Nerve Transfer Surgery in Acute Flaccid Myelitis: Prognostic Factors, Long-Term Outcomes, Comparison With Natural History.

BACKGROUND: Nerve transfer surgery is sometimes offered to patients with acute flaccid myelitis (AFM). The objectives of this study were to evaluate surgical efficacy, assess which clinical and neurophysiological data are valuable for preoperative planning, and report long-term outcomes. METHODS: This is a single-center, retrospective case series of patients with AFM who received nerve transfer surgery. All patients had preoperative electromyography and nerve conduction studies (EMG/NCS). Matched control muscles that did not receive nerve transfer surgery were defined in the same cohort. RESULTS: Ten patients meeting inclusion criteria received a total of 23 nerve transfers (19 upper extremity, four lower extremity). The mean age at symptom onset was 3.8 years, surgery was 0.5 to 1.25 years after diagnosis, and mean follow-up was 2.3 years (range 1.3 to 4.5 years). Among muscles with preoperative strength Medical Research Council (MRC) grade 0, muscles receiving nerve transfers performed significantly better than those that did not (MRC grade 2.17 ± 0.42 vs 0 ± 0, respectively, P = 0.0001). Preoperative EMG/NCS predicted worse outcomes in recipient muscles with more abundant acute denervation potentials (P = 0.0098). Donor nerves found to be partially denervated performed equally well as unaffected nerves. Limited data suggested functional improvement accompanying strength recovery. CONCLUSIONS: Nerve transfer surgery is an effective strategy to restore strength for patients with AFM with persistent, severe motor deficits. Postoperative outcomes in patients with complete paralysis are better than the natural history of disease. This study demonstrates the utility of preoperative clinical and electrophysiological data in guiding patient selection for nerve transfer surgery.

Targeting Sarcopenia as an Objective Clinical Outcome in the Care of Children with Spinal Cord-Related Paralysis: A Clinician's View.

Muscle loss is consistently associated with immobility and paralysis and triggers significant metabolic and functional changes. The negative effects of sarcopenia are amplified in children who are in the process of building their muscle mass as part of development. Because muscle mass loss is consistently associated with increased morbidity and mortality throughout life, optimizing the size and health of muscles following a neurologic injury is an objective target for therapeutic interventions. This review hypothesizes that muscle mass correlates with functional outcomes in children with paralysis related to spinal cord-related neurologic deficits. We propose that the measurement of muscle mass in this population can be used as an objective outcome for clinical long-term care. Finally, some practical clinical approaches to improving muscle mass are presented.

Using Billing Codes to Create a Pediatric Functional Status e-Score for Children Receiving Inpatient Rehabilitation.

OBJECTIVE: Provide proof-of-concept for development of a Pediatric Functional Status eScore (PFSeS). Demonstrate that expert clinicians rank billing codes as relevant to patient functional status and identify the domains that codes inform in a way that reliably matches analytical modeling. DESIGN: Retrospective chart review, modified Delphi, and nominal group techniques. SETTING: Large, urban, quaternary care children's hospital in the Midwestern United States. PARTICIPANTS: Data from 1955 unique patients and 2029 hospital admissions (2000-2020); 12 expert consultants representing the continuum of rehabilitation care reviewed 2893 codes (procedural, diagnostic, pharmaceutical, durable medical equipment). MAIN OUTCOME MEASURES: Consensus voting to determine whether codes were associated with functional status at discharge and, if so, what domains they informed (self-care, mobility, cognition/ communication). RESULTS: The top 250 and 500 codes identified by statistical modeling were mostly composed of codes selected by the consultant panel (78%-80% of the top 250 and 71%-78% of the top 500). The results provide evidence that clinical experts' selection of functionally meaningful codes corresponds with codes selected by statistical modeling as most strongly associated with WeeFIM domain scores. The top 5 codes most strongly related to functional independence ratings from a domain-specific assessment indicate clinically sensible relationships, further supporting the use of billing data in modeling to create a PFSeS. CONCLUSIONS: Development of a PFSeS that is predicated on billing data would improve researchers' ability to assess the functional status of children who receive inpatient rehabilitation care for a neurologic injury or illness. An expert clinician panel, representing the spectrum of medical and rehabilitative care, indicated that proposed statistical modeling identifies relevant codes mapped to 3 important domains: self-care, mobility, and cognition/communication.

