Upper Motor Neuron Syndrome Research Articles

Spasticity or periodic limb movements?

Spasticity and spasms are distressing features of the upper motor neuron syndrome (UMNS) following spinal cord injuries (SCI) or multiple sclerosis (MS), and have common therapeutic implications. Despite an increase of antispastic drugs and strategies, sometimes up to the surgical implant of intrathecal baclofen pump, some patients still complain of disabling spasms, which poses diagnostic and therapeutic challenges. Although clinically similar, flexor spasms due to pyramidal tract disruption must be clearly differentiated from periodic limb movements (PLM), often accompanying restless leg syndrome (RLS) and occurring during sleep. We raised the hypothesis of this differential as a diagnostic confusion in this particular population. The aim of this study was twofold: 1) to search for RLS with PLM in consecutive patients referred for uncontrolled disabling spasms despite treatment, by nocturnal polysomnography (PSG); 2) to report the efficacy of dopaminergic agonists on PLM in this population. Observational prospective study. Spasticity Clinic at the Raymond-Poincaré University Hospital, Garches, France. All consecutive patients with MS or SCI, referred to our spasticity clinic from March 2014 to July 2016 for the management of persistent and disabling spasms despite treatment. Obvious daytime spasticity or flexor spasms were not considered. When spasms prevailed at evening, night, or in supine position, patients underwent a nocturnal PSG. Patients were assessed for RLS by clinical interview and for PLM by PSG. Patients with confirmed PLM (≥15 per hour of sleep) were treated with low dosage of pramipexole (after an iron deficiency was ruled out) and reassessed by PSG the following night. Forty-five patients were included. All fulfilled RLS criteria, and PLMs were confirmed in 39 patients. Median PLM index, and related arousals were 45.9 (19.8-76.2) and 5.1 (1.5-15) events per hour respectively. Nine patients treated with pramipexole underwent an early second PSG which showed an improvement of PLM index and arousals (P=0.0007 and P=0.01, respectively). In this princeps study, we demonstrated that in SCI and MS patients, "persistent spasms" inefficiently treated by antispastic drugs could in fact be PLM. Furthermore, we first reported the efficacy of dopaminergic agonists for PLM in an MS and SCI population. This study brings new insights on abnormal movements, often misinterpreted as spasticity, and their management. We suggest to include a PSG in the diagnostic approach of uncontrolled spasms prevailing at night or in supine position, to search for PLM, which are easily treatable.

Prediction of sexual function following spinal cord injury: a case series.

Spinal Cord Injury (SCI) affects sexual response based on the level and degree of completeness of injury. By using the International Standards for the Neurologic Classification of SCI in conjunction with lumbo-sacral reflexes these effects can be predicted. The International Standards for the Assessment of Autonomic Function after SCI (ISAFSCI) document the impact of SCI on sexual responses including psychogenic and reflex arousal (erection or lubrication), orgasm, ejaculation, and sensation of menses. Responses are described based upon a 0 to 2 scale with 0 being absent, 1 altered, and 2 normal response. Additionally, the lesion is described as supraconal, conal or infraconal. We present 4 representative cases of the impact of SCI on sexual responses and course of treatment. Case 1 describes a complete supraconal lesion above T6 with upper motor neuron syndrome. Case 2 describes a supraconal complete lesion including the T11-L2 segment. Case 3 describes an infraconal (cauda equina) lesion with lower motor neuron syndrome. Case 4 is theoretical and describes a supraconal lesion above T6 with upper motor neuron syndrome, partial sensation in T11-L2 dermatomes, and other medical comorbidities. Neurologic examination combined with reflex testing allows prediction of sexual responses after SCI. It would be useful for version 2.0 of the ISAFSCI to assist clinicians in determining the anticipated changes, whether their patients are functioning as anticipated sexually after SCI or whether other concerns also require treatment.

Dartos reflex as autonomic assessment in persons with spinal cord injury.

