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Verduzco-Gutierrez M, Raghavan P, Pruente J, Moon D, List CM, Hornyak JE, Gul F, Deshpande S, Biffl S, Al Lawati Z, Alfaro A. AAPM&R consensus guidance on spasticity assessment and management. PM R 2024; 16:864-887. [PMID: 38770827 DOI: 10.1002/pmrj.13211] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2023] [Revised: 03/19/2024] [Accepted: 04/08/2024] [Indexed: 05/22/2024]
Abstract
BACKGROUND The American Academy of Physical Medicine and Rehabilitation (AAPM&R) conducted a comprehensive review in 2021 to identify opportunities for enhancing the care of adult and pediatric patients with spasticity. A technical expert panel (TEP) was convened to develop consensus-based practice recommendations aimed at addressing gaps in spasticity care. OBJECTIVE To develop consensus-based practice recommendations to identify and address gaps in spasticity care. METHODS The Spasticity TEP engaged in a 16-month virtual meeting process, focusing on formulating search terms, refining research questions, and conducting a structured evidence review. Evidence quality was assessed by the AAPM&R Evidence, Quality and Performance Committee (EQPC), and a modified Delphi process was employed to achieve consensus on recommendation statements and evidence grading. The Strength of Recommendation Taxonomy (SORT) guided the rating of individual studies and the strength of recommendations. RESULTS The TEP approved five recommendations for spasticity management and five best practices for assessment and management, with one recommendation unable to be graded due to evidence limitations. Best practices were defined as widely accepted components of care, while recommendations required structured evidence reviews and grading. The consensus guidance statement represents current best practices and evidence-based treatment options, intended for use by PM&R physicians caring for patients with spasticity. CONCLUSION This consensus guidance provides clinicians with practical recommendations for spasticity assessment and management based on the best available evidence and expert opinion. Clinical judgment should be exercised, and recommendations tailored to individual patient needs, preferences, and risk profiles. The accompanying table summarizes the best practice recommendations for spasticity assessment and management, reflecting principles with little controversy in care delivery.
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Affiliation(s)
- Monica Verduzco-Gutierrez
- Department of Rehabilitation Medicine, University of Texas Health Science Center at San Antonio, San Antonio, Texas, USA
| | - Preeti Raghavan
- Department of Physical Medicine and Rehabilitation and Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Jessica Pruente
- Department of Physical Medicine & Rehabilitation, University of Michigan, Ann Arbor, Michigan, USA
| | - Daniel Moon
- Department of Physical Medicine and Rehabilitation, Jefferson Moss-Magee Rehabilitation Hospital, Elkins Park, Pennsylvania, USA
| | | | - Joseph Edward Hornyak
- Department of Physical Medicine & Rehabilitation, University of Michigan, Ann Arbor, Michigan, USA
| | - Fatma Gul
- Department of Physical Medicine and Rehabilitation Department, University of Texas, Southwestern Medical Center, Dallas, Texas, USA
| | - Supreet Deshpande
- Department of Pediatric Rehabilitation Medicine, Gillette Children's Hospital, St.Paul, Minnesota, USA
- Department of Rehabilitation Medicine, University of Minnesota, Minneapolis, Minnesota, USA
| | - Susan Biffl
- Division Pediatric Rehabilitation Medicine Department of Orthopedic Surgery, UCSD Rady Children's Hospital, San Diego, California, USA
| | - Zainab Al Lawati
- Department of Rehabilitation Medicine, University of Minnesota, Minneapolis, Minnesota, USA
| | - Abraham Alfaro
- Rehabilitation Medicine, AtlantiCare Health Services, Inc., Federally Qualified Health Center (FQHC), Atlantic City, New Jersey, USA
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Skoutelis VC, Dimitriadis Z, Kanellopoulos A, Dinopoulos A, Papagelopoulos PJ, Kanellopoulos V, Kontogeorgakos VA. Changes in Lower Extremity Passive Range of Motion and Muscle Strength After Selective Percutaneous Myofascial Lengthening and Functional Physiotherapy in Children With Cerebral Palsy. Cureus 2024; 16:e67325. [PMID: 39301341 PMCID: PMC11412655 DOI: 10.7759/cureus.67325] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/20/2024] [Indexed: 09/22/2024] Open
