Acute Flaccid Myelitis.

Acute flaccid myelitis (AFM) is an incompletely understood neurologic disorder occurring in epidemic fashion causing weakness ranging from mild paresis to devastating paralysis in children and some adults. This article reviews the case definition of AFM as well as its epidemiology and association with enteroviral infection. The clinical presentation, diagnostic investigation with particular attention to electrodiagnostics, acute management, and surgical options are described. Clinical outcomes and considerations for acute and long-term rehabilitation management are discussed extensively based on review of current literature, highlighting avenues for further study.

Practical Approaches and Knowledge Gaps in the Care for Children With Leukodystrophies.

Leukodystrophies are a group of neurodegenerative genetic disorders that affect approximately 1 in 7500 individuals. Despite therapeutic progress in individual leukodystrophies, guidelines in neurologic care are sparse and consensus among physicians and caregivers remains a challenge. At patient advocacy meetings hosted by Hunter's Hope from 2016-2018, multidisciplinary experts and caregivers met to conduct a literature review, identify knowledge gaps and summarize best practices regarding neurologic care. Stages of severity in leukodystrophies guided recommendations to address different levels of need based on a newly defined system of disease severity. Four core neurologic domains prioritized by families were identified and became the focus of this guideline: sleep, pain, seizures/epilepsy, and language/cognition. Based on clinical severity, the following categories were used: presymptomatic, early symptomatic, intermediate symptomatic, and advanced symptomatic. Across the leukodystrophies, neurologic care should be tailored to stages of severity while accounting for unique aspects of every disease and multiple knowledge gaps present. Standardized tools and surveys can help guide treatment but should not overburden families.

Functional status of children with encephalitis in an inpatient rehabilitation setting: a case series.

INTRODUCTION: Patterns and predictors of recovery from encephalitis are poorly understood. METHODS: This study examined functional status and reviewed charts of all children who presented to a pediatric inpatient rehabilitation facility with encephalitis between 1996 and 2010. Functional status at admission and discharge from inpatient rehabilitation was evaluated using the Functional Independence Measure for Children (WeeFIM) Self-care, Mobility, Cognitive, and Total Developmental Functional Quotient scores (DFQ, % of age-appropriate function). Charts were reviewed to characterize key clinical features and findings. RESULTS: Of the 13 children identified, the mean age was 9 years (range 5-16) with 54% males. Mean WeeFIM Total DFQ at admission was 37 (range: 15-90) and at discharge was 64 (range: 16-96). Average change in WeeFIM Total DFQ from admission to discharge was 26.7 (range 0-55, p < 0.001). WeeFIM domain scores improved between admission and discharge (Self-Care: p < 0.001, Cognition: p < 0.01, Mobility: p < 0.001). Eleven children displayed significant impairments in functional skills, defined as DFQ of < or =85, at discharge. Key clinical features and findings were diverse and not related to functional outcome. CONCLUSIONS: Results suggest that significant functional improvement in children with encephalitis occurs during inpatient rehabilitation. Further research is necessary to identify predictors of outcome in children with encephalitis.

Update on pharmaceutical intervention for disorders of consciousness and agitation after traumatic brain injury in children.

Responsiveness and agitation are common targets for pharmaceutical intervention after traumatic brain injury (TBI) in children. This focused review presents a critical discussion of the limited literature available on the use of medications for disorders of consciousness and agitation in children with TBI. For disorders of consciousness, evidence from several small studies supports a potential benefit of dopaminergic agents for improving responsiveness in some children with lower levels of function after TBI. Larger studies, likely requiring multicenter collaborations, are needed to more definitively address questions regarding the use of medications for responsiveness in children with TBI. The literature regarding use of pharmaceutical agents for agitation in children with TBI is even more limited. The dearth of literature regarding the effects of medications used for agitation in children with TBI highlights the need for additional basic and clinical science contributions in this area.

Examining acute rehabilitation outcomes for children with total functional dependence after traumatic brain injury: a pilot study.

OBJECTIVE: To examine in a pilot cohort factors associated with functional outcome at discharge and 3-month follow-up after discharge from inpatient rehabilitation in children with severe traumatic brain injury (TBI) who entered rehabilitation with the lowest level of functional skills. PARTICIPANTS: Thirty-nine children and adolescents (3-18 years old) who sustained a severe TBI and had the lowest possible rating at rehabilitation admission on the Functional Independence Measure for Children (total score = 18). METHODS: Retrospective review of data collected as part of routine clinical care. RESULTS: At discharge, 59% of the children were partially dependent for basic activities, while 41% remained dependent for basic activities. Initial Glasgow Coma Scale score, time to follow commands, and time from injury to rehabilitation admission were correlated with functional status at discharge. Time to follow commands and time from injury to rehabilitation admission were correlated with functional status at 3-month follow-up. Changes in functional status during the first few weeks of admission were associated with functional status at discharge and follow-up. CONCLUSIONS: Even children with the most severe brain injuries, who enter rehabilitation completely dependent for all daily activities, have the potential to make significant gains in functioning by discharge and in the following few months. Assessment of functional status early in the course of rehabilitation contributes to the ability to predict outcome from severe TBI.

