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.
View Article and Find Full Text PDFLeukodystrophies 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.
View Article and Find Full Text PDFIntroduction: 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).
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.
View Article and Find Full Text PDFObjective: 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.
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.
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.
View Article and Find Full Text PDFExperimental 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.
View Article and Find Full Text PDFWe 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.
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