Management of a Thumb-In-Palm Deformity in Adult Patients With Upper Motor Neuron Syndrome.

The thumb-in-palm (TIP) deformity in adults with upper motor neuron syndrome results from an imbalance of extrinsic and intrinsic muscular forces. Traditionally, the thumb is adducted against the index ray, and flexed to varying degrees at the metacarpophalangeal and interphalangeal joints. However, not all TIP deformities result from the same underlying imbalances. The severity of the deformity ranges over a spectrum dependent upon the involved muscle groups and underlying spasticity, myostatic contracture, and/or joint contractures. Surgical procedures for correcting a TIP deformity can be classified broadly as procedures used for functional, present volitional motor control, versus nonfunctional, absent motor control. Techniques include tenotomies, tenodeses, tendon lengthenings, tendon transfers, tendon reroutings, neurectomies, and joint releases. A focused physical examination is key in developing a patient-specific treatment algorithm.

Surgical Management of Spastic Shoulder Deformities in Adult Patients.

Spastic shoulder deformity in patients with upper motor neuron syndrome results from an imbalance of muscle forces about the shoulder girdle. In typical spastic deformities, the shoulder assumes an adducted and internally rotated posture. The severity of the deformity can range over a spectrum depending on the involved muscle groups, degree of spasticity, and presence of myostatic and/or joint contractures. Surgical options to correct the spastic shoulder deformity can be broadly classified as procedures for the functional versus nonfunctional shoulder or, in other words, preserved versus absent volitional motor control, respectively. Techniques include tenotomy, fractional lengthening, tenodesis, and periarticular soft tissue release. A focused physical examination is imperative in developing a patient-specific treatment algorithm.

Development of muscle contractures and spasticity during subacute rehabilitation after severe acquired brain injury: a prospective cohort study.

Objective: This study investigated the development of contractures, passive stiffness, and spasticity in the ankle joint in patients with severe acquired brain injury (ABI) from admission to rehabilitation unit until 1-year post-injury compared to healthy controls. Design: An observational longitudinal cohort study Methods and procedures: Nineteen patients (26 affected ankle joints) with severe ABI >17 years old and with paresis of a lower limb admitted to sub-acute neurorehabilitation were compared to 14 healthy controls (28 ankle joints). Passive and reflex-mediated ankle joint stiffness was obtained measuring torque, range of motion, velocity and acceleration of the ankle movements. Data was collected at inclusion, after 4-5 weeks, after 8-9 weeks and at 1-year follow-up. Outcomes and results: At admittance to rehabilitation range of motion and stiffness was significantly lower compared to controls. Range of motion decreased by one degree in three weeks and passive ankle joint stiffness increased significantly by 1% per week. More patients than controls had no stretch reflex. Conclusion: Patients with severe ABI show reduced mobility and increased passive stiffness despite less spasticity in the ankle joint compared to healthy controls. Research and clinical practice should therefore focus intensively on the prevention of contractures in the ankle joint. ISRCTN17910097.

Surgical Management of Upper Extremity Deformities in Patients With Upper Motor Neuron Syndrome.

Injury to the central nervous system can create upper extremity deformities and dysfunction, typically caused by a cerebrovascular accident, traumatic brain injury, anoxic brain injury, or spinal cord injury. Regardless of the etiology, disruption of inhibitory upper motor neuron (UMN) pathways can lead to a constellation of symptoms such as muscle weakness, decreased motor control, hyperexcitable tendon reflexes, muscle spasticity, and agonist-antagonist cocontraction that characterizes a condition known as UMN syndrome. The magnitude of neurorecovery varies among patients who have sustained brain injuries and can be classified as having a functional or nonfunctional upper extremity based on the presence or absence of volitional motor control at a specific joint, respectively. Many surgical procedures can be employed to optimize function, decrease pain, improve hygiene, and enhance cosmesis in patients with UMN syndrome.

Effect of radial shock wave therapy on pain and muscle hypertonia: a double-blind study in patients with multiple sclerosis.