Challenges in the Clinical Recognition of Acute Flaccid Myelitis and its Implications.

OBJECTIVES: To explore the challenges in diagnosing acute flaccid myelitis (AFM) and evaluate clinical features and treatment paradigms associated with under recognition. STUDY DESIGN: This was a retrospective multicenter study of pediatric patients (≤18 years) who were diagnosed with AFM from 2014 to 2018 using the Centers for Disease Control and Prevention's case definition. RESULTS: In 72% of the cases (126 of 175), AFM was not considered in the initial differential diagnosis (n = 108; 61.7%) and/or the patient was not referred for acute care (n = 90; 51.4%) at the initial clinical encounter, and this did not improve over time. Although many features of the presentation were similar in those initially diagnosed with AFM and those who were not; preceding illness, constipation, and reflexes differed significantly between the 2 groups. Patients with a non-AFM initial diagnosis more often required ventilatory support (26.2% vs 12.2%; OR, 0.4; 95% CI, 0.2-1.0; P = .05). These patients received immunomodulatory treatment later (3 days vs 2 days after neurologic symptom onset; 95% CI, -2 to 0; P = .05), particularly intravenous immunoglobulin (5 days vs 2 days; 95% CI, -4 to -2; P < .001). CONCLUSIONS: Delayed recognition of AFM is concerning because of the risk for respiratory decompensation and need for intensive care monitoring. A non-AFM initial diagnosis was associated with delayed treatment that could have a clinical impact, particularly as new treatment options emerge.

Bilateral Dega and Varus Derotational Osteotomies for Painful Hip Subluxation in Acute Flaccid Myelitis: A Case Report.

Introduction: Acute flaccid myelitis (AFM) is a recently described diagnosis that primarily impacts the pediatric population. It is characterized by profound proximal muscle weakness with resultant orthopedic manifestations similar to well-known neuromuscular conditions. While the incidence of AFM has been rising, management outcomes are understudied. Here, we describe the first known case of hip reconstruction in AFM. Case Report: A 5-year-old female presented with painful bilateral hip subluxations 2 years after being diagnosed with AFM. Imaging confirmed substantial uncovering of the femoral heads, right greater than left, with reduction on abduction views. Given the extent of her hip pathology and symptoms, she underwent bilateral Dega and varus derotational osteotomies with adductor lengthening, achieving a 35° correction in femoral neck angle and 30° reduction in femoral anteversion bilaterally. At 2 years postoperatively, she was asymptomatic without recurrence of hip displacement. Conclusion: Reconstructive femoral osteotomies can be effective for achieving painless, reduced hips in patients with AFM. Thus, surgeons may reasonably extrapolate current concepts utilized for other low-tone neuromuscular conditions to inform approach to AFM.

Acute flaccid myelitis: cause, diagnosis, and management.

Acute flaccid myelitis (AFM) is a disabling, polio-like illness mainly affecting children. Outbreaks of AFM have occurred across multiple global regions since 2012, and the disease appears to be caused by non-polio enterovirus infection, posing a major public health challenge. The clinical presentation of flaccid and often profound muscle weakness (which can invoke respiratory failure and other critical complications) can mimic several other acute neurological illnesses. There is no single sensitive and specific test for AFM, and the diagnosis relies on identification of several important clinical, neuroimaging, and cerebrospinal fluid characteristics. Following the acute phase of AFM, patients typically have substantial residual disability and unique long-term rehabilitation needs. In this Review we describe the epidemiology, clinical features, course, and outcomes of AFM to help to guide diagnosis, management, and rehabilitation. Future research directions include further studies evaluating host and pathogen factors, including investigations into genetic, viral, and immunological features of affected patients, host-virus interactions, and investigations of targeted therapeutic approaches to improve the long-term outcomes in this population.

A Primary Care Provider's Guide to Bone Health in Spinal Cord-Related Paralysis.

