Prolonged duration of post-traumatic amnesia: A sensitive classification for predicting cognitive outcomes in children recovering from traumatic brain injury.

OBJECTIVE: A paucity of data exists regarding the duration of post-traumatic amnesia (PTA) as a predictor of cognitive functioning among children after traumatic brain injury (TBI). The study aimed to assess the relationship between PTA duration and areas of neurocognitive function among the pediatric population in the sub-acute phase of recovery and rehabilitation. METHODS: Data were collected from medical files on 103 children aged 5.5-16.5 hospitalized at a pediatric rehabilitation department with a diagnosis of moderate-severe TBI (msTBI) between the years 2004-2019. The Children Orientation and Amnesia Test was used to evaluate PTA duration. Measures of high-order cognitive abilities of attention and executive function were collected using the Test of Everyday Attention-Child version (TEA-Ch). RESULTS: Three PTA duration groups were assembled out of a cluster analysis: "Long PTA" (M = 21 days), "Very Long PTA" (M = 47 days), and "Extremely Long PTA" (M = 94 days). Analyses revealed that the "Long PTA" group preformed significantly better than the "Very Long PTA" and "Extremely Long PTA" groups on all TEA-Ch measures, that is, Selective Attention, Attentional Control Switching, and Sustained Attention. CONCLUSIONS: This study is the first to demonstrate that PTA duration is a useful predictor of high-order cognitive functions among children with msTBI in the sub-acute phase of recovery and rehabilitation. The findings emphasize the importance of using a more sensitive classification of prolonged PTA durations to improve outcome prediction and allocation of resources to those who can benefit most after severe brain injuries.

Mitochondrial augmentation of hematopoietic stem cells in children with single large-scale mitochondrial DNA deletion syndromes.

Patients with single large-scale mitochondrial DNA (mtDNA) deletion syndromes (SLSMDs) usually present with multisystemic disease, either as Pearson syndrome in early childhood or as Kearns-Sayre syndrome later in life. No disease-modifying therapies exist for SLSMDs. We have developed a method to enrich hematopoietic cells with exogenous mitochondria, and we treated six patients with SLSMDs through a compassionate use program. Autologous CD34+ hematopoietic cells were augmented with maternally derived healthy mitochondria, a technology termed mitochondrial augmentation therapy (MAT). All patients had substantial multisystemic disease involvement at baseline, including neurologic, endocrine, or renal impairment. We first assessed safety, finding that the procedure was well tolerated and that all study-related severe adverse events were either leukapheresis-related or related to the baseline disorder. After MAT, heteroplasmy decreased in the peripheral blood in four of the six patients. An increase in mtDNA content of peripheral blood cells was measured in all six patients 6 to 12 months after MAT as compared baseline. We noted some clinical improvement in aerobic function, measured in patients 2 and 3 by sit-to-stand or 6-min walk testing, and an increase in the body weight of five of the six patients suffering from very low body weight before treatment. Quality-of-life measurements as per caregiver assessment and physical examination showed improvement in some parameters. Together, this work lays the ground for clinical trials of MAT for the treatment of patients with mtDNA disorders.

Online Awareness among Adolescents with Acquired Brain Injury: Preliminary Findings following Performance of Motor, Cognitive and Functional Tasks.

Aims: Online awareness is an ongoing ability to monitor performance within the stream of action. It involves the ability to detect errors emerging during actual performance, as well as to anticipate potential problems. This preliminary within-subject study aimed to evaluate emergent and anticipatory online awareness among adolescents with Acquired Brain Injury (ABI) during performance of motor, cognitive and functional tasks.Methods: 14 adolescents (11-18 years) following ABI were recruited. Before and after completion of the tasks, participants fulfilled task-related awareness questionnaires.Results: In the motor task, no significant correlations were found between heart rate and the subjective perceived exertion scale (emergent awareness). In the cognitive task, no significant correlations were found between the estimated difficulty before the task (anticipatory awareness) and actual performance, however a significant correlation was found between performance and the self-evaluation of performance (emergent awareness), in the easiest item of the task. In the functional task, two main patterns of online awareness were recognized: accurate and overestimation of performance.Conclusions: Online awareness deficits in adolescents after ABI, vary as a function of task characteristics. Clinicians who aim to improve online awareness should direct interventions to mainly include functional tasks, as compared to cognitive and motor tasks.

Growing up with Bilateral parieto-occipital injury: over ten years of clinical observation and neuropsychological follow-up.

This paper presents a follow-up of a child with Balint's syndrome over more than a decade. The patient experienced traumatic brain injury before age 12, resulting in bilateral occipito-parietal infarctions and a clinical presentation of Balint's syndrome. Neuropsychological assessments at three time points showed average verbal abilities alongside persistent difficulties in visual orientation, mirrored in the patient's daily life. Her outstanding compensatory abilities in the face of these impairments are discussed with respect to the recruitment of the ventral visual stream and the role of top-down processing. This profile may help to determine interventions for younger patients with similar lesions.

Despite dystonia: natural history of delayed-onset pediatric secondary dystonia.