Physical Abilities and Mobility Scale: reliability and validity in children receiving inpatient rehabilitation for acquired brain injury.

OBJECTIVE: To investigate the psychometric properties of the Physical Abilities and Mobility Scale (PAMS) in children receiving inpatient rehabilitation for acquired brain injury (ABI). DESIGN: Admission and discharge PAMS item and total scores were evaluated. The WeeFIM was used as the criterion standard. A case study was used to illustrate the complementary nature of the PAMS and WeeFIM. SETTING: A single, free-standing, academically affiliated pediatric rehabilitation hospital. PARTICIPANTS: Children (N=107) aged 2 through 18 years receiving inpatient rehabilitation for ABI between March 2009 and March 2012. Forty-two additional children treated during this time were excluded because of missing PAMS data. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Internal consistency was evaluated using Cronbach alpha. Interrater reliability was evaluated through overall agreement, Pearson correlations, and intraclass correlations. Construct validity was examined through exploratory factor analysis. Criterion validity was explored through correlations of PAMS overall and factor scores with WeeFIM total and subscale scores. Sensitivity to recovery was examined using paired t tests, examining differences between admission and discharge scores for each item and for the total score. RESULTS: Internal consistency and interrater reliability were high. Factor analysis revealed 2 factors: lower-level skills and higher-level mobility skills. Correlations with the WeeFIM ranged from moderate to very strong; total PAMS score most strongly correlated with the WeeFIM mobility subscore. Total PAMS score and each item score significantly increased between admission and discharge. CONCLUSIONS: The PAMS is a reliable and valid measure of progress during inpatient rehabilitation for children with ABI. By capturing fine-grain progress toward both lower-level and higher-level mobility skills, the PAMS complements the WeeFIM in assessing functional gains during the rehabilitation stay.

Pediatric rehabilitation: 2. Environmental factors affecting participation.

OBJECTIVE: This self-directed learning module highlights the environmental factors that influence the participation of children and youth with disabilities in life situations, including activities of self-care, mobility, socialization, education, recreation, and community life. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. This module emphasizes the participation of children with disabilities in adapted sports and reviews mobility devices to promote function, in the context of the International Classification of Functioning and in reference to the Americans with Disabilities Act. It provides the physiatrist with strategies to promote community participation, functional independence and healthcare transitions for children with disabilities as they approach adulthood. The goal of this article is to improve the learner's treatment strategies to maximize the participation of children and youth with disabilities in all settings, particularly schools and communities.

Pediatric rehabilitation: 3. Facilitating family-centered treatment decisions.

OBJECTIVE: This self-directed learning module focuses on the role of accurate diagnosis, psychological support, and family integration of children who have chronic impairments such as pain, spasticity, or cognitive disability. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The role of therapeutic, medical (traditional and nontraditional) and psychological interventions that improve family and individual function are emphasized. The goal of this article is to refine a learner's knowledge of the impact family-centered care can have on the medical, psychological, financial, and functional capabilities of families to improve treatment decisions in the context of children with disability.

Pediatric rehabilitation: 4. Prescribing assistive technology to promote community integration.

OBJECTIVE: This self-directed learning module highlights the equipment and assistive technology needs of children and youth with disabilities. This article specifically focuses on preparing families and patients for equipment transitions that occur over the course of childhood and adolescence including progressing from stroller to wheelchair to powerchair, as well as job training and use of augmentative communication. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The goal of this article is to modify the learner's current practice techniques to ensure that assistive technology is used to promote community integration from early childhood through transition and into adulthood.

Pediatric rehabilitation: 1. Common medical conditions in children with disabilities.

OBJECTIVE: This self-directed learning module focuses on the physiatric management of the common morbidities associated with pediatric traumatic brain injury and cerebral palsy. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The goal of this article is to enhance the learner's knowledge regarding current physiatric management of complications related with pediatric traumatic brain injury and cerebral palsy.

Pediatric rehabilitation: 5. Transitioning teens with disabilities into adulthood.

OBJECTIVE: This self-directed learning module focuses on preparing adolescent patients with special health care needs for adulthood by promoting their independence in their own self-care; helping them to navigate issues of sexuality, marriage, and parenting; preparing the patient and family to make guardianship decisions during the transition between childhood and adulthood; and planning for higher education or vocation. Emphasis will be on the role of the physiatrist in providing this guidance and its importance in improving the patient's quality of life. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The goal of this article is to refine the learner's knowledge of preparing adolescent patients with special health care needs for adulthood to improve their quality of life.

Physiatric findings in individuals with Sturge-Weber syndrome.

