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BACKGROUND: To estimate the prevalence of sensory symptoms in people with cerebral palsy (CP) across the lifespan. METHODS: In this cross-sectional study, the self-reported Sensory Processing Scale Inventory (SPS-I) was administered via Research Electronic Data Capture (REDCap) between February 1, 2022, and August 15, 2022, to people with CP or their caregivers enrolled in the online MyCP Community Registry. We determined the association between SPS-I scores and age (Pearson correlation) and functional status as assessed using five validated functional classification systems for CP (analysis of variance [ANOVA]). We hypothesized that sensory symptoms would differ between younger and older individuals with CP. RESULTS: Of 155 responses (28% response rate, age one to 76 years, 34% male), 97% reported at least one bothersome sensory symptom. Total sensory symptoms decreased with age (R2 = 0.12, P < 0.0001), driven by decreases in hyposensitivity symptoms (R2 = 0.32, P < 0.0001), primarily tactile hyposensitivity (R2 = 0.29, P < 0.0001). Sensory symptoms increased with greater functional impairment across all functional domains (ANOVA, P < 0.0001). However, the age-specific decrease in hyposensitivities was most pronounced in people with the greatest gross motor functional impairment (R2 = 0.70, P = 0.0004). CONCLUSION: Our findings suggest that hyposensitivity, primarily tactile sensitivity, decreases with age in people with CP. Future work should assess whether decreased hyposensitivity contributes to other age-related changes in CP like increased pain.
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Parálisis Cerebral , Humanos , Parálisis Cerebral/fisiopatología , Parálisis Cerebral/complicaciones , Masculino , Femenino , Estudios Transversales , Adulto , Persona de Mediana Edad , Niño , Preescolar , Adulto Joven , Adolescente , Anciano , Lactante , Trastornos de la Sensación/etiología , Trastornos de la Sensación/fisiopatología , PrevalenciaAsunto(s)
Parálisis Cerebral , Distonía , Humanos , Parálisis Cerebral/tratamiento farmacológico , Parálisis Cerebral/complicaciones , Distonía/tratamiento farmacológico , Distonía/etiología , Niño , Toxinas Botulínicas Tipo A/uso terapéutico , Toxinas Botulínicas Tipo A/administración & dosificaciónRESUMEN
OBJECTIVE: Selective dorsal rhizotomy (SDR) is a neurosurgical procedure to reduce spasticity in children with cerebral palsy and spastic diplegia. The authors developed a procedure called focal SDR for children with spasticity predominantly in the L5 or S1 motor distribution, which can be combined with orthopedic correction of fixed soft-tissue or bony deformity. The authors describe in detail the technique of minimally invasive focal SDR and propose selection criteria. METHODS: The authors conducted a retrospective study of patients who underwent focal SDR at their institution and underwent baseline and 1-year postoperative 3D gait analysis. Modified Ashworth scale (MAS) and Gait Deviation Index (GDI) scores were the primary outcome measures. RESULTS: Ten patients met the study criteria, all with an underlying diagnosis of cerebral palsy. All underwent focal SDR at the unilateral or bilateral S1 level, and 4 additionally underwent focal SDR at the L5 level unilaterally or bilaterally. All but 1 patient underwent concurrent orthopedic surgery. The improvement in spasticity of the plantar flexors, as measured by the MAS score, was 2.2 (p < 0.001). In the patients who underwent L5 focal SDR, there was an improvement in the hamstring MAS score of 1.4 (p = 0.004). The mean improvement in the GDI score following focal SDR was 11 (range -6 to 29, p < 0.001). CONCLUSIONS: Focally impairing spasticity in the gastrocsoleus complex and/or hamstrings muscle group in the setting of less functionally impactful proximal tone is extremely common in cerebral palsy. The novel technique of focal SDR, combined with orthopedic intervention, improves spasticity scores and overall gait mechanics. Further investigation is warranted to define the ideal candidacy and outcomes.