Individuals with spinal cord injury/disorder (SCI/D) are at high risk for developing secondary osteoporosis. Bone loss after neurologic injury is multifactorial and is dependent on the time from and extent of neurologic injury. Most bone loss occurs in the first year after complete motor paralysis, and fractures occur most commonly in the distal femur and proximal tibia (paraplegic fracture). The 2019 International Society for Clinical Densitometry Position Statement in SCI establishes that dual-energy X-ray absorptiometry (DXA) can be used to both diagnose osteoporosis and predict lower extremity fracture risk in individuals with SCI/D. Pharmacologic treatments used in primary osteoporosis have mixed results when used for SCI/D-related osteoporosis. Ambulation, standing, and electrical stimulation may be helpful at increasing bone mineral density (BMD) in individuals with SCI/D but do not necessarily correlate with fracture risk reduction. Clinicians caring for individuals with spinal cord-related paralysis must maintain a high index of suspicion for fragility fractures and consider referral for surgical evaluation and management.

Early use of an implantable diaphragm pacing stimulator for a child with severe acute flaccid myelitis-a case report.

Introduction: Acute Flaccid Myelitis (AFM) is a recently recognized, polio-like illness of children that can be functionally devastating. Severe cases can lead to ventilatory failure. Incomplete phrenic nerve injuries in other populations has been shown to respond to diaphragmatic stimulation. We therefore proposed an early assessment for incomplete denervation by laparoscopic direct stimulation of the diaphragm and placement of a diaphragmatic pacing system to enhance diaphragm function. Case presentation: A 3 year-old girl presented with AFM with clinically and electrodiagnostically severe involvement of all four limbs and muscles of respiration. Direct stimulation of the diaphragm demonstrated contraction and a diaphragmatic stimulator was placed at 3 weeks post presentation. The patient was immediately able to tolerate short bouts of reduced ventilation settings. Electromyography via the pacing wires demonstrated intact motor units consistent with partial denervation/reinnervation in the left hemidiaphragm, and no motor units in the right hemidiaphragm. At three months, she tolerated 6 h of pacing on pressure support setting. At 5 months she demonstrated larger tidal volumes with active pacing than without. Discussion: In our experience, AFM patients who require chronic ventilator support are rarely able to be weaned. Despite clinical and surface electrodiagnostic evidence of complete phrenic nerve involvement, the patient's diaphragm responded to direct stimulation. The patient preferred pacing over non-pacing times and showed improved ventilatory ability with pacing as opposed to without, though remains ventilator-dependent. These findings support augmentation of diaphragm function and possible enhanced recovery of spontaneous function.

Subject-specific regional measures of water diffusion are associated with impairment in chronic spinal cord injury.

PURPOSE: We aimed to identify non-invasive imaging parameters that can serve as biomarkers for the integrity of the spinal cord, which is paramount to neurological function. Diffusion tensor imaging (DTI) indices are sensitive to axonal and myelin damage, and have strong potential to serve as such biomarkers. However, averaging DTI indices over large regions of interest (ROIs), a common approach to analyzing the images of injured spinal cord, leads to loss of subject-specific information. We investigated if DTI-tractography-driven, subject-specific demarcation approach can yield measures that are more specific to impairment. METHODS: In 18 individuals with chronic spinal cord injury (SCI), subject-specific demarcation of the injury region was performed using DTI tractography, which yielded three regions relative to injury (RRI; regions superior to, at, and below injury epicenter). DTI indices averaged over each RRI were correlated with measures of residual motor and sensory function, obtained using the International Standard of Neurological Classification for Spinal Cord Injury (ISNCSCI). RESULTS: Total ISNCSCI score (ISNCSCI-tot; sum of ISNCSCI motor and sensory scores) was significantly (p < 0.05) correlated with fractional anisotropy and axial and radial diffusivities. ISNCSCI-tot showed strongest correlation with indices measured from the region inferior to the injury epicenter (IRRI), the degree of which exceeded that of those measured from the entire cervical cord-suggesting contribution from Wallerian degeneration. CONCLUSION: DTI tractography-driven, subject-specific injury demarcation approach provided measures that were more specific to impairment. Notably, DTI indices obtained from the IRRI region showed the highest specificity to impairment, demonstrating their strong potential as biomarkers for the SCI severity.

Characteristics of individuals seeking activity-based restorative therapy following spinal cord injury: A focus on hope.