Abstract
Background Children with cerebral palsy (CP) often experience motor and postural disorders, along with spasticity, muscle weakness, muscle-tendon contractures, and decreased joint range of motion (ROM). Muscle-tendon contractures are typically addressed through orthopaedic surgery to improve joint ROM, which can result in further muscle weakness. This study aimed to investigate the impact of selective percutaneous myofascial lengthening (SPML) combined with functional physiotherapy on joint passive ROM and isometric muscle strength in the lower extremities of children with spastic CP. Methods A single-group pre- and post-test design was utilised in this study. Twenty-six children aged five to seven years with spastic CP and Gross Motor Function Classification System levels II-IV underwent the SPML procedure and received nine months of postoperative functional strength training physiotherapy. Joint passive ROM and isometric muscle strength were measured using a universal goniometer and a digital hand-held dynamometer, respectively. Paired-sample t-tests were conducted to compare baseline and follow-up measurements. Results Significant improvements (p < 0.05) were observed in passive ROM of hip abduction, straight leg raise, popliteal angle, and ankle dorsiflexion, as well as in isometric strength of hip flexors, extensors, abductors and adductors, knee extensors, and ankle dorsiflexors. Conclusions The SPML procedure supported by postoperative functional physiotherapy can effectively address fixed contractures by significantly increasing passive joint ROM and muscle strength. Further research with longer-term follow-up measurements is necessary to confirm and expand upon these findings.
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Affiliation(s)
- Vasileios C Skoutelis
- Department of Physiotherapy, ATTIKON University General Hospital, Chaidari, GRC
- Department of Physiotherapy, University of West Attica, Egaleo, GRC
- School of Medicine, National and Kapodistrian University of Athens, Athens, GRC
| | | | | | - Argirios Dinopoulos
- School of Medicine, National and Kapodistrian University of Athens, Athens, GRC
- Third Department of Paediatrics, ATTIKON University General Hospital, Chaidari, GRC
| | - Panayiotis J Papagelopoulos
- School of Medicine, National and Kapodistrian University of Athens, Athens, GRC
- First Department of Orthopaedic Surgery, ATTIKON University General Hospital, Chaidari, GRC
| | | | - Vasileios A Kontogeorgakos
- School of Medicine, National and Kapodistrian University of Athens, Athens, GRC
- First Department of Orthopaedic Surgery, ATTIKON University General Hospital, Chaidari, GRC
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Song AX, Saad A, Hutnik L, Chandra O, McGrath A, Chu A. A PRISMA-IPD systematic review and meta-analysis: does age and follow-up improve active range of motion of the wrist and forearm following pediatric upper extremity cerebral palsy surgery? Front Surg 2024; 11:1150797. [PMID: 38444901 PMCID: PMC10913191 DOI: 10.3389/fsurg.2024.1150797] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2023] [Accepted: 01/30/2024] [Indexed: 03/07/2024] Open
Abstract
Purpose Surgical treatments such as tendon transfers and muscle lengthening play a significant role in cerebral palsy management,but timing of upper extremity cerebral palsy surgery remains controversial. This study systematically reviews the current literature and investigates the correlation between age at surgery and follow-up time with surgical outcomes in pediatric upper extremity cerebral palsy patients. Methods A comprehensive search of PubMed, Cochrane, Web of Science, and CINAHL databases was performed from inception to July 2020 and articles were screened using PRISMA guidelines to include full-text, English papers. Data analysis was performed using itemized data points for age at surgery, follow-up length, and surgery outcomes, reported as changes in active forearm and wrist motion. A 3D linear model was performed, to analyze the relationship between age, follow-up length, and