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(1) Background: The Modified Ashworth Scale (MAS) is commonly used clinically to evaluate spasticity, but its qualitative nature introduces subjectivity. We propose a novel metric scale to quantitatively measure spasticity using mechanomyography (MMG) to mitigate these subjective effects. (2) Methods: The flexor and extensor muscles of knee and elbow joints were assessed with the Modified Ashworth Scale (MAS) during the acquisition of mechanomyography (MMG) data. The median absolute amplitude of the MMG signals was utilized as a key descriptor. An algorithm was developed to normalize the MMG signals to a universal gravitational (G) acceleration scale, aligning them with the limits and range of MAS. (3) Results: We evaluated 34 lower and upper limbs from 22 volunteers (average age 39.91 ± 13.77 years) of both genders. Polynomial regression provided the best fit (R2 = 0.987), with negligible differences (mean of 0.001 G) between the MAS and MMG. We established three numerical sets for the median, minimum, and maximum MMG(G) values corresponding to each MAS range, ensuring consistent alignment of the Modified Ashworth levels with our proposed scale. (4) Conclusions: Muscle spasticity can now be quantitatively and semi-automatically evaluated using our algorithm and instrumentation, enhancing the objectivity and reliability of spasticity assessments.

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STUDY DESIGN: A psychometric study. OBJECTIVES: To introduce a novel simple tool designed to evaluate the intensity of the phasic (dynamic) component of spastic motor behavior in spinal cord injury (SCI) people and to assess its reliability and validity. SETTING: The study was developed in the Spinal Cord Unit at University Hospital Motol and Paraple Centre in Prague, Czech Republic. METHODS: The Muscle Excitability Scale (MES) is designed to rate muscle motor response to exteroceptive and proprioceptive stimuli. The impairment rating ranges from zero muscle/muscle group spasm or clonus to generalized spastic response. The selected 0 to 4 scale allows for comparing the MES results with those of the Modified Ashworth Scale (MAS). After long-term use and repeated revisions, a psychometric analysis was conducted. According to the algorithm, two physiotherapists examined 50 individuals in the chronic stage after SCI. RESULTS: The inter-rater reliability of MES for both legs showed κ = 0.52. The intra-rater reliability of MES for both legs showed κ = 0.50. The inter-rater reliability of simultaneously assessed MAS for both legs was higher, with κ = 0.69. The intra-rater reliability of MAS for both legs showed κ = 0.72. Spearman's rank correlation coefficient between MES and spasm frequency of Penn Spasm Frequency Scale (PSFS) was low, while the correlation coefficient between MES and the severity part of PSFS was moderate. CONCLUSIONS: The MES is a complementary tool for assessing the dynamic component of spastic motor behavior in SCI people. It allows a more comprehensive clinical characterization of spastic reflexes when used along with the MAS.

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INTRODUCTION: The increase in the number of people with upper limb spasticity as a sequela of cerebrovascular disease, which negatively impacts their autonomy, functional independence and participation, and affects their quality of life, calls for the application of precise and objective instruments for its measurement and evaluation. OBJECTIVE: To assess the validity and reliability of the Tardieu scale in the evaluation of upper extremity spasticity in adults with cerebrovascular disease. MATERIALS AND METHODS: The search strategy was implemented in eight databases; the systematic review protocol was registered beforehand in INPLASY (with registration no. 2023110076). The evidence was synthesised in three phases: a tabular presentation of results, an evaluation of the quality of the articles, and a narrative synthesis of the findings. RESULTS: Only three of the 33 articles identified fulfilled the variables that enable the validity and reliability of the Tardieu scale to be established. The measurements of angles and velocities R1, R2 and R2-R1 were analysed. Student's t-test to assess the reliability between the measurements of R1 and R2; and angles R2 and R2-R1 showed statistical significance, which confirmed the reliability of the scale. CONCLUSIONS: The Tardieu scale proved robust. It is important to note that the sample size, the time of evolution of the disease and the age of the patients may influence the results of the scale.