BACKGROUND: Radial shock wave therapy (RSWT) has been extensively used in rehabilitative medicine to treat pain, and more recently muscle hypertonia, in patients with cerebral palsy and stroke. OBJECTIVES: To assess the long-term effects of RSWT in a cohort of subjects affected by multiple sclerosis (MS) who were suffering from painful hypertonia of ankle extensor muscles. METHODS: In this randomised, double blind, placebo-controlled study, we treated 34 patients with four sessions of RSWT (once weekly) and treated 34 patients with placebo. Participants were assessed at baseline, 1 week after the first session, and 1 week and 4 weeks after the last session. We measured pain using the visual analogue scale for pain, while we assessed muscle tone using the modified Ashworth scale and evaluated spinal excitability using the H-reflex. RESULTS: After RSWT, muscle tone decreased 1 week after the last session and pain decreased at all the follow-up evaluations, while spinal excitability was unaffected. No significant changes were found after the placebo treatment. CONCLUSIONS: RSWT can reduce pain and muscle tone in MS patients without adverse effects. The lack of RSWT effects on spinal excitability supports the idea that RSWT is likely to act on non-reflex hypertonia, for example reducing muscle fibrosis.

Treatment of spasticity.

Spasticity is characterized by an enhanced size and reduced threshold for activation of stretch reflexes and is associated with "positive signs" such as clonus and spasms, as well as "negative features" such as paresis and a loss of automatic postural responses. Spasticity develops over time after a lesion and can be associated with reduced speed of movement, cocontraction, abnormal synergies, and pain. Spasticity is caused by a combination of damage to descending tracts, reductions in inhibitory activity within spinal cord circuits, and adaptive changes within motoneurons. Increased tone, hypertonia, can also be caused by changes in passive stiffness due to, for example, increase in connective tissue and reduction in muscle fascicle length. Understanding the cause of hypertonia is important for determining the management strategy as nonneural, passive causes of stiffness will be more amenable to physical rather than pharmacological interventions. The management of spasticity is determined by the views and goals of the patient, family, and carers, which should be integral to the multidisciplinary assessment. An assessment, and treatment, of trigger factors such as infection and skin breakdown should be made especially in people with a recent change in tone. The choice of management strategies for an individual will vary depending on the severity of spasticity, the distribution of spasticity (i.e., whether it affects multiple muscle groups or is more prominent in one or two groups), the type of lesion, and the potential for recovery. Management options include physical therapy, oral agents; focal therapies such as botulinum injections; and peripheral nerve blocks. Intrathecal baclofen can lead to a reduction in required oral antispasticity medications. When spasticity is severe intrathecal phenol may be an option. Surgical interventions, largely used in the pediatric population, include muscle transfers and lengthening and selective dorsal root rhizotomy.

Expert consensus on the surgical evaluation and management of upper extremity spasticity in adults.

In the last decade there has been incredible interest and advancement in the surgical care of adult patients with upper motor neuron (UMN) injuries. Spasticity represents a prevalent and debilitating feature of UMN syndrome, which can result from cerebral palsy, spinal cord injury, cerebrovascular accident and traumatic or anoxic brain injury. While several diagnostic tools and management strategies have been described for upper limb spasticity, evidence-based practice guidelines do not currently exist due to low patient volume and a paucity of surgeons routinely performing surgeries in UMN syndrome patients. As such, expert consensus may help provide guidance for patients, therapists and clinicians alike. In this article an expert panel was assembled, and the Delphi method was utilized to present diagnostic considerations, define operative indications, discuss surgical treatment modalities and encourage a standard set of outcome measures for patients with upper extremity spasticity.

Early versus late injections of Botulinumtoxin type A in post-stroke spastic movement disorder: A literature review.