surgery outcomes. Results A total of 3,855 papers were identified using the search terms and a total of 8 studies with itemized patient data (n=126) were included in the study. The studies overall possessed moderate bias according to the ROBINS-I scale. Regression analysis showed that age is a significant predictor of change (|t| > 2) in active forearm supination (Estimate = -2.3465, Std. Error = 1.0938, t-value= -2.145) and wrist flexion (Estimate = -2.8474, Std. Error = 1.0771, t-value = -2.643) post-intervention, with older individuals showing lesser improvements. The duration of follow-up is a significant predictor of improvement in forearm supination (Estimate = 0.3664, Std. Error = 0.1797, t-value = 2.039) and wrist extension (Estimate = 0.7747, Std. Error = 0.2750, t-value = 2.817). In contrast, forearm pronation (Estimate = -0.23756, Std. Error = 0.09648, t-value = -2.462) and wrist flexion (Estimate = -0.4243, Std. Error=0.1859, t-value = -2.282) have a significant negative association with follow-up time. Conclusion These results suggest that there is significant correlation between the age and follow up after surgery with range of motion gains. Most notably, increased age at surgery had a significant negative correlation with select active range of motion postoperative outcomes. Future research should focus on identifying other factors that could affect results of surgical treatment in upper extremity.
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Affiliation(s)
- Amy X. Song
- Department of Orthopedic Surgery, Rutgers New Jersey Medical School, Newark, NJ, United States
| | - Anthony Saad
- Department of Orthopedic Surgery, Rutgers New Jersey Medical School, Newark, NJ, United States
| | - Lauren Hutnik
- Department of Orthopedic Surgery, Rutgers New Jersey Medical School, Newark, NJ, United States
| | - Onrina Chandra
- Department of Statistics, Rutgers University, New Brunswick, NJ, United States
| | - Aleksandra McGrath
- Department of Clinical Science, Faculty of Medicine, Umeå University, Umeå, Sweden
- Department of Surgical and Perioperative Sciences, Faculty of Medicine, Umeå University, Umeå, Sweden
| | - Alice Chu
- Department of Orthopedic Surgery, Rutgers New Jersey Medical School, Newark, NJ, United States
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Fong MM, Gibson N, Williams SA, Jensen L. Clinical functional outcome measures for children with cerebral palsy after gait corrective orthopaedic surgery: A scoping review. Dev Med Child Neurol 2023; 65:1573-1586. [PMID: 37147852 DOI: 10.1111/dmcn.15622] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/29/2022] [Revised: 03/20/2023] [Accepted: 03/22/2023] [Indexed: 05/07/2023]
Abstract
AIM To identify the most frequently reported non-instrumented measures of gait, activity, and participation in children with cerebral palsy (CP) after undergoing gait corrective orthopaedic surgery. METHOD Four databases were searched from database inception to the 9th December 2021 for studies that evaluated functional outcomes for children with CP under 18 years undergoing gait corrective orthopaedic surgery. RESULTS Of 547 citations, 44 publications (n = 3535 participants, n = 1789 males, mean age 10 years 5 months [SD = 3 years 3 months], Gross Motor Function Classification System levels I-III at the time of surgery) were eligible for inclusion. Fourteen different outcome measures were used: one measure of gait, 10 measures of activity, and three measures of participation. Gait was measured with the Edinburgh Visual Gait Scale (EVGS; 4 out of 44). The most common activity and participation measures were the Functional Mobility Scale (FMS; 15 out of 44) and Pediatric Outcomes Data Collection Instrument (11 out of 44) respectively. No studies reported a combination of gait, activity, and participation measures. INTERPRETATION The EVGS and FMS should be considered as core outcome measures in gait corrective orthopaedic surgery, while a measure of participation is unclear. Additional considerations for developing a comprehensive suite of outcomes include identifying a combination of clinical measures and performance-reflective questionnaires that are standardized for children with CP undergoing surgery and meaningful to clinicians and families.
