Anti-NMDAR Encephalitis Clinical Practice Guideline: Improving Time to Diagnosis, Treatment, and Hospital Length of Stay.

Background and Objectives: A clinical practice guideline (CPG) was created to standardize evaluation and treatment for patients with suspected anti-methyl-d-aspartate receptor (NMDAR) autoimmune encephalitis (AE), the most common AE in children. The objective of this study was to evaluate the CPG effect on time to diagnosis, treatment, and hospital length of stay (LOS). Methods: Patients with an inpatient consult to pediatric rheumatology for AE during a 4-year period (period 2) after CPG implementation were identified. Data were extracted and compared with data over the preceding 4-year period (period 1). Results: During period 1, fewer patients underwent diagnostic testing than during period 2 (34 vs 80). Number of patients diagnosed with AE did not differ from period 1 to that from period 2 (NMDAR AE 9 vs 8; seronegative AE 4 vs 5). The average time to diagnostic evaluation with lumbar puncture decreased from 5.4 to 1.5 days (p = 0.0082), and time to treatment decreased from 7.6 to 3.9 days (p = 0.018). LOS showed a trend toward improvement (40.4-29.2 days (p = 0.23)). Discussion: Creation of a CPG for patients with suspected AE was associated with an improved time to diagnostic evaluation and treatment. With the CPG, more patients underwent AE testing, though total diagnoses remained the same.

Epilepsy with myoclonic absences: a case series highlighting clinical heterogeneity and surgical management.

OBJECTIVE: Epilepsy with myoclonic absences is a rare epilepsy syndrome with distinct features and high rates of drug resistance. Identifying this syndrome may help guide treatment decisions. We highlight clinical heterogeneity in this case series and two cases in which corpus callosotomy was performed. METHODS: Medical records were reviewed between 2017 and 2021 to identify demographics, comorbidities, age at onset, EEG findings, diagnostic evaluations, seizure semiologies, seizure frequency, anti-seizure medications, diet therapy and surgical treatments in patients with myoclonic absences. RESULTS: Ten patients were identified including twins with myoclonic absence status epilepticus. Forty percent had an atonic component, 20% presented with myoclonic absence status epilepticus and 60% had incomplete control of seizures at last follow-up visit. Two patients with epilepsy with myoclonic absences with atonia underwent corpus callosotomy; one patient was seizurefree eight months after surgery and the other had greater than 50% seizure reduction over a five-month period. SIGNIFICANCE: Phenotypic heterogeneity was evident based on seizure semiologies, comorbidities, seizure frequency and response to anti-seizure medications and non-medication treatments. Of patients with an atonic component, 75% did not achieve seizure freedom with medication alone. Corpus callosotomy was performed in two of these patients with encouraging seizure response thus far, however, the efficacy of this treatment should be further evaluated in a larger study.

Multimodal Neurologic Monitoring in Children With Acute Brain Injury.

Children with acute neurologic illness are at high risk of mortality and long-term neurologic disability. Severe traumatic brain injury, cardiac arrest, stroke, and central nervous system infection are often complicated by cerebral hypoxia, hypoperfusion, and edema, leading to secondary neurologic injury and worse outcome. Owing to the paucity of targeted neuroprotective therapies for these conditions, management emphasizes close physiologic monitoring and supportive care. In this review, we will discuss advanced neurologic monitoring strategies in pediatric acute neurologic illness, emphasizing the physiologic concepts underlying each tool. We will also highlight recent innovations including novel monitoring modalities, and the application of neurologic monitoring in critically ill patients at risk of developing neurologic sequelae.

Rapid ictal transition of focal epilepsy to infantile spasms in neurofibromatosis type 1 captured with EEG.

We report a novel case of an infant with neurofibromatosis type 1 (NF1) who presented with new onset presumed focal impaired awareness seizures with motor onset followed by rapid progression to infantile spasms (IS). Electroencephalography (EEG) captured evolution from focal epileptiform discharges to multifocal and generalized discharges, then to hypsarrhythmia over three days. Development of IS within days of focal seizure onset is rapid, and to our knowledge, has not been demonstrated electrographically. The pattern of rapid ictal transition to hypsarrhythmia is essential for neurologists to be able to recognize as it can help lead to early treatment, which is necessary for improved outcomes in IS.

Cerebral regional oxygen saturation trends in infants with hypoxic-ischemic encephalopathy.

BACKGROUND: Neurological outcomes in neonatal hypoxic-ischemic encephalopathy (HIE) continue to be sub-optimal despite therapeutic hypothermia (TH). Cerebral near-infrared spectroscopy provides real-time regional oxygen saturation (CrSO2) that may be a marker of adverse MRI findings and neurodevelopmental outcomes. AIM: The aim of this study was to examine the value of CrSO2 monitoring in infants with HIE undergoing TH. STUDY DESIGN AND SUBJECTS: In this prospective study, CrSO2 was continuously recorded in 21 infants with HIE admitted for TH. OUTCOME MEASURES: Brain MRI signal abnormalities at 2weeks were scored in individual brain region and classified as none/mild, moderate and severe. 13 infants completed Bayley Scales of Infant Development (BSID) testing at 18-24months. RESULTS: Between 24 and 36h of life, there was a significant increase in odds of having moderate-severe brain MRI abnormalities with higher absolute CrSO2 values. Per 10% increase in absolute CrSO2, the odds ratio for moderate-severe brain MRI abnormalities was greatest at 30h (OR 3.78; confidence intervals (CI): 1.23-11.6, p=0.011). CrSO2 increased more rapidly in infants with greater injury seen on MRI (0.20/h for MRI scores 0/1, by 0.48/h for MRI score 2, and by 0.68/h for MRI score 3, p=0.05). At 30h, absolute CrSO2 correlated significantly with abnormal MRI findings in basal ganglia (92% vs. 78%, p=0.001), white matter (88% vs. 76%, p=0.01), posterior limb of internal capsule (92% vs. 78%, p=0.001), and brain stem (94% vs. 80%, p=0.03) but not with cortical injury (86% vs. 80%, p=0.17). Higher CrSO2 beyond 24h correlated with greater odds of worse BSID scores. CONCLUSIONS: Increasing CrSO2 is associated with moderate-severe brain injury as assessed by MRI. Higher absolute CrSO2 values during TH correlates with subcortical injury on MRI and poor neurodevelopmental outcomes in infants with HIE undergoing TH. CrSO2 can inform providers seeking early identification of patients at risk of worse injury who may benefit from further intervention.