Post-stroke spastic movement disorder (PS-SMD) is one of the main causes of severe disability in the chronic phase after stroke. The prevalence of SMD goes up with time after stroke to more than 28% in the chronic phase., Its secondary complications such as contracture, abnormal postures and/or movement patterns, spasticity-associated pain also increase with time after stroke when physical and medical management of PS-SMD have been delayed in the early stroke phase. It has been published by several controlled studies that the earlier physical and medical measures, such as botulinum toxin type A (BoNT-A) therapy are included in rehabilitative strategies for the SMD, the fewer secondary complications, especially soft tissue contractures and pain occurred. Several studies showed that goal-orientated management of PS-SMD including BoNT-A therapy, applied within a few weeks and three months - in the early subacute phase after stroke onset - prevented or reduced the development of severe or disabling SMD and its secondary complications, more effective than late application of BoNT-A therapy - in the chronic phase after stroke. In multiple prospective cohort studies, various predictors and predictive approaches for detection of patients on risk to development PS-SMD were found. Based on that information and the controlled studies that showed reduction in PS-SMD complications following early treatment with BoNT-A nowadays, early treatment of PS-SMD in the early subacute phase following stroke is recommended to avoid or reduce the development of post-stroke disability and to improve the outcome of rehabilitation. In this review, we discuss the optimal timing to apply BoNT-A therapy in patients with already present as well as those at high risk of severe PS-SMD.

Surgical management of the spastic elbow.

Background: We performed a retrospective review of patients undergoing surgery for elbow spasticity. We present our findings and expected outcomes according to degree of elbow fixed flexion contracture. Methods: Data collected included age, Modified Ashworth Score, pre and post-operative range of motion, indications for surgery and the Goal Attainment Score. Contracture severity was classified into five groups based on goniometric measurements. Surgical procedures were categorised into three groups. Results: A total of 114 elbows underwent surgical release. The mean age at surgery was 18.5 years and the mean follow-up was 20 months. Preoperatively, the median Modified Ashworth Score was 2 and the mean contracture was 68° (35° fixed and 33° dynamic). The median number of structures released was 3 (range: 1-6). Surgical procedures were classified as biceps sparing (27%), biceps lengthening (53%) and extended releases (18%). Mean improvement in extension was 59°, and the mean improvement in Goal Attainment Score was 36 (mean attainment score 62). The mean residual contracture was 9°. Patient satisfaction was high with over 90% of surgical indications met. Discussion: Contracture severity of the spastic elbow can be categorised by degree of fixed flexion deformity and therefore treatment can be allocated accordingly.

An Observational Cross-Sectional Study of Gender and Disability as Determinants of Person-Centered Medicine in Botulinum Neurotoxin Treatment of Upper Motoneuron Syndrome.

The motor behaviour of patients with Upper Motor Neuron Syndrome (UMNS) is characterised by spasticity. The first-line treatment for this clinical condition is Botulinum neurotoxin A (BoNTA), but the number and key locations of muscles which need to be treated is not much discussed in the literature. Cross-sectional analysis of outpatient cohort with UMNS spasticity, who were potential candidates for BoNTA treatment, was performed. Between November 2020 and November 2021, all consecutive adult patients eligible for BoNTA treatment were enrolled. The inclusion criteria encompass UMNS spasticity (onset being ≥6 months), with disabling muscles hypertonia. Patients underwent a clinical evaluation, a comprehensive assessment with the Modified Ashworth Scale, with the Modified Rankin Scale, and a patients' perception-centred questionnaire. In total, 68 participants were enrolled in the study, among them 40 (58.8%) were male; mean age 57.9 ± 15.1. In women, BoNTA was more frequently required for adductor group muscles, independently from potential confounders (OR = 7.03, 95%CI: 1.90-25.97). According to the pattern of disability, patients with hemiparesis more frequently need to be treated in the upper limb, whereas the diplegia/double-hemiparesis group needed to be treated more frequently at the adductor and crux muscles compared to their counterparts. UMNS spasticity in women could require more attention to be paid to the treatment of adductor muscle spasticity, potentially because the dysfunction of those muscles could influence sphincteric management, required for perineal hygiene and/or sexual life.

Effectiveness of Botulinum Toxin on Pain in Stroke Patients Suffering from Upper Limb Spastic Dystonia.