BACKGROUND: There is a lack of evidence regarding the psychosocial characteristics of individuals with spinal cord injury (SCI) undergoing activity-based restorative therapy (ABRT) treatment. OBJECTIVES: This study seeks to describe the hopefulness of a sample of ABRT participants and describe the relationship between hopefulness and level of rehabilitation engagement. METHODS: A prospective cross-sectional cohort study was conducted using a convenience sample of 73 individuals with SCI (mean time since injury = 66.6 months) seeking out-patient, post-acute rehabilitation at a metropolitan ABRT center. Outcome measures included a demographic survey, The Hope Scale, the Patient Health Questionare-9 and the Hopkins Rehabilitation Engagement Rating Scale. RESULTS: The ABRT group reported higher levels of hope (M = 54.78, SD = 7.13) than have been reported in a sample of individuals with SCI seeking traditional rehabilitation in the acute (M = 24.58, SD = 4.06) setting. Rehabilitation engagement was not related to hopefulness in the ABRT group. CONCLUSIONS: The ABRT group demonstrated high levels of hopefulness. The difference in hopefulness noted between this group and previous studies could be due to the time elapsed since injury, the presence of choice in the rehabilitation process, or the possibility that individuals who chose to participate in ABRT may have inherently different hopefulness characteristics than the broader SCI population.

Activity-Based Restorative Therapies after Spinal Cord Injury: Inter-institutional conceptions and perceptions.

This manuscript is a review of the theoretical and clinical concepts provided during an inter-institutional training program on Activity-Based Restorative Therapies (ABRT) and the perceptions of those in attendance. ABRT is a relatively recent high volume and intensity approach toward the restoration of neurological deficits and decreasing the risk of secondary conditions associated with paralysis after spinal cord injury (SCI). ABRT is guided by the principle of neuroplasticity and the belief that even those with chronic SCI can benefit from repeated activation of the spinal cord pathways located both above and below the level of injury. ABRT can be defined as repetitive-task specific training using weight-bearing and external facilitation of neuromuscular activation. The five key components of ABRT are weight-bearing activities, functional electrical stimulation, task-specific practice, massed practice and locomotor training which includes body weight supported treadmill walking and water treadmill training. The various components of ABRT have been shown to improve functional mobility, and reverse negative body composition changes after SCI leading to the reduction of cardiovascular and other metabolic disease risk factors. The consensus of those who received the ABRT training was that ABRT has much potential for enhancement of recovery of those with SCI. Although various institutions have their own strengths and challenges, each institution was able to initiate a modified ABRT program.

Traumatic Spinal Cord Injury Emergency Service Triage Patterns and the Associated Emergency Department Outcomes.

Paralysis is an indication for trauma patients to be preferentially triaged by emergency services to designated level I or II trauma centers (TC). We sought to describe triage practices for patients with acute traumatic spinal cord injury (TSCI) and its associated emergency department (ED) outcomes. Adults ages ≥ 18 years with a diagnosis of acute TSCI (International Classification of Diseases-9: 806 and 952) in the 2006-2011 United States Nationwide Emergency Department Sample were included in these analyses. Outcomes assessed include triage to non-trauma centers (NTC), which is referred to as "under-triage," and ED mortality. Of 117,444 adults with TSCI, 33.4% were under-triaged to NTC. Under-triage was more prevalent with increasing age. Among patients under-triaged to NTC, 37.4% had new injury severity score (NISS) >15, representing severe injuries or polytrauma. Among patients with NISS >15, the odds of ED mortality in NTC were four-fold greater compared to level I trauma centers (TC-I) (adjusted odds ratio [AOR] = 4.06; 95% confidence interval = 1.87-8.79; p < 0.001). In conclusion, under-triage of adults with acute TSCI occurred in at least one-third of the cases. Patients triaged to NTC rather than TC-I experienced higher likelihood of death in the ED even after controlling for personal and injury characteristics. Further research is necessary to elucidate detailed clinical and logistical factors that may be associated with under-triage of acute TSCI, to facilitate interventions aimed at improving patient experience and outcomes.

Functional electrical stimulation as a component of activity-based restorative therapy may preserve function in persons with multiple sclerosis.