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Affiliation(s)
- Maxine M Fong
- Curtin School of Allied Health, Curtin University, Perth, Western Australia, Australia
- Department of Physiotherapy, Perth Children's Hospital, Nedlands, Western Australia, Australia
| | - Noula Gibson
- Curtin School of Allied Health, Curtin University, Perth, Western Australia, Australia
- Department of Physiotherapy, Perth Children's Hospital, Nedlands, Western Australia, Australia
- Kids Rehab WA, Perth Children's Hospital, Nedlands, Western Australia, Australia
| | - Sian A Williams
- Curtin School of Allied Health, Curtin University, Perth, Western Australia, Australia
- Liggins Institute, University of Auckland, Auckland, New Zealand
| | - Lynn Jensen
- Department of Physiotherapy, Perth Children's Hospital, Nedlands, Western Australia, Australia
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Factors Associated With Short-Term Recovery Following Single-Event Multilevel Surgery for Children With Cerebral Palsy. Pediatr Phys Ther 2023; 35:93-99. [PMID: 36638036 DOI: 10.1097/pep.0000000000000976] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/14/2023]
Abstract
PURPOSE To examine the role of multiple factors, including therapy dose, on recovery of mobility function during post-single-event multilevel surgery (SEMLS) rehabilitation in youth with cerebral palsy. METHODS Several factors expected to influence postoperative change in Gross Motor Function Measure (GMFM) were examined: age, Gross Motor Function Classification System (GMFCS) level, cognition, number of osteotomies, surgical complications, medical comorbidities, number of therapy sessions, and preoperative measures of gait, balance, and gross motor function. RESULTS Sixty-nine youth with cerebral palsy, GMFSC levels I-IV, who had undergone SEMLS and rehabilitation had on average 2.6 osteotomies and 89 postoperative therapy sessions. Fewer osteotomies, higher therapy dose, higher preoperative GMFM, and lower GMFM at postoperative admission were significant in determining GMFM change. CONCLUSIONS The most relevant factors on post-SEMLS recovery were therapy dose, surgical burden, and level of gross motor function immediately before and after surgery.
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MacWilliams BA, Prasad S, Shuckra AL, Schwartz MH. Causal factors affecting gross motor function in children diagnosed with cerebral palsy. PLoS One 2022; 17:e0270121. [PMID: 35849563 PMCID: PMC9292109 DOI: 10.1371/journal.pone.0270121] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2021] [Accepted: 06/05/2022] [Indexed: 11/18/2022] Open
Abstract
Background Cerebral palsy (CP) is a complex neuromuscular condition that may negatively influence gross motor function. Children diagnosed with CP often exhibit spasticity, weakness, reduced motor control, contracture, and bony malalignment. Despite many previous association studies, the causal impact of these impairments on motor function is unknown. Aim In this study, we proposed a causal model which estimated the effects of common impairments on motor function in children with spastic CP as measured by the 66-item Gross Motor Function Measure (GMFM-66). We estimated both direct and total effect sizes of all included variables using linear regression based on covariate adjustment sets implied by the minimally sufficient adjustment sets. In addition, we estimated bivariate effect sizes of all measures for comparison. Method We retrospectively evaluated 300 consecutive subjects with spastic cerebral palsy who underwent routine clinical gait analysis. Model data included standard information collected during this analysis. Results The largest causal effect sizes, as measured by standardized regression coefficients, were found for selective voluntary motor control and dynamic motor control, followed by strength, then gait deviations. In contrast, common treatment targets, such as spasticity and orthopedic deformity, had relatively small effects. Effect sizes estimated from bivariate models, which cannot appropriately adjust for other causal factors, substantially overestimated the total effect of spasticity, strength, and orthopedic deformity. Interpretation Understanding the effects of impairments on gross motor function will allow clinicians to direct treatments at those impairments with the greatest potential to influence gross motor function and provide realistic expectations of the anticipated changes.