This observational study aimed at investigating pain in stroke patients with upper limb spastic dystonia. Forty-one consecutive patients were enrolled. A 0-10 numeric rating scale was used to evaluate pain at rest and during muscle tone assessment. Patients were asked to indicate the most painful joint at passive mobilization (shoulder, elbow, wrist-fingers). The DN4 questionnaire was administered to disclose neuropathic pain. All patients were assessed just before and 1 month after incobotulinumtoxin-A treatment. Pain was present in 22 patients, worsened or triggered by passive muscle stretching. DN4 scored < 4 in 20 patients. The most painful joints were wrist-fingers in 12 patients, elbow in 5 patients and shoulder in the remaining 5 patients. Both elbow and wrist-fingers pain correlated with muscle tone. BoNT-A treatment reduced pain in all the joints, including the shoulder. We discussed that nociceptive pain is present in a vast proportion of patients with upper limb spastic dystonia. BoNT-A treatment reduced both spastic dystonia and pain in all the joints but the shoulder, where the effect on pain could be mediated by the reduction of pathological postures involving the other joints.

Assessment of 30-Day Adverse Events in Single-Event, Multilevel Upper Extremity Surgery in Adult Patients with Upper Motor Neuron Syndrome.

BACKGROUND: Upper motor neuron (UMN) syndrome consists of muscle spasticity, weakness, and dyssynergy due to a brain or spinal cord injury. The purpose of this study is to describe the perioperative adverse events for adult patients undergoing single-event, multilevel upper extremity surgery (SEMLS) due to UMN syndrome. METHODS: A retrospective case series was performed for 12 consecutive adult patients who underwent SEMLS to correct upper extremity dysfunction or deformity secondary to UMN syndrome. The evaluation consisted of primary outcome measures to identify readmission rates and classify adverse events that occurred within 30 days after surgery. RESULTS: All 12 patients were functionally dependent with 50% (n = 6) men and 50% (n = 6) women at a mean age of 43.6 years (range: 21-73) with a mean of 5.92 (range: 0-16) comorbid diagnoses at the time of surgery. There were no intraoperative complications, hospital readmissions, or deaths among the 12 patients. Five patients experienced 5 minor postoperative complications that consisted of cast- or orthosis-related skin breakdown remote from the incision (n = 3), incidental surgical site hematoma that required no surveillance or intervention (n = 1), and contact dermatitis attributed to the surgical dressing that resolved with topical corticosteroids (n = 1). CONCLUSIONS: With an appropriate multidisciplinary approach, there is minimal risk for developing perioperative and 30-day postoperative adverse events for adults undergoing SEMLS to correct upper extremity deformities secondary to UMN syndrome. LEVEL OF EVIDENCE: Level IV.

Teenage Neurologic Manifestation of a Complex Brain Malformation Consisting of Gray Matter Heterotopia, Schizencephaly and Absent Septum Pellucidum.

Teaching point: Since some minor congenital brain defects manifest long after birth, even in adults they should be kept in the differential of new epileptic seizures or focal neurologic deficits.

Introduction to spasticity and related mouse models.

Although spasticity is one of the most common causes of motor disability worldwide, its precise definition and pathophysiology remain elusive, which to date renders its experimental targeting tricky. At least in part, this difficulty is caused by heterogeneous phenotypes of spasticity-causing neurological disorders, all causing spasticity by involving upper motor neurons. The most common clinical symptoms are a series of rapid muscle contractions (clonus), an increased muscle tone (hypertonia), and augmented tendon reflex activity (hyperreflexia). This muscle overactivity is due to disturbed inhibition of spinal reflexes following upper motor neuron dysfunction. Despite a range of physical and pharmacological therapies ameliorating the symptoms, their targeted application remains difficult. Therefore, to date, spasticity impacts rehabilitative therapy, and no therapy exists that reverses the pathology completely. In contrast to the incidence and importance of spasticity, only very little pre-clinical work in animal models exists, and this research is focused on the cat or the rat spastic tail model to decipher altered reflexes and excitability of the motor neurons in the spinal cord. Meanwhile, the characterization of spasticity in clinically more relevant mouse models of neurological disorders, such as stroke, remains understudied. Here, we provide a brief introduction into the clinical knowledge and therapy of spasticity and an in-depth review of pre-clinical studies of spasticity in mice including the current experimental challenges for clinical translation.