OBJECTIVE: To examine the effect of functional electrical stimulation (FES) cycling on disability progression in persons with multiple sclerosis (MS). DESIGN: Retrospective cohort, 40 participants with mean follow-up of 15 months. Setting International Center for Spinal Cord Injury at Kennedy Krieger Institute in Baltimore, a rehabilitation referral center. PARTICIPANTS: Forty consecutive persons with MS undergoing rehabilitation from 2007 to 2011, with at least two evaluations based on the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI). Interventions FES cycling as part of activity-based restorative therapy interventions. OUTCOME MEASURES: Change in Expanded Disability Status Scale (EDSS) and ISNCSCI motor, light touch, and pin prick scores from baseline to latest evaluation. RESULTS: In 71% of patients, activity-based rehabilitation included FES cycling. There was no disability progression on the EDSS. Lower extremity motor scores improved or stabilized in 75% of patients with primary progressive MS (PPMS), 71.4% with secondary progressive MS (SPMS), and 54.5% with relapsing remitting MS (RRMS). Among patients with improved or stabilized lower extremity motor function, PPMS recorded a mean 9% improvement, SPMS 3% and RRMS 6%. In PPMS, use of FES showed trend towards improvement in motor scores (P = 0.070). CONCLUSIONS: FES as part of activity-based rehabilitation may help preserve or improve neurological function in patients with MS.

Bone mass in individuals with chronic spinal cord injury: associations with activity-based therapy, neurologic and functional status, a retrospective study.

OBJECTIVE: To describe the prevalence of osteoporosis and its association with functional electrical stimulation (FES) use in individuals with spinal cord injury (SCI)-related paralysis. DESIGN: Retrospective cross-sectional evaluation. SETTING: Clinic. PARTICIPANTS: Consecutive persons with SCI (N=364; 115 women, 249 men) aged between 18 and 80 years who underwent dual-energy x-ray absorptiometry (DXA) examinations. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: Prevalence of osteoporosis defined as DXA T score ≤-2.5. RESULTS: The prevalence of osteoporosis was 34.9% (n=127). Use of FES was associated with 31.2% prevalence of osteoporosis compared with 39.5% among persons not using FES. In multivariate adjusted logistic regression analysis, FES use was associated with 42% decreased odds of osteoporosis after adjusting for sex, age, body mass index, type and duration of injury, Lower Extremity Motor Scores, ambulation, previous bone fractures, and use of calcium, vitamin D, and anticonvulsant; (adjusted odds ratio [OR]=.58; 95% confidence interval [CI], .35-.99; P=.039). Duration of injury >1 year was associated with a 3-fold increase in odds of osteoporosis compared with individuals with injury <1 year; (adjusted OR=3.02; 95% CI, 1.60-5.68; P=.001). CONCLUSIONS: FES cycling ergometry may be associated with a decreased loss of bone mass after paralysis. Further prospective examination of the role of FES in preserving bone mass will improve our understanding of this association.

The epidemiology of childhood and adolescent traumatic spinal cord injury in the United States: 2007-2010.

The burden of acute traumatic spinal cord injury (TSCI) among U. S. children and adolescents was last described over a decade ago using inpatient data. We describe cumulative incidence, mortality, discharge disposition, and inflation-adjusted charges of childhood and adolescent TSCI in the U.S. using emergency department (ED) data from the Nationwide Emergency Department Sample (2007-2010). Patients ages 17 years and younger with a diagnosis of acute TSCI were identified using the International Classification of Diseases, Ninth Revision (ICD-9) diagnosis codes 806.* and 952.* (N = 6132). The cumulative incidence of childhood and adolescent TSCI averaged 17.5 per million population per year. The median age at presentation was 15 years (interquartile range [IQR] = 12-16) and the majority of patients were male (72.5%). The overall median new injury severity score (NISS) was 16 (IQR = 9-27), remaining unchanged during the study duration (p=0.703). Children 5 years and younger were more likely to be injured from a road traffic accident (RTA; 50.9%), sustain C1-C4 injuries (47.4%), have more severe injury (median NISS = 22; IQR = 13-29), and concurrent brain injury (24%) compared to older children and adolescents, p<0.001. Firearms were implicated in 8.3% of injuries, of which 94.7% were among adolescents ages 13-17 years. Of the 35 TSCI-related ED deaths, 40% occurred among children ages 5 years and younger. 62.4% of patients required inpatient admission. Despite stable cumulative incidence and overall injury severity, ED treatment charge per visit increased on average $1394 from $3495 in 2007 to $4889 in 2010 (p=0.008). RTA-related TSCI disproportionately affects young children, while firearm-related TSCI is most common among adolescents. These findings inform TSCI prevention strategies. Prevention may be key in mitigating rising healthcare cost.