TITLE: Validez y fiabilidad de la escala de Tardieu para evaluar la espasticidad en miembro superior en adultos con enfermedad cerebrovascular. Revisión sistemática.Introducción. El incremento en el número de personas con espasticidad en los miembros superiores como secuela de una enfermedad cerebrovascular, que impacta negativamente en la autonomía, la independencia funcional y la participación, y afecta a la calidad de vida de las personas, demanda la aplicación de herramientas clínicas precisas y objetivas para su medición y evaluación. Objetivo. Evaluar la validez y la fiabilidad de la escala de Tardieu en la evaluación de la espasticidad en las extremidades superiores de adultos con enfermedad cerebrovascular. Materiales y métodos. La estrategia de búsqueda se implementó en ocho bases de datos; el protocolo de revisión sistemática se registró previamente en INPLASY (registro n.o 2023110076). La síntesis de la evidencia se llevó a cabo en tres fases: presentación tabular de resultados, evaluación de la calidad de los artículos y síntesis narrativa de los hallazgos. Resultados. De los 33 artículos identificados, sólo tres cumplieron con las variables que permiten establecer la validez y la fiabilidad de la escala de Tardieu. Se analizaron las medidas de los ángulos y velocidades R1, R2 y R2-R1. La prueba de la t de Student para evaluar la fiabilidad entre las medidas de R1 y R2; los ángulos R2 y R2-R1 mostraron significancia estadística, lo que confirmó la confiabilidad de la escala. Conclusiones. La escala de Tardieu demostró robustez. Es importante considerar que el tamaño de la muestra, el tiempo de evolución de la enfermedad y la edad de los pacientes pueden influir en los resultados de la escala.

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BACKGROUND: Autosomal recessive spastic ataxia ofCharlevoix-Saguenay (ARSACS) is a rare neurodegenerative disorder characterizedby early-onset cerebellar ataxia, peripheral sensorimotor neuropathy, and lowerlimb spasticity. We present clinical andgenetic data of the first Bulgarian patients diagnosed with ARSACS by wholeexome sequencing (WES). METHODS: Variant filtering was performed usinglocally established pipeline and the selected variants were analysed by Sangersequencing. All patients underwent clinical examination and testingincluding the standard rating scales for spastic paraplegia and ataxia. RESULTS: Five different SACS gene variants, three of which novel, have been identified inpatients from three different ethnic groups. In addition to the classicalclinical triad, brain MRI revealed cerebellar atrophy, linear pontineT2-hypointensities, and hyperintense rim lateral tothalamus combined with retinal nerve fiber layer thickening on opticcoherence tomography (OCT). CONCLUSION: We expand the mutation, geographic, and phenotypic spectrum of ARSACS, adding Bulgaria to the world map of the disease, and drawing attention to the fact that it is still misdiagnosed. We demonstrated that brain MRI and OCT are necessary clinical tests for ARSACS diagnosis, even if one of the cardinal clinical features is lacking.

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BACKGROUND: Evaluating muscle spasticity in children with cerebral palsy (CP) is essential for determining the most effective treatment strategies. This scoping review assesses the current methods used to evaluate muscle spasticity, highlighting both traditional and innovative technologies, and their respective advantages and limitations. METHODS: A search (to April 2024) used keywords such as muscle spasticity, cerebral palsy, and assessment methods. Selection criteria included articles involving CP children, assessing spasticity objectively/subjectively, comparing methods, or evaluating method effectiveness. RESULTS: From an initial pool of 1971 articles, 30 met our inclusion criteria. These studies collectively appraised a variety of techniques ranging from well-established clinical scales like the modified Ashworth Scale and Tardieu Scale, to cutting-edge technologies such as real-time sonoelastography and inertial sensors. Notably, innovative methods such as the dynamic evaluation of range of motion scale and the stiffness tool were highlighted for their potential to provide more nuanced and precise assessments of spasticity. The review unveiled a critical insight: while traditional methods are convenient and widely used, they often fall short in reliability and objectivity. CONCLUSION: The review discussed the strengths and limitations of each method and concluded that more reliable methods are needed to measure the level of muscle spasticity more accurately.