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Affiliation(s)
- Bruce A. MacWilliams
- Shriners Hospitals for Children, Salt Lake City, Utah, United States of America
- Department of Orthopedic Surgery, University of Utah, Salt Lake City, Utah, United States of America
- * E-mail:
| | - Sarada Prasad
- Shriners Hospitals for Children, Salt Lake City, Utah, United States of America
| | - Amy L. Shuckra
- Shriners Hospitals for Children, Salt Lake City, Utah, United States of America
| | - Michael H. Schwartz
- James R. Gage Center for Gait and Motion Analysis, Gillette Children’s Specialty Healthcare, St. Paul, Minnesota, United States of America
- Department of Orthopedic Surgery, University of Minnesota, Minneapolis, Minnesota, United States of America
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Guinet AL, Khouri N, Desailly E. Rehabilitation After Single-Event Multilevel Surgery for Children and Young Adults With Cerebral Palsy: A Systematic Review. Am J Phys Med Rehabil 2022; 101:389-399. [PMID: 34393188 DOI: 10.1097/phm.0000000000001864] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
ABSTRACT This review sought to describe and analyze published protocols for rehabilitation after single-event multilevel surgery for people with cerebral palsy, to identify their differences and limits, and to introduce a common step-by-step framework for future descriptions and assessments of postoperative rehabilitation protocols.The MEDLINE, Embase, CINAHL, and the Cochrane Library databases were searched. Inclusion criteria were as follows: (1) single-event multilevel surgery, (2) full-text reports published after 1985, and (3) articles with a method section describing the rehabilitation protocol. Interventions were coded using the Oxford Levels of Evidence and the Methodological Index for Non-Randomized Studies Index.Twenty-four articles were included in the review. Studies included patients aged 4-30 yrs with spastic cerebral palsy (hemiplegia, diplegia, and quadriplegia). The mean postoperative rehabilitation duration was 4.5 mos, with 4 sessions per week, and rehabilitation took place in a rehabilitation center. This review provides relevant information about the modalities, contents, limits, and difficulties associated with the post-SEMS rehabilitation protocol reported in the literature. Pain was identified as a major problem.A more precise and comprehensive description of post-SEMS rehabilitation protocols would be useful. The proposed five-step framework could be used by future studies to standardize their protocol description in terms of objective, content, and intensity.
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Affiliation(s)
- Anne-Laure Guinet
- From the Fondation Ellen Poidatz, Pôle Recherche & Innovation, Saint-Fargeau-Ponthierry, France (ALG, NK, ED); University Paris-Saclay, Univ. Evry, IBISC, Evry (ALG); and Orthopaedic Department, Necker-Enfants Malades Hospital, Paris, France (NK)
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The Ability to Run in Young People with Cerebral Palsy before and after Single Event Multi-Level Surgery. J Pers Med 2021; 11:jpm11070660. [PMID: 34357127 PMCID: PMC8303998 DOI: 10.3390/jpm11070660] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2021] [Revised: 07/05/2021] [Accepted: 07/07/2021] [Indexed: 11/16/2022] Open
Abstract
The objective of the study is to identify and evaluate possible factors that influence the ability to run before and after single event multi-level surgery (SEMLS). Young patients (6–25 years) with spastic cerebral palsy (GMFCSI-II) were retrospectively included. Type and number of surgical procedures, time for recovery and 3D gait analysis variables were analyzed with respect to the ability to run. In total, 98 patients (38 females; 60 males) who received SEMLS (12 years, SD 3.4) were included and compared to a control group of 71 conservatively treated patients. Of 60 runners pre-surgery, 17 (28%) lost the ability, while gained in 8 of 38 (21%) non-runners. The number of surgical procedures was a significant predictor and those who lost their ability to run had significantly more (mean = 5.9, SD = 1.7), compared to the patients who gained the ability (mean = 3.5, SD = 0.9). Further, pre-surgical function (e.g., gait speed) was significantly different (p < 0.001). Pre-surgical function and the number of surgical procedures seem to play an important role for the gain or loss of the ability to run after surgery. Caution is warranted in patients with lower pre-surgical function and the ability to run, as they seem at a higher risk to lose the ability.
