disorder.