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Parálisis Cerebral , Espasticidad Muscular , Rizotomía , Humanos , Rizotomía/métodos , Parálisis Cerebral/complicaciones , Parálisis Cerebral/cirugía , Masculino , Femenino , Niño , Estudios Retrospectivos , Espasticidad Muscular/cirugía , Espasticidad Muscular/fisiopatología , Preescolar , Resultado del Tratamiento , AdolescenteRESUMEN
The prevalence of cerebral palsy (CP) varies globally, with higher rates and burden of disease in low- and middle-income countries. CP is a lifelong condition with no cure, presenting diverse challenges such as motor impairment, epilepsy, and mental health disorders. Research progress has been made but more is needed, especially given consumer demands for faster advancements and improvements in the scientific evidence base for interventions. This paper explores three strategies to accelerate CP research: consumer engagement, global clinical trial networks, and adaptive designs. Consumer engagement involving individuals with lived experience enhances research outcomes. Global clinical trial networks provide efficiency through larger and more diverse participant pools. Adaptive designs, unlike traditional randomized controlled trials, allow real-time modifications based on interim analyses, potentially answering complex questions more efficiently. The establishment of a CP Global Clinical Trials Network, integrating consumer engagement, global collaboration, and adaptive designs, marks a paradigm shift. The Network aims to address consumer-set research priorities. While challenges like ethical considerations and capacity building exist, the potential benefits for consumers, clinicians, researchers, and funding bodies are substantial. This paper underscores the urgency of transforming CP research methodologies for quicker translation of novel treatments into clinical practice to improve quality of life for those with CP.
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Parálisis Cerebral , Trastornos Mentales , Humanos , Parálisis Cerebral/terapia , Calidad de Vida , Ensayos Clínicos como AsuntoRESUMEN
Objective: To determine how physicians approach pharmacologic dystonia treatment in people with CP and assess physician readiness to participate in a randomized trial comparing existing pharmacologic dystonia treatments. Methods: We administered a REDCap survey to physician members of the American Academy of Cerebral Palsy and Developmental Medicine and of the Child Neurology Society to assess which pharmacologic agents they use to treat dystonia in CP and their preferred indications and dosing. Results: Of 479 physicians surveyed, 240 (50%) responded. Respondents treated functionally limiting (95%) and generalized (57%) dystonia and most commonly used six medications: baclofen (95%), trihexyphenidyl (79%), gabapentin (67%), carbidopa/levodopa (55%), clonazepam (55%), and diazepam (54%). Baclofen was preferred in people with co-existing spasticity (81%), gabapentin was preferred in people with co-existing pain (49%), and trihexyphenidyl was avoided in people with constipation (34%) or urinary retention (42%). Preferred dosing regimens followed published regimens for dystonia, when available, but otherwise followed published regimens for other CP symptoms (spasticity and seizures). Baclofen was preferred by 64% of respondents as first line treatment, but there was no clear consensus on second or third-line medications. Most respondents (51%) were comfortable randomizing their patients to receive any of the six most commonly used medications used to treat dystonia in CP. Conclusions: This study summarizes current indications and dosing for the six most commonly used medications to treat dystonia in CP as per treating physicians in the US and Canada and also demonstrates physician support for a randomized trial comparing the effectiveness of these treatments.
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BACKGROUND: Acute flaccid myelitis (AFM) is a childhood illness characterized by sudden-onset weakness impairing function. The primary goal was to compare the motor recovery patterns of patients with AFM who were discharged home or to inpatient rehabilitation. Secondary analyses focused on recovery of respiratory status, nutritional status, and neurogenic bowel and bladder in both cohorts. METHODS: Eleven tertiary care centers in the United States performed a retrospective chart review of children with AFM between January 1, 2014, and October 1, 2019. Data included demographics, treatments, and outcomes on admission, discharge, and follow-up visits. RESULTS: Medical records of 109 children met inclusion criteria; 67 children required inpatient rehabilitation, whereas 42 children were discharged directly home. The median age was 5 years (range 4 months to 17 years), and the median time observed was 417 days (interquartile range = 645 days). Distal upper extremities recovered better than the proximal upper extremities. At acute presentation, children who needed inpatient rehabilitation had significantly higher rates of respiratory support (P < 0.001), nutritional support (P < 0.001), and neurogenic bowel (P = 0.004) and bladder (P = 0.002). At follow-up, those who attended inpatient rehabilitation continued to have higher rates of respiratory support (28% vs 12%, P = 0.043); however, the nutritional status and bowel/bladder function were no longer statistically different. CONCLUSIONS: All children made improvements in strength. Proximal muscles remained weaker than distal muscles in the upper extremities. Children who qualified for inpatient rehabilitation had ongoing respiratory needs at follow-up; however, recovery of nutritional status and bowel/bladder were similar.