Prospective clinical series. The dartos reflex (DR) produces unilateral elevation of the testis with vermicular contraction of the scrotal skin and penile retraction. It is a somato-autonomic reflex that depends on the T11-L2 sympathetic segment. Its presence was analysed in men with various levels and severities of spinal cord injury (SCI). France. Male patients with SCI undergoing urodynamic or sexual assessment were included. DR of the scrotum and penis were systematically assessed in addition to the usual neurological examination that included assessment of the autonomic system, cremaster, and sacral reflexes. The DR was evoked by applying a small ice pack on the scrotum, separately to each side. Forty-six patients were assessed. DRs were always present when the T11-L2 segment was intact above the lesion (low paraplegia below L2) or below the lesion in an upper motor neuron (UMN) syndrome (spastic paraplegia above T10). They were more pronounced in the case of UMN syndrome. In the case of lower motor neuron (LMN) syndrome that included the T11-L2 segment, DRs disappeared in all but one patient. Patients with a LMN lesion were more prone to retrograde or absent ejaculation, loss of psychogenic erection, and open bladder neck. The DR is a valuable test for the assessment of the integrity and excitability of the T11-L2 spinal segment. It is easy to perform and is predictive of some aspects of sexual and bladder neck function in men. DR should be considered as an addition to the autonomic standards.

A Toolkit for Objective Clinical Outcome Measures of Muscle Tone

To evaluate a wearable sensor-based toolkit for quantifying muscle tone in patients with upper motor neuron syndrome (UMNS).

Постинсультная спастичность

Spasticity is a common complication of stroke that results in impairments and limitations in performance of activities and participation. Although it is an important problem, spasticity, defined as velocity-dependent muscle overactivity, is only one component of the upper motor neuron syndrome (UMNS). It is often related to other motor impairments, such as weakness, and disordered motor control, which magnify the functional impact of spasticity. The mere presence of spasticity is not enough reason to initiate treatment. A thoughtful assessment of the clinical picture and functional impairment is the first step in establishing a management strategy, followed by treatment goal setting. Patient engagement in goal setting is an important component of a collaborative management with the clinician. Various pharmacologic and surgical interventions have been used for a different clinical situations and indications with varying outcomes. The related evidence is presented, and specific practice recommendations are provided.

Botulinum Toxin Type A Injection May Restore Ankle Strategy Use in Stroke Patients: A Preliminary Report

Introduction: Stroke causes upper motor neuron syndrome, and spasticity of foot ankle complex further leads to equine gait and posture stability disturbance. Botulinum type A (BTX-A) injection has been widely used in treating spasticity, yet few studies report its effect on posture control. This study aimed at applying BTX-A injection in treating ankle-foot complex spasticity and investigating its effect on ankle strategy use with sensory organization test. Material and method: 9 stroke patients and 5 healthy subjects were recruited. BTX-A injection was applied over calf flexor muscle group under electrical stimulation guide. Strategy score (ST) and Composite score were assessed using standard sensory organization test(SOT) with SMART Balance Master. The higher ST and Composite score indicated that the subjects used more ankle strategy. The individual test conditions were as following: EO: eyes open; EC: eyes closed; SV: swayed vision and fixed support; EOSS: eyes open and swayed support; ECSS: eyes closed and swayed support; SVSS: swayed vision and swayed support). For the patient group, SOT was performed before BTX-A injection, and at 4 weeks, 8 weeks and 12 weeks follow up. For control group, the assessments were performed once. Generalized estimating equation was implemented for data analysis, and set p<0.05 as statistical significance.

Surgical management of intractable spasticity.