Sturge-Weber syndrome is a rare neurocutaneous disorder that often results in functional impairment caused by motor (typically hemiparesis) and cognitive deficits. A retrospective chart review of physiatric evaluation of 30 individuals, aged 4 mos to 55 yrs (median age, 2.4 yrs), with Sturge-Weber syndrome with brain involvement was conducted for the purpose of summarizing physiatric findings and recommendations in this cohort. Presence or absence of motor, cognitive, and behavioral concerns and need for orthoses, spasticity management, and therapy services were noted. Hemiparesis was common, but the need for intervention for spasticity was rare. Cognitive and behavioral concerns were noted frequently, meriting additional evaluation. Case vignettes are presented to highlight (1) a child with repeated functional setbacks in association with increased seizure frequency who, with seizure control, demonstrated return to functional baseline and subsequent further skill development and (2) a child with Sturge-Weber syndrome who made functional gains with constraint-induced movement therapy.

Pediatric constraint-induced movement therapy: a promising intervention for childhood hemiparesis.

Experimental and clinical evidence is accumulating that supports the assertion that the damaged human brain is capable of responding to sensory stimulation in a sufficient manner to result in sustainable and useful benefits. The intensity and duration of therapeutic maneuvers that elicit improvement are under active investigation. Recent studies in animals, adults, and children with hemiparesis have shown that constraint of the less involved upper limb coupled with a behavioral program that repetitively encourages graded unilateral movements can result in long-term "new" functional activities. Constraint-induced movement therapy (CIMT) is a promising approach for treatment of children with stroke-related hemiparesis from either prenatal or postnatal causes due to the enhanced neuroplasticity of the brain during early life.

Preliminary evaluation of the Cognitive and Linguistic Scale: a measure to assess recovery in inpatient rehabilitation following pediatric brain injury.

OBJECTIVE: A preliminary investigation of the psychometric properties of the Cognitive and Linguistic Scale (CALS), a measure of cognitive and linguistic recovery following brain injury in children and adolescents. PARTICIPANTS: One hundred children and adolescents (aged 2-19 years) with acquired or traumatic brain injury were included. METHODS: The CALS was administered at inpatient rehabilitation admission and discharge. RESULTS: Internal consistency and interrater reliability were high. Factor analysis revealed 2 factors (basic responding, higher-level cognitive skills). Correlations with the Functional Independence Measure for Children (WeeFIM) ranged from 0.51 to 0.89; highest correlation was between WeeFIM cognitive domain and CALS total score. CALS scores improved significantly between admission and discharge. CONCLUSION: On the basis of these preliminary analyses, the CALS is a promising measure to track cognitive and linguistic recovery in children and adolescents with brain injury during inpatient rehabilitation.

Achieving functional ambulation in a patient with Chopart amputation.

Chopart amputation provides an interesting functional dilemma for the patient, physiatrist, and prosthetist. This problem is further complicated when the patient develops an equinus contracture from an unbalanced short forefoot lever. Also, genu recurvatum occurs as a consequence of recurrent knee hyperextension from this contracture. There are few reports on the most functional prostheses for this type of patient. We present a case of a pediatric patient with a Chopart amputation who demonstrated genu recurvatum during ambulation. After experimenting with many different types of prostheses, he had persistent lower-limb pain. We prescribed a Chopart prosthesis with a flexible inner socket and a carbon fiber energy-storing keel with posterior calf struts. At his follow-up clinic visit, he no longer had pain, and he was able to play soccer. This device provides a functional alternative for those patients with Chopart amputation who are unable to participate in athletic activities.

The efficacy of donepezil hydrochloride on memory functioning in three adolescents with severe traumatic brain injury.

OBJECTIVE: To explore efficacy of donepezil on memory in adolescents with severe traumatic brain injury (TBI). DESIGN: Single subject, unblinded, multiple baseline design. METHODS AND PROCEDURES: Memory functioning was examined in three adolescents with TBI on and off medication (5 and 10 mg) using the Selective Reminding Test. Four variables were examined: Total recall (TR) = number of words total words recalled, Long Term Storage (LTS) = words recalled on two trials in a row, Consistency of Long Term Retrieval (CLTR) = words continuously recalled, Delay = number of words recalled after delay. RESULTS: On medication, three out of three participants demonstrated better memory. Two showed greatest improvement on 10 mg. All participants demonstrated improvement in TR and LTS. Two participants demonstrated improved CLTR. No participants displayed improvement in Delay. No adverse side effects were reported. CONCLUSIONS: Results suggests that donepezil may be effective in improving memory in adolescents with severe TBI and warrant further examination.

Characteristics of auditory agnosia in a child with severe traumatic brain injury: a case report.

We present a case that is unusual in many respects from other documented incidences of auditory agnosia, including the mechanism of injury, age of the individual, and location of neurological insult. The clinical presentation is one of disturbance in the perception of spoken language, music, pitch, emotional prosody, and temporal auditory processing in the absence of significant deficits in the comprehension of written language, expressive language production, or peripheral auditory function. Furthermore, the patient demonstrates relatively preserved function in other aspects of audition such as sound localization, voice recognition, and perception of animal noises and environmental sounds. This case study demonstrates that auditory agnosia is possible following traumatic brain injury in a child, and illustrates the necessity of assessment with a wide variety of auditory stimuli to fully characterize auditory agnosia in a single individual.