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We present an infant referred to Developmental Paediatrics for delays, slow growth, hypotonia, esotropia and spasticity. Over the course of 2 months, the infant's exam progressed, demonstrating worsening spasticity and tonal changes in the setting of a normal brain MRI with acquired microcephaly. Genetic testing demonstrated a pathogenic CTNNB1 nonsense mutation. Following the discovery of the underlying cause for the child's clinical picture, the child was evaluated by therapeutic services and neurology, which was initially only available via asynchronous telehealth, due to a resource limited area. Cerebral palsy is a nonprogressive neurodevelopmental disorder and, when associated with developmental delay, qualifies for further genetic investigation into the underlying aetiology. Genetic testing recommendations exist for developmental delay, but there is no current algorithm regarding testing for cerebral palsy. Education and clear guidelines on genetic testing allow for better prognostication and potential treatment in cases of cerebral palsy, especially when associated with other disorders.

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The goal-setting process is pivotal in managing patients with disabling spasticity. This case-control study assessed the role of diagnostic nerve blocks in guiding the goal-setting process within goal-targeted treatment of spasticity with botulinum neurotoxin-A. In this case-control study, patients with disabling spasticity underwent either a goal-setting process based on the patient's needs and clinical evaluation (control group) or additional diagnostic nerve block procedures (case group). All enrolled patients underwent a focal treatment with botulinum neurotoxin-A injection and a 1-month follow-up evaluation during which goal achievement was quantified using the goal attainment scaling-light score system. Data showed a higher goal achievement rate in the case group (70%) than in the control group (40%). In conclusion, diagnostic nerve blocks may help guide the goal-setting process within goal-targeted treatment of spasticity with botulinum neurotoxin-A towards more realistic and achievable goals, thereby improving the outcomes of botulinum neurotoxin-A injection. Future studies should better explore the role of diagnostic nerve blocks to further personalize botulinum neurotoxin-A according to individual patients' preferences and requirements.

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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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BACKGROUND: The Leg Activity Measure is the only self-report measure that has been published to date that takes into account both the influence on quality of life and passive and active function in the literature. AIMS: The purpose is to examine the translation, cross-cultural adaptation validity and reliability of the Turkish version of the Leg Activity Measure (Tr-LegA). METHODS: Neurological patients (n = 52) with lower limb spasticity (aged 47.09 ± 14.74 years) were enrolled. The study consisted of two stages. At the first stage, the scale was translated into Turkish and culturally adapted. Validity and reliability analyses were conducted at the second stage. Construct validity was evaluated by exploratory factor analysis (EFA). The Rivermead Mobility Index (RMI), Nottingham Health Profile (NHP), and Functional Independence Measure (FIM) were used for convergent validity. The reproducibility (test-retest reliability) was assessed by the intraclass correlation coefficient (ICC). Furthermore, the standard error of measurement (SEM) was calculated. RESULTS: EFA suggested one factor for the Passive Function and two factors for the Active Function and Impact on Quality of Life Scales (QoL). Tr-LegA Passive and Active Function Scales were correlated with the total RMI, NHP, and FIM (p < 0.05). Tr-LegA Impact on Quality of Life Scale was correlated with the RMI and NHP (p < 0.05). Tr-LegA Passive Function Scale (ICC = 0.997), Tr-LegA Active Function Scale (ICC = 0.996), and Tr-LegA Impact on Quality of Life Scale (ICC = 0.976) had good reliability. Only Passive Function Scale had a significant floor effect (25%). CONCLUSIONS: Tr-LegA is a valid and reliable multidimensional scale for passive and active function and quality of life in patients with lower limb spasticity. THE CLINICAL TRIAL NUMBER: NCT05182411.