Spasticity is motor disorder characterized by a velocity-dependent increase in tonic stretch reflexes (muscle tone) with exaggerated tendon jerks, resulting from hyper-excitability of the stretch reflex, as one component of the upper motor neuron syndrome. This study aimed at comparing between spinally based (dorsal rhizotomy) versus peripherally based (selective neurotomy) surgical procedures in management of hypertonia in the lower limbs of pediatrics. Over a 3-year period, 50 children with intractable, lower limb spasticity were prospectively treated by selective neurotomy (group A, 35 patients) and dorsal rhizotomy (group B, 15 patients) with 6 months' follow-up period. The operative duration was longer with dorsal rhizotomy with mean of 292.2 min versus 76.8 min with neurotomy (P = 0.001) and the hospital stay of dorsal rhizotomy was longer with mean of 6.2 days versus 1.7 days with neurotomy (P = 0.001). Muscles power exhibited significant improvement in 53.3% of the total rhizotomies (P = 0.001). Following neurotomies; muscle tone showed marked improvement in 69.3% muscles which had normal tone and 31.9% of muscles had mild spasticity (P = 0.001). The H/M ratio following dorsal rhizotomies showed marked reduction of the ratio, and the mean was 0.11 versus 0.58 preoperatively. Both neurotomies and dorsal rhizotomies were safe surgical procedures and were provided with good improvement in respect of: muscle power, severity of spasticity, patient's ambulation, gait, range of joint movement, associated pain, functional disability, and nerve excitability with no significant difference between both procedures.

A robotic device for ankle motor evaluation and rehabilitation in patients with upper motor neuron syndrome

A robotic device for ankle motor evaluation and rehabilitation in patients with upper motor neuron syndrome

TOWARDS A CLINICALLY COMPLIANT UPPER LIMB PART-TASK TRAINER IN SIMULATED LEARNING PROGRAM

Patient simulator is one of the methods physiotherapists and occupational therapists trainee use to improve their skills. The focus here is on spasticity as part of the upper motor neuron (UMN) syndrome. The rehabilitation process for patients with UMN syndrome and management of spasticity is very important because spasticity will affect function and quality of life. A rehabilitation process requires physicians, occupational therapists and physiotherapists to assess the spasticity level using clinical assessment methods. To engage directly with the patients, the clinicians should have enough skill and experience to reduce risk of injury to the patients. Thus, it is mandatory for the physiotherapists and occupational therapists trainee to go through comprehensive training before they can conduct the therapy session. This paper reveals the research urgency in therapist education tools for upper limb rehabilitation training and points out the significance of having compliance with clinical assessment scales.

Measuring task performance after acquired brain injury: Construct and concurrent validity of ‘Upper Limb Performance Analysis’

Objective: This preliminary investigation studies selected aspects of validity of the Upper Limb Performance Analysis (ULPA), an occupation-based functional upper limb (UL) measure.Methods: The study investigated the ULPA–Task Performance Mastery (ULPA-TPM) in 35 community dwelling adults with upper motor neuron syndrome following acquired brain injury and 26 healthy controls. Construct and concurrent validity of the ULPA were determined via group discrimination between adults with and without ABI; and ABI participants who were and were not referred for UL spasticity management with botulinum toxin-A injections (injected and non-injected group). Concurrent validity was examined by investigating the relationships between the ULPA and an existing functional UL measure, the Action Research Arm Test, using Spearman’s rank-order correlation.Results: Significant differences in UL performance were demonstrated between the ABI and the Control group on all ULPA sub-scales (including: Omission (z = −2.6 to −3.6, rspb = 0.37–0.48), Accuracy (z = −5.8 to −6.0, rspb = 0.78–0.82), Repetition (z = −5.1 to −5.4, rspb = 0.63–0.73) and Timing errors (z = −5.9 to −6.2, rspb = 0.77–0.88). Those in the Injected group demonstrated more task performance errors than the Non-injected group, with significant differences in Accuracy (z = −2.1 to −2.4, rspb = 0.37–0.45), Repetition (z = −2.5 to −2.1, rspb = 0.43) and Timing (z = −2.0, rspb = 0.37).Conclusions: This study demonstrated good construct and concurrent validity of the ULPA-TPM.