Spasticity, spastic dystonia, and static stretch reflex in hypertonic muscles of patients with multiple sclerosis.

OBJECTIVE: To investigate prevalence of EMG patterns underlying hypertonia in multiple sclerosis (MS) and whether these patterns indicate different levels of spinal excitability. METHODS: We investigated the EMG activity recorded from 108 hypertonic muscles of 59 consecutive MS patients. To investigate spastic dystonia (SD), we looked for the presence of EMG activity in muscles in a resting position. To investigate dynamic stretch reflex (DSR) and static stretch reflex (SSR), we looked for the presence of EMG activity in response to a manually performed passive stretch of the muscle. RESULTS: DSR was evoked in 104 muscles. In 51 muscles, DSR was the sole EMG activity. This pattern corresponds to the classical notion of spasticity, and was predominant in extensors. In contrast, SSR was detected in 48 muscles - predominantly in flexors. SD was observed in 28 muscles, showing even distribution in flexor and extensor muscles. Only in the flexors, SSR was associated with a larger DSR compared to spasticity. CONCLUSIONS: These findings likely depend on the central effects of both flexor and extensor spindle afferents on the homonymous spinal motor neurons. SIGNIFICANCE: Improving our capacity to assess spinal excitability in MS patients.

Effect of multichannel transcranial direct current stimulation to reduce hypertonia in individuals with prolonged disorders of consciousness: A randomized controlled pilot study.

BACKGROUND: Spasticity management in severely brain-injured patients with disorders of consciousness (DOC) is a major challenge because it leads to complications and severe pain that can seriously affect quality of life. OBJECTIVES: We aimed to determine the feasibility of a single session of transcranial direct current stimulations (tDCS) to reduce spasticity in chronic patients with DOC. METHODS: We enrolled 14 patients in this double-blind, sham-controlled randomized crossover pilot study. Two cathodes were placed over the left and right primary motor cortex and 2 anodes over the left and right prefrontal cortex. Hypertonia of the upper limbs and level of consciousness were assessed by the Modified Ashworth Scale (MAS) and the Coma Recovery Scale-Revised (CRS-R). Resting state electroencephalography was also performed. RESULTS: At the group level, spasticity was reduced in only finger flexors. Four responders (29%) showed reduced hypertonicity in at least 2 joints after active but not sham stimulation. We found no behavioural changes by the CRS-R total score. At the group level, connectivity values in beta2 were higher with active versus sham stimulation. Relative power in the theta band and connectivity in the beta band were higher for responders than non-responders after the active stimulation. CONCLUSION: This pilot study highlights the potential benefit of using tDCS for reducing upper-limb hypertonia in patients with chronic DOC. Large-sample clinical trials are needed to optimize and validate the technique.

Motion analysis for the evaluation of muscle overactivity: A point of view.

Muscle overactivity is a general term for pathological increases in muscle activity such as spasticity. It is caused by damage to the central nervous system at the cortical, subcortical or spinal levels, leading to an upper motor neuron syndrome. In routine clinical practice, muscle overactivity, which induces abnormal muscle tone, is usually evaluated by using the Modified Ashworth Scale or the Tardieu Scale. However, both of these scales involve testing in passive conditions that do not always reflect muscle activity during dynamic tasks such as gait or reaching. To determine appropriate treatment strategies, muscle overactivity should be evaluated by using objective measures in dynamic conditions. Instrumental motion analysis systems that include 3-D motion analysis and electromyography are very useful for this purpose. The method can be used to identify patterns of abnormal muscle activity that can be related to abnormal kinematic patterns. It allows for objective and accurate assessment of the effects of treatments to reduce muscle overactivity on the movement to be improved. The aim of this point-of-view article is to describe the utility of instrumental motion analysis and to outline both its numerous advantages in evaluating muscle overactivity and to present the current limitations for its use (e.g., cost, the need for an engineer, errors relating to marker placement and cross talk between electromyography sensors).

