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OBJECTIVES: To assess clinical and electroencephalogram (EEG) predictors of epilepsy and to describe the percentage of electrographic seizures and development of epilepsy among patients with spontaneous intracerebral hemorrhage (ICH) due to arteriovenous malformation (AVM) rupture. STUDY DESIGN: Retrospective review of patients admitted to the pediatric intensive care unit with ICH secondary to AVM rupture over 11 years. Clinical variables were collected by review of the electronic medical record. Seizures were described as acute symptomatic (7 days after AVM rupture), subacute (7-30 days after AVM rupture) and remote (greater than 30 days after AVM rupture). Outcome metrics included mortality, and the development of epilepsy post discharge. Descriptive statistics were used. RESULTS: Forty-three patients met inclusion criteria with a median age of 12.2 years (IQR 7.3-14.8) and 49% (21/43) were female. Sixteen percent (7/43) presented with a clinical seizure prior to EEG placement. EEG was performed in 62% (27/43) of patients; one had electrographic status epilepticus without clinical signs. Sixteen percent (7/43) of patients were diagnosed with epilepsy, with a median time to diagnosis of 1.34 years (IQR 0.55-2.07) after AVM rupture. One-year epilepsy-free survival was 84% (95% CI 70%-98%) and 2-year epilepsy-free survival was 79% (95% CI 63%-95%) Remote seizures were associated with epilepsy (P < .001), but acute symptomatic seizures were not (P = .16). CONCLUSIONS: EEG-confirmed seizures are uncommon in patients with ICH secondary to AVM rupture; however, when identified, the seizure burden appears to be high. Patients with seizures 30 days after AVM rupture are more likely to develop epilepsy.
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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.