Retrospective Pediatric Cohort Study Validates NEOS Score and Demonstrates Applicability in Children With Anti-NMDAR Encephalitis.

BACKGROUND AND OBJECTIVES: Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is the most common form of autoimmune encephalitis in children and adults. Although our understanding of the disease mechanisms has progressed, little is known about estimating patient outcomes. Therefore, the NEOS (anti-NMDAR Encephalitis One-Year Functional Status) score was introduced as a tool to predict disease progression in NMDARE. Developed in a mixed-age cohort, it currently remains unclear whether NEOS can be optimized for pediatric NMDARE. METHODS: This retrospective observational study aimed to validate NEOS in a large pediatric-only cohort of 59 patients (median age of 8 years). We reconstructed the original score, adapted it, evaluated additional variables, and assessed its predictive power (median follow-up of 20 months). Generalized linear regression models were used to examine predictability of binary outcomes based on the modified Rankin Scale (mRS). In addition, neuropsychological test results were investigated as alternative cognitive outcome. RESULTS: The NEOS score reliably predicted poor clinical outcome (mRS ≥3) in children in the first year after diagnosis (p = 0.0014) and beyond (p = 0.036, 16 months after diagnosis). A score adapted to the pediatric cohort by adjusting the cutoffs of the 5 NEOS components did not improve predictive power. In addition to these 5 variables, further patient characteristics such as the "Herpes simplex virus encephalitis (HSE) status" and "age at disease onset" influenced predictability and could potentially be useful to define risk groups. NEOS also predicted cognitive outcome with higher scores associated with deficits of executive function (p = 0.048) and memory (p = 0.043). DISCUSSION: Our data support the applicability of the NEOS score in children with NMDARE. Although not yet validated in prospective studies, NEOS also predicted cognitive impairment in our cohort. Consequently, the score could help identify patients at risk of poor overall clinical outcome and poor cognitive outcome and thus aid in selecting not only optimized initial therapies for these patients but also cognitive rehabilitation to improve long-term outcomes.

The Curse of Apneic Spells.

A 6-year-old girl had reduced fetal movements, numerous apneic spells, muscle hypotonia, and developmental motor delay. Her muscle biopsy tissue showed variation in myofiber diameters, small minicores by electron microscopy, and near-uniformity of type I fibers. Although no mutations were detected in RYR1, SEPN1, and DMPK genes, the RAPSN gene revealed one known mutation, p.Asn88Lys, from the mother, and one novel mutation, p.Cys366Gly, from the father. Life-saving pyridostigmine treatment suppressed her apneic spells and improved her motor development.

Recovery of the precision grip in children after traumatic brain injury.

OBJECTIVE: To identify quantitative parameters that are sensitive enough to detect impairments and improvements of grasping in children after traumatic brain injury (TBI) by analyzing the isometric fingertip forces of a precision grip-lift task. DESIGN: Follow-up and case-control study. SETTING: Tertiary pediatric trauma rehabilitation center in Germany. PARTICIPANTS: Thirteen children (age range, 5-14 y) with moderate or severe TBI. Trauma severity was assessed with the Glasgow Coma Scale (score range, 3-9) and the Injury Severity Score (range, 16-66 points). Control data were obtained from 13 age- and gender-matched healthy children. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Children were examined 3 times (t0, t1, t2). The first date of examination (t0) was defined by the Barthel Index (part B, >20 points). Reexaminations followed after 1 (t1) and 5 (t2) months of inpatient rehabilitation. Quantitative measures included 3 grip-force parameters, 2 load force parameters, 1 parameter of the coordination between grip force and load force, and 3 timing parameters in a precision grip-lift task. Clinical improvements and recovery of activities of daily living were described with the Barthel Index (qualitative measure). RESULTS: Peak grip force, maximum negative load force, grip force in the static phase and its standard deviation, and grip-force/load-force ratio at maximum grip force showed significant improvements during the observation period (5 mo). Also, the preparation phase and preload duration, but not the load duration, changed significantly. CONCLUSIONS: Impairments and the recovery of grasping in children after TBI can be objectified with quantitative analyses of the precision grip. Several grip-force and timing parameters were sensitive for the description of restitution processes.

Sensorimotor recovery in children after traumatic brain injury: analyses of gait, gross motor, and fine motor skills.

The recovery of gait, gross motor proficiency, and hand function was examined in 23 children (13 males, 10 females; age 4 years 7 months to 15 years 10 months) with traumatic brain injury (TBI) over five months of in patient rehabilitation. We used gait analysis, the Gross Motor Function Measure, the Developmental Hand Function Test, and the Purdue Pegboard test. Brain injury had been severe (initial Glasgow Coma Scale GCS <8) in 17 children and moderate (GCS 8-10) in six children. Compared with healthy control children of the same age and sex, repeated gait analyses in ambulatory children with brain injury showed significant reductions of velocity, stride length and cadence, and impaired balance. Spatiotemporal gait variables were correlated with Gross Motor Function Measure scores. Hand function tests revealed deficits in fine motor skills, speed, and coordination. Degree of impairment increased with trauma severity. Despite significant improvements, differences in gait velocity, stride length, and hand function of children with brain injuries and controls were still present about 8 months after TBI. Hand motor skills improved less than gait. Young age at injury was not associated with better recovery.

Prenatal infarction of the left middle cerebral artery: A case report of excellent functional outcome.

Extensive cortical-subcortical damage of the left hemisphere, most likely due to prenatal infarction of the left middle cerebral artery, was accidentally detected in a 12-year-old left-handed girl. Since clinical symptoms were minimal, the lesion had not been discovered for more than a decade. Meticulous examination revealed some slowness and somatosensory deficits of the right hand. Yet, independent finger movements were preserved, and functional skills such as reaching and grasping were unimpaired, as documented with optoelectronic motion analysis. There were no mirror movements. Transcranial magnetic stimulation indicated that the right hand was controlled by spared cortical areas of the damaged hemisphere. Gait was normal. Neuropsychological examination confirmed regular development of language, intellect, and visuospatial abilities. This case report demonstrates exceptionally good functional outcome after early brain damage.