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.

Mental Health Disorders and Psychosocial Distress in Pediatric Celiac Disease.

OBJECTIVES: Celiac disease (CeD) has been associated with increased mental health disorders (MHD) and psychosocial distress in children, which may complicate treatment with the gluten-free diet (GFD). This single-center cross-sectional study examined psychological comorbidities in children with CeD to assess psychological needs in CeD care. METHODS: Participants were 73 parents (95% mothers) of children (ages 3-18) attending a multidisciplinary celiac disease clinic. Parents completed electronic surveys about their child's MHD history, psychological symptoms, and GFD experiences. Rates of MHD were calculated and compared with National Institute of Mental Health population-level data. Differences in psychosocial symptoms and GFD experiences were examined based on child age, time since CeD diagnosis, and MHD. RESULTS: Thirty-four percentage of children had at least 1 MHD; anxiety disorders (16%, P < 0.001) and attention-deficit/hyperactivity disorder (ADHD; 16%, P = 0.01) were more common than general population rates. More than 1 quarter of parents reported current child psychosocial distress (28%-39%), and approximately half reported parent stress (51%) and financial burden (46%) associated with the GFD. Parents of children with new CeD diagnoses reported lower confidence in the GFD (P < 0.01) but MHD, stress, and financial burden did not differ by time since CeD diagnosis. Children with MHD had more anxiety, anger, overall distress, and parent distress than those without MHD (Ps < .05). CONCLUSIONS: Comorbid CeD and MHD was common and was associated with increased child and parent psychosocial distress. Our findings emphasize the importance of psychological screening and services to assess for and treat comorbid MHD and to mitigate psychosocial distress associated with the GFD.

Fueling the FIRES: Hemophagocytic lymphohistiocytosis in febrile infection-related epilepsy syndrome.

OBJECTIVES: Although secondary hemophagocytic lymphohistiocytosis (HLH) has been reported in children with critical illness of various etiologies, it has not been reported in patients with febrile infection-related epilepsy syndrome (FIRES). We describe a series of patients with concurrent HLH and FIRES in an effort to establish common pathophysiologic abnormalities. METHODS: Five patients with FIRES who were assessed for HLH were identified from a neurocritical care database. All were previously healthy and had extensive diagnostic testing. All had clinical deterioration with multiorgan dysfunction prompting HLH screening 20-29 days after hospitalization. Markers for inflammatory dysregulation were assessed in cerebrospinal fluid (CSF) and serum at various time points. Outcomes were assessed 6 months after presentation. RESULTS: Three patients met clinical criteria for secondary HLH. Elevation of specific cytokines/chemokines was variable. CSF neopterin, high mobility group box 1 (HMGB1), and C-X-C motif chemokine ligand 8 (CXCL8) were significantly elevated in all. Interleukin-1ß (IL-1ß) and IL-18 were not elevated in any of the samples. Treatment and outcomes were variable. SIGNIFICANCE: We describe 3 patients with HLH and FIRES. The co-occurrence of these 2 rare disorders suggests the possibility of a common immune dysregulation phenotype prolonging epileptogenesis. HLH screening in critically ill patients with FIRES may yield a broader understanding of shared inflammatory processes.