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A 24-year-old man presented with progressive gait instability, marked spinal cord atrophy, and dental radiography showing the absence of several elements, microdontia, and taurodontia. What is your diagnosis?

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INTRODUCTION/AIMS: The frequency and distribution of upper motor neuron (UMN) signs in primary lateral sclerosis (PLS) are unknown. We aimed to study the spectrum of UMN signs in PLS and compare it with hereditary spastic paraplegia (HSP). METHODS: We retrospectively analyzed the frequency of different UMN signs, including hyperreflexia (limbs and jaw), limb and tongue spasticity, Babinski, and Hoffman signs, in PLS patients at first observation and compared this respect to onset region and symptom duration. We also compared PLS versus HSP patients. RESULTS: We included 34 PLS and 20 HSP patients, with a median symptom duration at first visit of 3.0 (interquartile range, IQR = 4.0) and 19.0 (IQR = 22.0) years, respectively. In PLS patients, hyperreflexia of upper (UL) (88.2%) and lower (LL) (91.2%) limbs, and LL spasticity (79.4%) were the most common findings. Spasticity of LL was significantly (p = .012) more frequent in LL-spinal onset subgroup, tongue spasticity in bulbar-onset subgroup (p = .021), and Hoffman sign in UL-spinal onset subgroup (p = .024). The PLS subgroup with shorter disease duration had a higher frequency of abnormal jaw jerk reflex (p = .037). Compared with HSP, PLS patients had a higher frequency of UL hyperreflexia (88.2% vs. 42.1%, p < .001) and UL spasticity (44.1% vs. 0.0%, p < .001). Asymmetric distribution of UMN signs was present in PLS and not in HSP. DISCUSSION: In PLS, UL UMN signs are nearly always present and UMN sign distribution appears to be associated with onset region. At first observation, bulbar involvement, asymmetrical distribution of UMN signs and UL spasticity may indicate PLS versus HSP.

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Objective: To explore the diagnosis and treatment of adolescence-onset methylenetetrahydrofolate reductase (MTHFR) deficiency. Methods: This was a retrospective case study. Nine patients with adolescence-onset MTHFR deficiency were diagnosed at Peking University First Hospital from January 2016 to December 2022, and followed up for more than 1 year. Their general information, clinical manifestations, laboratory tests, cranial images, MTHFR gene variants, diagnosis, treatment, and outcome were analyzed retrospectively. Results: The 9 patients came from 8 families. They had symptoms at age of 8.0 years to 17.0 years and diagnosed at 9.0 years to 17.5 years. Eight were male and 1 was female. Two patients were brothers, the elder brother developed abnormal gait at 17.0 years; and the younger brother was then diagnosed at 15.0 years of age and treated at the asymptomatic stage, who was 18.0 years old with normal condition during this study. The main manifestations of the 8 symptomatic patients included progressive dyskinesia and spastic paralysis of the lower limbs, with or without intellectual decline, cognitive impairment and behavioral abnormalities. Totally, 15 variants of MTHFR gene were identified in the 9 patients, including 8 novel variants. Five patients had brain image abnormalities. Increased plasma total homocysteine level (65-221 µmol/L) was found in all patients, and decreased to 20-70 µmol/L after treatment with betaine and calcium folinate. Besides, the 8 symptomatic patients had their behavior and cognitive problems significantly improved, with a legacy of lower limb motor disorders. Conclusions: Late-onset MTHFR deficiency can occur in adolescence. The diagnosis is usually delayed because of non-specific clinical symptoms. The test of blood total homocysteine could be used as a selective screening test. Eight novel varients of MTHFR gene were identified. Timely treatment can improve clinical condition significantly, and pre-symptomatic treatment may prevent brain damage.