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Jung Y, Chung EJ, Chun HL, Lee BH. Effects of whole-body vibration combined with action observation on gross motor function, balance, and gait in children with spastic cerebral palsy: a preliminary study. J Exerc Rehabil 2020; 16:249-257. [PMID: 32724782 PMCID: PMC7365726 DOI: 10.12965/jer.2040136.068] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Accepted: 04/30/2020] [Indexed: 11/22/2022] Open
Abstract
This study evaluated the effects of whole-body vibration (WBV) com-bined with action observation on gross motor function, balance, and gait in children with spastic cerebral palsy. The participants were randomized into the WBV combined with action observation (WBVAO) group (n=7) and the WBV group (n=7). The WBVAO group received WBV combined with action observation training, and the WBV group received WBV training for 4 weeks. Both groups received 30 min of training a day, 3 times a week. All participants completed the 5 times sit to stand (FTSTS) test, Timed Up and Go (TUG) test, pediatric reach test, 10-m walk test, 6-min walk test (6MWT), and timed up and down stair (TUDS) test before and after the training intervention. Moreover, the participant’s Gross Motor Function Measure (GMFM) and Pediatric Balance Scale (PBS) scores were assessed. Both the WBVAO and WBV groups demonstrated significant increases in the scores of FTSTS test, GMFM (D and E dimensions), PBS, TUG test, 6MWT, and TUDS test. The WBVAO group improved more significantly compared to the WBV group based on the scores of the FTSTS test, GMFM (D dimension), PBS, 6MWT, and TUDS test. WBV combined with action observation improved lower extremity functional strength, gross motor function, and balance and gait in children with cerebral palsy. These results suggest that WBV combined with action observation is both feasible and suitable for individuals with cerebral palsy.
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Affiliation(s)
- Youngmin Jung
- Graduate School of Physical Therapy, Sahmyook University, Seoul, Korea
| | - Eun-Jung Chung
- Department of Physical Therapy, Andong Science College, Andong, Korea
| | - Hye-Lim Chun
- Graduate School of Physical Therapy, Sahmyook University, Seoul, Korea
| | - Byoung-Hee Lee
- Department of Physical Therapy, Sahmyook University, Seoul, Korea
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Papageorgiou E, Simon-Martinez C, Molenaers G, Ortibus E, Van Campenhout A, Desloovere K. Are spasticity, weakness, selectivity, and passive range of motion related to gait deviations in children with spastic cerebral palsy? A statistical parametric mapping study. PLoS One 2019; 14:e0223363. [PMID: 31603897 PMCID: PMC6788679 DOI: 10.1371/journal.pone.0223363] [Citation(s) in RCA: 35] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2019] [Accepted: 09/19/2019] [Indexed: 01/17/2023] Open
Abstract
This study aimed to identify the relationships between clinical impairments and gait deviations in children with cerebral palsy (CP). A retrospective convenience sample of 367 children with CP was selected (3-18 years old) and divided in two groups based on clinical symptomatology [unilateral (uCP) / bilateral CP (bCP), (n = 167/200)]. All children underwent a three-dimensional gait analysis and a standardized clinical examination. Gait was inspected on a vector level (all sagittal motions combined), and an individual joint level (pelvis, hip, knee and ankle joint motions). Statistical non-parametric mapping was applied to identify specific parts of the gait cycle displaying relationships between the gait deviations of both groups and the impairment scores of spasticity, weakness, selectivity, and passive range of motion. Impairment scores were summarized in two ways: a) composite impairment scores (e.g. combined spasticity of all assessed muscles acting around the hip, knee and ankle joints) and b) joint specific impairment scores (e.g. spasticity of the muscles acting around the knee joint). Results showed that the vector and most of the individual motions were related to the composite scores. Direct and carry-over relationships were found between certain individual motions and joint impairment scores (around the same or neighboring joints, respectively). All correlations were more prominent for children with bCP compared to uCP, especially regarding the relationships of gait deviations with weakness and reduced selectivity. In conclusion, this study enabled the mapping of relationships between clinical impairments and gait deviations in children with CP, by identifying specific parts of the gait cycle that are related to each of these impairments. These results provide a comprehensive description of these relationships, while simultaneously highlighting the differences between the two CP groups. Integration of these findings could lead to a better understanding of the pathophysiology of gait deviations and, eventually, support individualized treatment planning.