Botulinum toxin and its clinical aspects

Botulinum toxin (BTX), a potent neurotoxin which is produced by the bacterium Clostridium botulinum, consists of eight distinct neurotoxin serotypes referred to as (BTX type‑A [BTX‑A], B, C, D, E, F, G, H) all of which inhibit acetylcholine release at the neuromuscular junction. BTX‑A, by blocking acetylcholine release at neuromuscular junctions, accounts for its therapeutic action to relieve dystonia, spasticity, and related disorders. A wide variety of medical conditions such as bruxism, hyperhidrosis, achalasia,focal dystonia, upper motor neuron syndrome, blepharospasm, and chronic migraine are now treated with BTX. The cosmetological applications include correction of lines, creases, and wrinkling all over the face, chin, neck, and chest. Side effects are generally rare and minimal. Injections with BTX‑A are well‑tolerated. Discovery of further newer indications of this neurotoxin can enlighten the path of research in the field of neuroscience. Key words: Botulinum toxin, cosmetic uses, neurotoxin

78. Spatiotemporal changes in gait performance due to onabotulinumtoxinA injection to lower limb muscles in patients with upper motor neuron syndrome

78. Spatiotemporal changes in gait performance due to onabotulinumtoxinA injection to lower limb muscles in patients with upper motor neuron syndrome

143. Goal attainment scaling to improve current clinical practice with BoNT/A in the management of upper motor neuron syndrome in upper and lower limbs in adults

143. Goal attainment scaling to improve current clinical practice with BoNT/A in the management of upper motor neuron syndrome in upper and lower limbs in adults

Decision-Making in the Treatment of the Spastic Shoulder and Elbow: Tendon Release Versus Tendon Lengthening.

Upper extremity deformities and dysfunction are often the result of injury to the central nervous system. Upper motor neuron syndrome is a condition that arises as a result of the disruption of the upper motor neuron inhibitory pathways that frequently occurs in association with conditions such as traumatic brain injury, spinal cord injury, cerebral palsy, and cerebrovascular accident1. Damage to these pathways often leads to the constellation of symptoms that characterize this syndrome. Most notably, these symptoms include muscle weakness and diminished endurance as well as symptoms of hypertonicity, such as spasticity (muscle activation with a quick-stretch stimulus), rigidity (exaggerated response with a slow-stretch stimulus), dyssynergy or spastic co-contraction (antagonist co-activation alongside an agonist), and hyperreflexia. The presentation of this condition can be highly variable as any combination of these symptoms may be reported by the patient. The alteration of the muscular balance about the joints in the upper extremity often initially presents as a dynamic problem characterized by impaired motion. However, prolonged deformity may lead to the formation of contracture (fixed shortening)—a consequence that may impair the active or passive function of the affected limb. Patients with upper motor neuron syndrome often complain of pain. The reported prevalence of shoulder pain after a stroke has ranged from 24% to 84%2-5. The association between hemiplegia and shoulder pain is unclear, …

Computerised pinch dynamometry in the assessment of adult hand spasticity.

The hand engages with the environment through the grasp, stabilisation, manipulation and release of objects during everyday tasks, activities and routines. Upper motor neuron syndrome following acquired brain injury may negatively impact hand function, reducing strength, range of motion and motor control. It is important for clinicians to reliably measure such impacts, particularly for the impact of intervention and to monitor change in performance over time. Therefore, the aim of this study was to investigate the test-retest reliability and construct validity of Dynamic Computerised pinch Dynamometry for measuring fine hand motor performance following acquired brain injury. The Dynamic Computerised pinch Dynamometry protocol was completed by 36 community dwelling adults and 27 healthy adults using a simulated pinch and release task in lateral and pincer grip positions. Measurements were conducted over two testing occasions approximately five weeks apart. Dynamic Computerised pinch Dynamometry output was evaluated to determine the test-retest reliability and construct validity of the measure. Test-retest reliability scores using Kendall coefficient of concordance ranged from W = 0.61-0.94. Dynamic Computerised pinch Dynamometry discriminated between participants with and without acquired brain injury (z = 4.97-6.50, P < 0.05) and between the affected and non-affected hand of participants with acquired brain injury (z = 3.37-5.22, P < 0.001). Dynamic Computerised pinch Dynamometry in both lateral and pincer positions had fair to excellent test-retest reliability, and had good construct validity for discrimination between participants with and without acquired brain injury as well as between the affected and non-affected hand of participants with acquired brain injury.

Pathophysiology of spasticity: implications for neurorehabilitation.