Non-invasive treatment of patients with upper extremity spasticity following stroke using paired trans-spinal and peripheral direct current stimulation.

BACKGROUND: Muscle spasticity is a common impediment to motor recovery in patients with chronic stroke. Standard-of-care treatments such as botulinum toxin injections can temporarily relieve muscle stiffness and pain associated with spasticity, but often at the expense of increased muscle weakness. Recent preclinical investigations of a non-invasive treatment that pairs trans-spinal direct current stimulation and peripheral nerve direct current stimulation (tsDCS+pDCS) provided promising data for a novel approach based on bioelectronic medicine for the treatment of patients with post-stroke spasticity. METHODS: Twenty-six patients with upper limb hemiparesis and wrist spasticity at least 6 months after their initial stroke participated in this single-blind crossover design study to test whether tsDCS+pDCS reduces chronic upper-extremity spasticity. Subjects received five consecutive daily sessions (20 min of stimulation or sham) of anodal tsDCS+pDCS, separated by a one-week washout period. The sham condition always preceded the active condition. Clinical and objective measures of spasticity and motor function were collected before and after each condition, and for five weeks after the completion of the active intervention. RESULTS: Subjects treated with active tsDCS+pDCS demonstrated significant reductions in both Modified Tardieu Scale scores (summed across the upper limb, P < 0.05), and in objective torque measures (Nm) of the spastic catch response at the wrist flexor (P < 0.05), compared to the sham condition. Motor function also improved significantly (measured by the Fugl-Meyer and Wolf Motor Function Test; P < 0.05 for both tests) after active treatment. CONCLUSIONS: tsDCS+pDCS intervention alone significantly reduced upper limb spasticity in participants with stroke. Decreased spasticity was persistent for five weeks after treatment, and was accompanied by improved motor function even though patients were unsupervised and there was no prescribed activity or training during that interval. TRIAL REGISTRATION: NCT03080454, March 15, 2017.

Transcutaneous electrical nerve stimulation for spasticity: A systematic review. / Estimulación eléctrica nerviosa transcutánea como tratamiento de la espasticidad: una revisión sistemática.

INTRODUCTION: Although transcutaneous electrical nerve stimulation (TENS) has traditionally been used to treat pain, some studies have observed decreased spasticity after use of this technique. However, its use in clinical practice is still limited. Our purpose was twofold: to determine whether TENS is effective for treating spasticity or associated symptoms in patients with neurological involvement, and to determine which stimulation parameters exert the greatest effect on variables associated with spasticity. DEVELOPMENT: Two independent reviewers used PubMed, PEDro, and Cochrane databases to search for randomised clinical trials addressing TENS and spasticity published before 12 May 2015, and selected the articles that met the inclusion criteria. Of the initial 96 articles, 86 were excluded. The remaining 10 articles present results from 207 patients with a cerebrovascular accident, 84 with multiple sclerosis, and 39 with spinal cord lesions. CONCLUSIONS: In light of our results, we recommend TENS as a treatment for spasticity due to its low cost, ease of use, and absence of adverse reactions. However, the great variability in the types of stimulation used in the studies, and the differences in parameters and variables, make it difficult to assess and compare any results that might objectively determine the effectiveness of this technique and show how to optimise parameters.

Muscle Overactivity in the Upper Motor Neuron Syndrome: Pathophysiology.

The upper motor neuron syndrome is composed of negative, positive, and rheologic features. The positive features have to do with muscle overactivity, which results from abnormal signal processing in the spinal cord, from altered supraspinal inputs and/or dysfunctional segmental spinal modulatory mechanisms. The negative features are characterized by a reduction of muscle activity and loss of selective muscle control. Third are rheologic features characterized by changes in the physical properties of muscle. These 3 types of clinical features share perpetuating cause-and-effect relationships. This discussion highlights pathophysiology involved in muscle overactivity in the context of the upper motor neuron syndrome.