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Affiliation(s)
- Eirini Papageorgiou
- KU Leuven Department of Rehabilitation Sciences, Leuven, Belgium
- Clinical Motion Analysis Laboratory, University Hospitals Leuven, Leuven, Belgium
| | | | - Guy Molenaers
- KU Leuven Department of Development and Regeneration, Leuven, Belgium
- Department of Orthopedics, University Hospitals Leuven, Leuven, Belgium
| | - Els Ortibus
- KU Leuven Department of Development and Regeneration, Leuven, Belgium
| | - Anja Van Campenhout
- KU Leuven Department of Development and Regeneration, Leuven, Belgium
- Department of Orthopedics, University Hospitals Leuven, Leuven, Belgium
| | - Kaat Desloovere
- KU Leuven Department of Rehabilitation Sciences, Leuven, Belgium
- Clinical Motion Analysis Laboratory, University Hospitals Leuven, Leuven, Belgium
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Xiao XW, Du J, Jiao B, Liao XX, Zhou L, Liu XX, Yuan ZH, Guo LN, Wang X, Shen L, Lin ZY. Novel ATL1 mutation in a Chinese family with hereditary spastic paraplegia: A case report and review of literature. World J Clin Cases 2019; 7:1358-1366. [PMID: 31236401 PMCID: PMC6580333 DOI: 10.12998/wjcc.v7.i11.1358] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/30/2019] [Revised: 03/23/2019] [Accepted: 04/09/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Hereditary spastic paraplegias (HSPs) refer to a group of heterogeneous neurodegenerative diseases characterized by lower limbs spasticity and weakness. So far, over 72 genes have been found to cause HSP (SPG1-SPG72). Among autosomal dominant HSP patients, spastic paraplegia 4 (SPG4/SPAST) gene is the most common pathogenic gene, and atlastin-1 (ATL1) is the second most common one. Here we reported a novel ATL1 mutation in a Chinese spastic paraplegia 3A (SPG3A) family, which expands the clinical and genetic spectrum of ATL1 mutations.
CASE SUMMARY A 9-year-old boy with progressive spastic paraplegia accompanied by right hearing loss and mental retardation for five years was admitted to our hospital. Past history was unremarkable. The family history was positive, and his grandfather and mother had similar symptoms. Neurological examinations revealed hypermyotonia in his lower limbs, hyperreflexia in knee reflex, bilateral positive Babinski signs and scissors gait. The results of blood routine test, liver function test, blood glucose test, ceruloplasmin test and vitamin test were all normal. The serum lactic acid level was significantly increased. The testing for brainstem auditory evoked potential demonstrated that the right side hearing was impaired while the left was normal. Magnetic resonance imaging showed mild atrophy of the spinal cord. The gene panel test revealed that the proband carried an ATL1 c.752A>G p.Gln251Arg (p.Q251R) mutation, and Sanger sequencing confirmed the existence of family co-segregation.
CONCLUSION We reported a novel ATL1 Q251R mutation and a novel clinical phenotype of hearing loss in a Chinese SPG3A family.