Background: Dystonia is a movement disorder involving involuntary movements and/or postures of the limbs, trunk, neck or face. Secondary dystonia following brain injury is uncommon, with unfavorable long-term consequences. Given the limited evidence regarding pediatric secondary dystonia, this study's aim was to document the natural history of the condition and the effect of interventions on its symptomatology. Methods: We describe three cases of girls (age 8 to 11 y) who developed dystonia secondary to an acquired brain injury, received intensive rehabilitation treatments and were followed for 8-33 months post-injury. In all three cases, secondary dystonia appeared 1-3 months post-insult. Results: In none of the cases was there alleviation of dystonic symptoms over time despite physical and pharmacological interventions; in two cases the dystonic hand is now used as an assisting hand only, whereas in the third it is completely non-functional. However, despite their impairment, two girls achieved basic functional independence and one is partly independent in activities of daily living. Conclusions: Rehabilitation professionals who work with pediatric patients susceptible to developing secondary dystonia should be aware of its possible consequences and inform families and staff. Intensive task-specific training during rehabilitation may be key to regaining overall functional capabilities despite residual impairment.

Functional Plasticity in the Absence of Structural Change.

This work presents a case of a young woman with apraxia and a severe body scheme disorder, 10 years after a childhood frontal and occipitoparietal brain injury. Despite specific limitations, she is independent in performing all activities of daily living. A battery of tests was administered to evaluate praxis and body representations. Specifically, the Hand Laterality Test was used to compare RS's dynamic body representation to that of healthy controls (N = 14). Results demonstrated RS's severe praxis impairment, and the Hand Laterality Test revealed deficits in accuracy and latency of motor imagery, suggesting a significant impairment in dynamic body representation. However, semantic and structural body representations were intact. These results, coupled with frequent use of verbalizations as a strategy, suggest a possible ventral compensatory mechanism (top-down processing) for dorsal stream deficits, which may explain RS's remarkable recovery of activities of daily living. The link between praxis and dynamic body representation is discussed.

Comparing memory and meta-memory abilities between children with acquired brain injury and healthy peers.

This study compared visual memory and meta-memory abilities of children with ABI to that of healthy peers. Participants included 16 children (aged 13.55 ± 3.29 years) with moderate or severe ABI and 16 healthy children (aged 12.44 ± 3.24 years) with typical development. Children completed the Contextual Memory Test for Children (CMT-CH). The study group showed significantly lower immediate and delayed recall abilities. While the controls used the context for better memorizing, most of the children with ABI used rehearsals. In both groups better delayed recall correlated with the use of a more efficient strategy. Meta-memory should be an integral part of the assessment for children with ABI. Therapists should enhance child's self-awareness to his/her abilities and encourage the use of strategies (e.g. context) for memorizing in daily life.

Discrepancies between mothers and clinicians in assessing functional capabilities and performance of children with cerebral palsy.

The current study is a cross-sectional study that aimed to investigate the concordance between health care professionals (HCPs) and mothers in rating capabilities and performance of children with cerebral palsy (CP), and the impact of CP gross motor severity on concordance. Seventy-three children with mild-to-severe CP (mean age 8.8 ± 2.10 years) and their mothers participated in this study. Two modes of Pediatric Evaluation Disability Inventory (PEDI) administration were used: mothers' interview by a social worker and HCPs' actual evaluation. Differences between raters were assessed by paired t-tests and intra-class correlation coefficients (ICCs). Agreement was defined as mean absolute difference of less than or equal to six points. The results indicated that in spite of excellent overall ICCs in PEDI (ICC>0.8), disagreement between raters was observed in all PEDI sub-domains: 38%, 56%, 72% and 59% disagreement in Functional Skills-Mobility, Functional Skills-Self Care, Caregiver Assistance-Mobility (CA-MO) and Caregiver Assistance-Self Care (CA-SC), respectively. In CA-SC and CA-MO disagreement mainly consisted of mothers rating their children lower in performance than HCPs. CP severity effected the agreement mostly in children with moderate CP severity. The implications of these results are that raters perceive child's activity differently, hence revealing hidden disability perceptions, with significant consequences for intended interventions.

Reliability and validity of Hebrew Pediatric Evaluation of Disability Inventory (PEDI) in children with cerebral palsy -- health care professionals vs. mothers.

AIM: To evaluate the reliability and validity of the PEDI in Hebrew (PEDI-H) in children with cerebral palsy (CP) using health care professionals' (HCP) and mothers' evaluations. METHODS: The sample comprised 73 participants (40 males, 33 females) with CP. Two modes of PEDI-H administration were used: interview of the mothers by a social worker and HCP evaluation. PEDI-H reliability was examined by two modes: 1) internal consistency via Cronbach's alpha and 2) overall absolute agreement within subject reliability via intraclass correlation coefficient (ICC). Discriminative validity using collapsed strata of the Gross Motor Functional Classification System (GMFCS) (area under the curve=AUC) were examined for each of the PEDI-H sub-domains. RESULTS: Participants' mean age was 8 years 8 months (standard deviation (SD) 2 years 10 months). The reliability of mothers' PEDI-H was good-to-excellent (Cronbach's alpha=0.889-0.964, ICC=0.845-0.938). The HCPs' reliability was excellent (Cronbach's alpha and ICCs > 0.90). The PEDI-H was also reliable in children with mild, moderate, and severe CP (GMFCS=I+II, III and IV+V, respectively), in younger (6-7 years) and older children (8-12 years), and in children with various CP distribution. Mothers and HCPs had low accuracy in Social-Function domains (AUC=0.538-0.686) and moderate-to-high accuracy in Mobility and Self-Care domains (AUC=0.887-0.967). PEDI-H was able to distinguish between children with various CP severities. CONCLUSION: The PEDI-H has good psychometric properties when administered by mothers and HCPs and can be used in older children with CP.