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Enfermedades Virales del Sistema Nervioso Central , Mielitis , Intestino Neurogénico , Enfermedades Neuromusculares , Humanos , Niño , Estados Unidos , Lactante , Estudios Retrospectivos , Intestino Neurogénico/complicaciones , Mielitis/terapia , Resultado del Tratamiento , Enfermedades Virales del Sistema Nervioso Central/complicaciones , Enfermedades Neuromusculares/complicacionesRESUMEN
Dystonia is a highly prevalent movement disorder that can manifest at any time across the lifespan. An increasing number of investigations have tied this disorder to dysfunction of a broad "dystonia network" encompassing the cerebellum, thalamus, basal ganglia, and cortex. However, pinpointing how dysfunction of the various anatomic components of the network produces the wide variety of dystonia presentations across etiologies remains a difficult problem. In this review, a discussion of functional network findings in non-mendelian etiologies of dystonia is undertaken. Initially acquired etiologies of dystonia and how lesion location leads to alterations in network function are explored, first through an examination of cerebral palsy, in which early brain injury may lead to dystonic/dyskinetic forms of the movement disorder. The discussion of acquired etiologies then continues with an evaluation of the literature covering dystonia resulting from focal lesions followed by the isolated focal dystonias, both idiopathic and task dependent. Next, how the dystonia network responds to therapeutic interventions, from the "geste antagoniste" or "sensory trick" to botulinum toxin and deep brain stimulation, is covered with an eye towards finding similarities in network responses with effective treatment. Finally, an examination of how focal network disruptions in mouse models has informed our understanding of the circuits involved in dystonia is provided. Together, this article aims to offer a synthesis of the literature examining dystonia from the perspective of brain networks and it provides grounding for the perspective of dystonia as disorder of network function.
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Objective: To investigate the differences in delivery mode, daily dose, and catheter tip location in pediatric patients using intrathecal baclofen (ITB) pumps with spasticity plus dystonia versus spasticity alone. Methods: A single-center, cross-sectional study was performed by collecting retrospective data from electronic medical records. Demographic and diagnostic information was obtained, comparing patients with spasticity with or without dystonia. The data were analyzed for group differences using a two-tailed Student's t-test. Categorical data were analyzed for group differences using Pearson's χ2 test. Results: A total of 137 patients met the criteria. The majority (114) had spasticity plus dystonia whereas only 23 were documented as spasticity alone. Simple continuous dosing was the most common delivery mode, but flex dosing was used more than twice as frequently with spasticity plus dystonia compared with spasticity alone (42% vs 17%). Patients with spasticity plus dystonia also had more rostral catheter tip locations. Conclusions: While it has been discussed anecdotally, this study confirms the supposition that patients with spasticity plus dystonia have increased dose requirements when compared with those with spasticity alone. Although there are no clear standards of care when managing these patients, they are often on higher daily dosages, are more likely to require flexed dosing method, and have higher catheter placements. Still, there are few studies that demonstrate improvements in dystonia with the use of ITB. In general, these patients would benefit from the development of universal standardizations as well as the confirmation that this is an appropriate treatment.
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OBJECTIVE: In nonambulatory children with predominantly spastic cerebral palsy (CP), the authors compared care needs, symptom burden, and complications after surgical treatment with either intrathecal baclofen (ITB) pump insertion or selective dorsal rhizotomy (SDR). The patients were treated at two Canadian centers with variability in practice pertaining to these surgical options. METHODS: The authors performed a retrospective analysis of nonambulatory children with predominantly spastic quadriplegic or diplegic CP who underwent treatment with ITB or SDR. These two strategies were retrospectively assessed by comparing patient data from the two treatment groups for demographic characteristics, outcomes, and complications. A partial least-squares analysis was performed to identify patient phenotypes associated with outcomes. RESULTS: Thirty patients who underwent ITB and 30 patients who underwent SDR were included for analysis. Patients in the ITB group were older and had lower baseline functional status, with greater burdens of spasticity, dystonia, pain, deformity, bladder dysfunction, and epilepsy than patients in the SDR group. In addition, children who underwent SDR had lower Gross Motor Function Classification System (GMFCS) levels and were less likely to experience complications than those who underwent ITB. However, children treated with SDR had fewer improvements in pain than children treated with ITB. A single significant latent variable explaining 88% of the variance in the data was identified. CONCLUSIONS: Considerable baseline differences exist within this pediatric CP patient population. Factors specific to individual children must be taken into account when determining whether ITB or SDR is the appropriate treatment.
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Pediatric spina bifida (SB) and spinal cord injury (SCI) are unfortunately common in our society, and their unique findings and comorbidities warrant special consideration. This manuscript will discuss the epidemiology, pathophysiology, prevention, and management strategies for children growing and developing with these unique neuromuscular disorders. Growth and development of the maturing child places them at high risk of spinal cord tethering, syringomyelia, ascending paralysis, pressure injuries, and orthopedic abnormalities that must be addressed frequently and judiciously. Similarly, proper neurogenic bladder and neurogenic bowel management is essential not just for medical safety, but also for optimal psychosocial integration into the child's expanding social circle.