Lower extremity functional electrical stimulation cycling promotes physical and functional recovery in chronic spinal cord injury.

OBJECTIVE: To examine the effect of long-term lower extremity functional electrical stimulation (FES) cycling on the physical integrity and functional recovery in people with chronic spinal cord injury (SCI). DESIGN: Retrospective cohort, mean follow-up 29.1 months, and cross-sectional evaluation. SETTING: Washington University Spinal Cord Injury Neurorehabilitation Center, referral center. PARTICIPANTS: Twenty-five people with chronic SCI who received FES during cycling were matched by age, gender, injury level, and severity, and duration of injury to 20 people with SCI who received range of motion and stretching. INTERVENTION: Lower extremity FES during cycling as part of an activity-based restorative treatment regimen. MAIN OUTCOME MEASURE: Change in neurological function: motor, sensory, and combined motor-sensory scores (CMSS) assessed by the American Spinal Injury Association Impairment scale. Response was defined as ≥ 1 point improvement. RESULTS: FES was associated with an 80% CMSS responder rate compared to 40% in controls. An average 9.6 CMSS point loss among controls was offset by an average 20-point gain among FES subjects. Quadriceps muscle mass was on average 36% higher and intra/inter-muscular fat 44% lower, in the FES group. Hamstring and quadriceps muscle strength was 30 and 35% greater, respectively, in the FES group. Quality of life and daily function measures were significantly higher in FES group. CONCLUSION: FES during cycling in chronic SCI may provide substantial physical integrity benefits, including enhanced neurological and functional performance, increased muscle size and force-generation potential, reduced spasticity, and improved quality of life.

Extensive neurological recovery from a complete spinal cord injury: a case report and hypothesis on the role of cortical plasticity.

Neurological recovery in patients with severe spinal cord injury (SCI) is extremely rare. We have identified a patient with chronic cervical traumatic SCI, who suffered a complete loss of motor and sensory function below the injury for 6 weeks after the injury, but experienced a progressive neurological recovery that continued for 17 years. The extent of the patient's recovery from the severe trauma-induced paralysis is rare and remarkable. A detailed study of this patient using diffusion tensor imaging (DTI), magnetization transfer imaging (MTI), and resting state fMRI (rs-fMRI) revealed structural and functional changes in the central nervous system that may be associated with the neurological recovery. Sixty-two percent cervical cord white matter atrophy was observed. DTI-derived quantities, more sensitive to axons, demonstrated focal changes, while MTI-derived quantity, more sensitive to myelin, showed a diffuse change. No significant cortical structural changes were observed, while rs-fMRI revealed increased brain functional connectivity between sensorimotor and visual networks. The study provides comprehensive description of the structural and functional changes in the patient using advanced MR imaging technique. This multimodal MR imaging study also shows the potential of rs-fMRI to measure the extent of cortical plasticity.

The changing field of rehabilitation: optimizing spontaneous regeneration and functional recovery.

For neurorehabilitation of patients with spinal cord injury (SCI), the traditional emphasis on social adaptation is being expanded to include strategies that promote plasticity and regeneration in the central nervous system. Such strategies are needed to optimize recovery of neurological function. For example, the known dependence of most cellular processes on physical activity has led to the novel concept that activity is important in neural repair. This hypothesis has given rise to activity-based restoration therapies (ABRT), which aim to optimize neural activity in the damaged spinal cord, particularly below the injury level. Here, we review the basic science and clinical evidence supporting the lifelong use of ABRT for recovery from spinal cord injury. We define and describe ABRT, and discuss its components, its clinical applications, its relationship to medical management of spinal cord injury, and the potential influences of medications on recovery. We also discuss the health benefits of ABRT under physiological and pathological conditions. We stress that lifelong ABRT is required to optimize return of function and to allow patients to benefit from any "cures" that will be discovered.