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Hemiparetic gait disorders are common in stroke survivors. A circumductory gait is often considered the typical hemiparetic gait. In clinical practice, a wide spectrum of abnormal gait patterns is observed, depending on the severity of weakness and spasticity, and the anatomical distribution of spasticity. Muscle strength is the key determinant of gait disorders in hemiparetic stroke survivors. Spasticity and its associated involuntary activation of synergistic spastic muscles often alter posture of involved joint(s) and subsequently the alignment of hip, knee, and ankle joints, resulting in abnormal gait patterns. Due to combinations of various levels of muscle weakness and spasticity and their interactions with ground reaction force, presentations of gait disorders are variable. From a neuromechanical perspective, a stepwise visual gait analysis approach is proposed to identify primary underlying causes. In this approach, the pelvic and hip joint movement is examined first. The pelvic girdle constitutes three kinematic determinants. Its abnormality determines the body vector and compensatory kinetic chain reactions in the knee and ankle joints. The second step is to assess the ankle and foot complex abnormality. The last step is to examine abnormality of the knee joint. Assessment of muscle strength and spasticity of hip, knee, and ankle/foot joints needs to be performed before these steps. Lidocaine nerve blocks can be a useful diagnostic tool. Recognizing different patterns and identifying the primary causes are critical to developing clinical interventions to improve gait functions.

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BACKGROUND: Following a severe acquired brain injury, neuro-orthopaedic disorders are commonplace. While these disorders can impact patients' functional recovery and quality of life, little is known regarding the assessment, management and treatment of neuro-orthopaedic disorders in patients with disorders of consciousness (DoC). OBJECTIVE: To describe neuro-orthopaedic disorders in the context of DoC and provide insights on their management and treatment. METHODS: A review of the literature was conducted focusing on neuro-orthopaedic disorders in patients with prolonged DoC. RESULTS: Few studies have investigated the prevalence of spastic paresis in patients with prolonged DoC, which is extremely high, as well as its correlation with pain. Pilot studies exploring the effects of pharmacological treatments and physical therapy show encouraging results yet have limited efficacy. Other neuro-orthopaedic disorders, such as heterotopic ossification, are still poorly investigated. CONCLUSION: The literature of neuro-orthopaedic disorders in patients with prolonged DoC remains scarce, mainly focusing on spastic paresis. We recommend treating neuro-orthopaedic disorders in their early phases to prevent complications such as pain and improve patients' recovery. Additionally, this approach could enhance patients' ability to behaviourally demonstrate signs of consciousness, especially in the context of covert awareness.

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Limb spasticity is caused by stroke, multiple sclerosis, traumatic brain injury and various central nervous system pathologies such as brain tumors resulting in joint stiffness, loss of hand function and severe pain. This paper presents with the Rehabotics integrated rehabilitation system aiming to provide highly individualized assessment and treatment of the function of the upper limbs for patients with spasticity after stroke, focusing on the developed passive exoskeletal system. The proposed system can: (i) measure various motor and kinematic parameters of the upper limb in order to evaluate the patient's condition and progress, as well as (ii) offer a specialized rehabilitation program (therapeutic exercises, retraining of functional movements and support of daily activities) through an interactive virtual environment. The outmost aim of this multidisciplinary research work is to create new tools for providing high-level treatment and support services to patients with spasticity after stroke.Clinical Relevance- This paper presents a new passive exoskeletal system aiming to provide enhanced treatment and assessment of patients with upper limb spasticity after stroke.

