I Kept Crying.

In this narrative medicine essay, a second-year pediatric resident bears the scars from the terror that descended the moment of her son's birth at 31 weeks' gestation, terror relived even after he has come home healthy.

Retrospective Comparison of Patients Evaluated for Pediatric Autoimmune Encephalitis with Typical and Atypical Premorbid Neuropsychiatric Development.

PURPOSE: Patients with neurodevelopmental disorders (NDD) (i.e. autism, developmental delay, early-onset psychiatric or seizure disorders) increasingly seek evaluation of new or exacerbated symptoms concerning for autoimmune encephalitis (AE). Clinical AE evaluation can be challenging in NDD patients with symptom overlap between anti-neuronal autoimmunity and baseline atypical neurodevelopment. This study sought to explore differences in AE features by neurodevelopmental status. METHODS: This retrospective chart review included 67 children with typical development (TD) or NDD evaluated for AE at the authors' institution. AE diagnosis included seronegative AE or seropositive AE with anti-NMDAR or anti-GAD antibodies. Reported AE clinical domains, symptom onset acuity, and treatment response were compared between three groups: (1) TD children with AE (TD-AE, N = 24); (2) NDD children with AE (NDD-AE, N = 21); and (3) NDD children with a non-AE diagnosis following appropriate workup (NDD-nonAE, N = 22). RESULTS: Children with AE had a greater number of reported clinical domains than non-AE children with NDD (p < 0.0001) regardless of baseline developmental status. There were no observed differences in reported domains between TD-AE and NDD-AE groups. Onset acuity differed across the three groups (p = 0.04). No treatment response differences were observed between groups. CONCLUSION: NDD children with AE had a comparable number of reported clinical domains relative to TD children and a similar treatment response. NDD patients with AE had a greater number of reported clinical domains than their NDD peers without an AE diagnosis. These findings suggest that AE is a multi-domain process in both TD and NDD children.

Goal-Concordance in Children with Complex Chronic Conditions.

OBJECTIVES: To characterize delivery of goal-concordant end-of-life (EOL) care among children with complex chronic conditions and to determine factors associated with goal-concordance. STUDY DESIGN: This was a retrospective review of goals of care discussions for 272 children with at least 1 complex chronic condition who died at a tertiary care hospital between January 1, 2014, and December 31, 2017. Goals of care and code status were assessed before and within the last 72 hours of life. Goals of care discussions were coded as full interventions; considering withdrawal of interventions (palliation); planned transition to palliation; or actively transitioning/transitioned to palliation. RESULTS: In total, 158 children had documented goals of care discussions before and within the last 72 hours of life, 18 had goals of care discussions only >72 hours before death, 54 only in the last 72 hours of life, and 42 had no documented goals of care. For children with goals of care, EOL care was goal-concordant for 82.2%, discordant in 7%, and unclear in 10.8%. Black children had a greater than 8-fold greater odds of discordant care compared with White children (OR 8.34, P = .007). Comparison of goals of care and code status before and within the last 72 hours of life revealed trends toward nonescalation of care. Specifically, rates of active palliation increased from 11.7% to 63.0%, and code status shifted from 32.6% do not resuscitate to 65.2% (P < .001). CONCLUSIONS: In this cohort, a majority of children had documented goals of care discussions and received goal-concordant EOL care. However, Black children had greater odds of receiving goal-discordant care. Goals of care and code status shifted toward palliation during the last 72 hours of life.

Pediatric GAD-65 Autoimmune Encephalitis: Assessing Clinical Characteristics and Response to Therapy With a Novel Assessment Scale.

BACKGROUND: Glutamic acid decarboxylase (GAD) encephalitis is a neuroinflammatory disease characterized by a broad range of symptoms including cognitive deficits, behavioral changes, and seizures. Children with this disorder have heterogeneous presentations, and little is known about symptom progression over time and response to immunotherapy. METHODS: This study reports 10 pediatric GAD encephalitis cases and symptoms found at presentation and follow-up. In addition, symptom severity was reported utilizing a novel scale evaluating functional outcomes across the domains affected by autoimmune encephalitis including cognition, language, seizures, psychiatric symptoms, sleep, and movement. Retrospective chart review was conducted for 10 patients aged <18 years, diagnosed with GAD encephalitis, and followed for one year or more. Chart review included clinical, imaging, and laboratory findings at time of diagnosis and at six- and 12-month follow-ups. RESULTS: At presentation, cognitive deficits were found in all patients, seizures in six of 10, and language decline in seven of 10. Psychiatric symptoms were prominent for all but one patient with three of nine patients presenting with psychosis. Fatigue, sleep disruption, and movement disorders were less prominent symptoms, occurring in approximately half of the cohort. Cognition and fatigue improved significantly over time when compared with symptom severity, whereas seizures, psychiatric symptoms, and sleep did not. Language and sleep showed improvement only in early stages. Analysis of seizure frequency and type noted variability mirroring trends noted in adult studies of GAD encephalitis. CONCLUSIONS: This study demonstrated the variability of symptom profiles of pediatric GAD encephalitis and benefits of symptom severity scales. Symptom profiles and progression vary in this population.