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Purpose of Review: This review provides guidance for acute spinal cord injury (SCI) management through an analytical assessment of the most recent evidence on therapies available for treating SCI, including newer therapies under investigation. We present an approach to the SCI patient starting at presentation to acute rehabilitation and prognostication, with additional emphasis on the pediatric population when evidence is available. Recent Findings: Further studies since the Surgical Timing in Acute Spinal Cord Injury Study (STASCIS) demonstrated a potential functional outcome benefit with ultra-early surgical intervention ≤ 8 h post-SCI. Subsequent analysis of the National Acute Spinal Cord Injury Study (NASCIS) II and NASCIS III trials have demonstrated potentially serious complications from intravenous methylprednisolone with limited benefit. Newer therapies actively being studied have demonstrated limited or no benefit in preclinical and clinical trials with insufficient evidence to support use in acute SCI treatment. Summary: Care for SCI patients requires a multi-disciplinary team. Immediate evaluation and management are focused on preventing additional injury and restoring perfusion to the affected cord. Rapid assessment and intervention involve focused neurological examination, targeted imaging, and surgical intervention when indicated. There are currently no evidence-based recommendations for pathomechanistically targeted therapies.
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The COVID-19 pandemic has been a challenge to healthcare systems around the world. Within pediatric rehabilitation medicine, management of intrathecal baclofen has been particularly challenging. This editorial reviews how programs in the US and Canada coped with the quickly changing healthcare environment and how we can learn from this pandemic to be prepared for future crises.
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Baclofeno/administración & dosificación , COVID-19/epidemiología , Espasticidad Muscular/tratamiento farmacológico , Pandemias , Niño , Comorbilidad , Humanos , Inyecciones Espinales , Relajantes Musculares Centrales/administración & dosificación , Espasticidad Muscular/epidemiología , SARS-CoV-2 , Resultado del TratamientoRESUMEN
This article overviews the surgical options for hypertonia management in cerebral palsy, both spasticity and dystonia. We review the history and use of intrathecal baclofen. We contrast its use with the indications for selective dorsal rhizotomy and review how it is the optimal technique to lower tone in the ambulatory spastic diplegic patient with cerebral palsy. This article reviews the advent of deep brain stimulation, with an emphasis on selection criteria and expected outcomes in this population. The article reviews the principles and use of selective peripheral neurotomy as it is applied to focal spasticity not requiring systemic tone reduction.
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Parálisis Cerebral/cirugía , Distonía/cirugía , Espasticidad Muscular/cirugía , Rizotomía , Baclofeno/uso terapéutico , Parálisis Cerebral/tratamiento farmacológico , Parálisis Cerebral/fisiopatología , Estimulación Encefálica Profunda , Distonía/tratamiento farmacológico , Distonía/fisiopatología , Humanos , Inyecciones Espinales , Relajantes Musculares Centrales/uso terapéutico , Espasticidad Muscular/tratamiento farmacológico , Espasticidad Muscular/fisiopatologíaRESUMEN
PURPOSE: To understand the relationship of walking speed to self-reported pain, fatigue, and physical function in adults with CP. METHODS: Design: Cross-sectional study. SETTING: Accredited clinical motion analysis laboratory in a regional children's hospital. PARTICIPANTS: 72 ambulatory patients ⩾ 18 years of age, diagnosed with CP, who previously had ⩾ 1 prior instrumented gait analysis at our facility. MAIN OUTCOME MEASURES: PROMIS-57 pain interference/intensity, physical function, and fatigue measures and walking speed. RESULTS: Physical function was significantly lower than able-bodied normal values by 1-2 standard deviations (40.3 ± 8.5). Pain interference (51.4 ± 9.0) and fatigue (50.2 ± 9.2) were not significantly different when compared to able-bodied normal values. Only physical function was statistically correlated with walking speed (p< 0.001), while pain interference (p= 0.39), pain intensity (p= 0.36), and fatigue (p= 0.75) were not. Pain interference, pain intensity, and fatigue were not statistically significant factors in the multiple regression of walking speed. Fatigue could significantly predict physical function, pain interference, and pain scores (p= 0.032, p< 0.001, p< 0.01, respectively), however, fatigue did not directly predict walking speed (p= 0.747). CONCLUSIONS: Self-reported physical function correlates with objectively measured walking speed in young adults with CP while patient-reported pain and fatigue did not, contrary to what would be predicted by the literature.