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Spasticity is a condition that profoundly impacts the ability to perform everyday tasks. However, its diagnosis requires trained physicians and subjective evaluations that may vary depending on the evaluator. Focal vibration of spastic muscles has been proposed as a non-invasive, pain-free alternative for spasticity modulation. We propose a system to estimate muscular tightness based on the propagation of elastic waves in the skin generated by focal vibration of the upper limb. The developed system generates focalized displacements on the biceps muscle at frequencies from 50 to 200 Hz, measures the vibration acceleration on the vibration source (input) and the distant location (output), and extracts features of ratios between input and output. The system was tested on 5 healthy volunteers while lifting 1.25 - 11.25 kg weights to increase muscle tone resembling spastic conditions, where the vibration frequency and weight were selected as explanatory variables. An increase in the ratio of the root mean squares proportional to the weight was found, validating the feasibility of the current approach to estimating muscle tightness.Clinical Relevance- This work presents the feasibility of a vibration-based system as an alternative method to objectively diagnose the degree of spasticity.

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Spasticity is a motor disorder with high prevalence and critical consequences following a stroke. Reliable and sensitive measurements are important to guide the selection and evaluation of treatment strategies. Technology-assisted methods, such as the surface electromyography (sEMG) technique, have been developed to measure spasticity as sensitive and accurate alternatives to commonly used clinical scales. However, sEMG amplitude based measures may confound spasticity-induced muscle activities with other types of muscle contractions. This study thus introduces the idea of using sEMG frequency information to detect spasticity as a potential solution to overcome the limitations of existing sEMG based measures. The preliminary results of three patients demonstrate the possibility and future research directions for this approach.

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the assessment of muscle properties is an essential prerequisite in the treatment of post-stroke muscle spasticity. Previous studies have shown that muscle coactivation, which reflects the simultaneous activation of agonist and antagonist muscle groups, is associated with muscle spasticity during voluntary contraction. However, current spasticity assessment approaches do not often consider muscle coactivation for passive contraction measured with surface electromyography (sEMG). The purpose here is to evaluate the validity and reliability of muscle co-activation based on sEMG for assessing spasticity of post-stroke patients. This study was conducted on 39 chronic hemiplegia post-stroke patients with varying degrees of elbow flexor spasticity. The severity of spasticity was assessed with Modified Ashworth Scale (MAS). The patients produced elbow flexion passively on affected arm. Two-channel surface sEMG recordings were acquired simultaneously for the biceps and triceps muscles. The effectiveness and reliability of the EMG-based spasticity assessment method were evaluated using Spearman's correlation analysis and intra class correlation coefficients (ICCs). The results showed that there was a statistically significant positive relationship between the level of activity and the coactivation index (R=0.710, P=0.003), while the ICCs for intra trial measures ranged between 0.928 and 0.976. Muscle coactivation is a promising tool for continuously quantifying muscle spasticity in post-stroke patients, suggesting that the EMG-based muscle coactivation index could be useful for assessing motor function.

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BACKGROUND: The causes of spasticity are various and include cerebral palsy, spinal cord injury, stroke, multiple sclerosis or other congenital or acquired lesions of the central nervous system (CNS). While there is often a partial functional component, spasticity also results in varying degrees of impairment of the quality of life. OBJECTIVE: A review of surgical treatment options for spasticity. MATERIAL AND METHODS: A systematic PubMed review of the literature on epidemiology and treatment options with a focus on neurosurgical interventions for spasticity and developments in the last 20 years as well as inclusion of still valid older landmark papers was carried out. Illustration of indications, technique, follow-up, and possible pitfalls of the different methods for the surgical treatment of spasticity. RESULTS: Depending on the affected region, the number of muscle groups, and the extent of spasticity, focal (selective peripheral neurotomy, nerve transfer), regional (selective dorsal rhizotomy), or generalized (baclofen pump) procedures can be performed. The indications are usually established by an interdisciplinary team. Conservative (physiotherapy, oral medications) and focally invasive (botulinum toxin injections) methods should be performed in advance. In cases of insufficient response to treatment or only short-term relief, surgical methods can be evaluated. These are usually preceded by test phases with, for example, trial injections. CONCLUSION: Surgical methods are a useful adjunct in cases of insufficient response to conservative treatment in children and adults with spasticity.

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