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Affiliation(s)
- Xue-Wen Xiao
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
| | - Juan Du
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
- National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
| | - Bin Jiao
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
- National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
- Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, Changsha 410008, Hunan Province, China
| | - Xin-Xin Liao
- Department of Geriatrics Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
| | - Lu Zhou
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
| | - Xi-Xi Liu
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
| | - Zhen-Hua Yuan
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
| | - Li-Na Guo
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
| | - Xin Wang
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
| | - Lu Shen
- Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
- National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
- Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, Changsha 410008, Hunan Province, China
- Key Laboratory of Organ Injury, Aging and Regenerative Medicine of Hunan Province, Changsha 410008, Hunan Province, China
| | - Zhang-Yuan Lin
- National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
- Department of Orthopedics, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
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Amirmudin NA, Lavelle G, Theologis T, Thompson N, Ryan JM. Multilevel Surgery for Children With Cerebral Palsy: A Meta-analysis. Pediatrics 2019; 143:peds.2018-3390. [PMID: 30918016 DOI: 10.1542/peds.2018-3390] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 01/10/2019] [Indexed: 11/24/2022] Open
Abstract
CONTEXT Multilevel surgery (MLS) is standard care for reducing musculoskeletal disorders among children with spastic cerebral palsy (CP). OBJECTIVE To summarize the literature examining effects of MLS and satisfaction with MLS for children with CP. DATA SOURCES Medline, Embase, Cumulative Index to Nursing and Allied Health Literature, and Cochrane Central Register of Controlled Trials were searched. STUDY SELECTION Studies in which authors reported effects of or satisfaction with MLS in children with CP were selected. DATA EXTRACTION Two authors screened and extracted data on gross motor function, gait speed, gait (eg, Gait Profile Score), range of motion, strength, spasticity, participation, quality of life, satisfaction, and adverse events. RESULTS Seventy-four studies (3551 participants) were identified. One was a randomized controlled trial (RCT) (n = 19); the remainder were cohort studies. Pooled analysis of cohort studies revealed that MLS did not have a long-term effect on gross motor function (standardized mean difference [SMD]: 0.38; 95% confidence interval [CI]: -0.25 to 1.01) or gait speed (SMD: 0.12; 95% CI: -0.01 to 0.25) but did improve gait (SMD: -0.80; 95% CI: -0.95 to -0.65). The RCT also revealed no effect of MLS on gross motor function but improvements in the Gait Profile Score at 1 year. Participation and quality of life were reported in only 5 studies, and adverse events were adequately reported in 17 studies. LIMITATIONS Data were largely from cohort studies. CONCLUSIONS Findings reveal that gait, but not gross motor function, improves after MLS. RCTs and improved reporting of studies of MLS are required.
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Affiliation(s)
- Noor Amirah Amirmudin
- Department of Epidemiology and Public Health Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland
| | - Grace Lavelle
- Department of Clinical Sciences, Brunel University London, London, United Kingdom; and
| | - Tim Theologis
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
| | - Nicky Thompson
- Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
| | - Jennifer M Ryan
- Department of Epidemiology and Public Health Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland; .,Department of Clinical Sciences, Brunel University London, London, United Kingdom; and
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Walton EL. Can cannibalizing cancer cells challenge classic cell death classification? Biomed J 2017; 40:129-132. [PMID: 28651733 PMCID: PMC6136293 DOI: 10.1016/j.bj.2017.06.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2017] [Accepted: 06/02/2017] [Indexed: 11/24/2022] Open
Abstract
In this issue of the Biomedical Journal, we learn about a novel are still largely mysterious mechanism of cell death that is challenging classification systems of cell death pathways and could have important implications for future cancer therapy. We also learn of a promising biomarker to stratify patients into risk groups after stroke. Finally, this issue also includes two studies investigating factors that influence outcome after heart surgery.
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Affiliation(s)
- Emma Louise Walton
- Staff Writer at the Biomedical Journal, 56 Dronningens Gate, 7012 Trondheim, Norway.
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