Spasticity is the velocity-dependent increase in muscle tone due to the exaggeration of stretch reflex. It is only one of the several components of the upper motor neuron syndrome (UMNS). The central lesion causing the UMNS disrupts the balance of supraspinal inhibitory and excitatory inputs directed to the spinal cord, leading to a state of disinhibition of the stretch reflex. However, the delay between the acute neurological insult (trauma or stroke) and the appearance of spasticity argues against it simply being a release phenomenon and suggests some sort of plastic changes, occurring in the spinal cord and also in the brain. An important plastic change in the spinal cord could be the progressive reduction of postactivation depression due to limb immobilization. As well as hyperexcitable stretch reflexes, secondary soft tissue changes in the paretic limbs enhance muscle resistance to passive displacements. Therefore, in patients with UMNS, hypertonia can be divided into two components: hypertonia mediated by the stretch reflex, which corresponds to spasticity, and hypertonia due to soft tissue changes, which is often referred as nonreflex hypertonia or intrinsic hypertonia. Compelling evidences state that limb mobilisation in patients with UMNS is essential to prevent and treat both spasticity and intrinsic hypertonia.

Management of spasticity revisited

Spasticity is common after stroke and other neurological conditions and causes considerable limitations of movement, activities of daily living and participation. Interaction with other components of the upper motor neurone syndrome (UMNS) and the heterogeneity of patients' presentations together with limited tools for outcome measurement have hampered the production of randomised controlled trial data for management strategies. Specialist multi-disciplinary goal-centered management programmes are the mainstay of treatment. Pharmacological therapies have limited effect, and physical and positional management are crucial. Targeted intramuscular botulinum toxin injection is now the most popular pharmacological treatment. Intrathecal therapies also play a lesser role. A team approach and holistic assessment are essential to beneficial outcomes.

Does calf muscle spasticity contribute to postural imbalance? A study in persons with pure hereditary spastic paraparesis

ObjectivesThe contribution of spasticity to postural imbalance in patients with upper motor neuron syndrome is still unclear. This study aimed to evaluate the responses to support-surface perturbations in patients with hereditary spastic paraparesis (HSP). These patients typically suffer from bilateral spasticity with relatively preserved muscle strength of the lower limbs. Particularly toes-up rotations were expected to be destabilizing due to insufficient suppression of calf muscle stretch reflexes. MethodsParticipants were seventeen symptomatic community-dwelling patients with autosomal dominant pure HSP and seventeen healthy controls. All patients had increased muscle tone of the triceps surae (TS) but no muscle contractures. Perturbations were applied by rotating or translating a platform with increasing intensity in four sagittal-plane directions. The primary outcome was maximum intensity (‘limit of stability’) sustained without stepping or grabbing in each type of perturbation. Leg muscle tone and strength were assessed with the Modified Ashworth Scale and Medical Research Council (MRC) scale, respectively. ResultsFor toes-up perturbations, limits of stability in patients were substantially lower than in controls, which were related to TS muscle tone but not to tibialis anterior (TA) strength. Toes-down rotations were indiscriminative. For backward perturbations, patients also had lower limits of stability, unrelated to TA strength or TS muscle tone. In forward perturbations, patients with TS strength MRC 4 were less stable than patients with normal TS strength and controls. ConclusionCalf muscle spasticity and weakness differently contribute to postural imbalance in patients with HSP. This notion could have implications for the clinical management of spasticity.

Myotendinous lengthening of the elbow flexor muscles to improve active motion in patients with elbow spasticity following brain injury

The objective of this study was to evaluate the outcomes of a novel technique of fractional myotendinous lengthening of the elbow flexors in patients with volitional motor control and spastic elbow flexion deformities after brain injury. A retrospective review of 42 consecutive patients with spastic elbow flexion deformities and upper motor neuron (UMN) syndrome was performed. Each patient had volitional motor control but limited elbow extension and underwent myotendinous lengthening of the elbow flexor muscles. Outcome measures included pre and post-operative active and passive arc of motion, Modified Ashworth Scale (MAS) of spasticity, and complications. There were 26 men and 16 women. The etiologies of UMN syndrome were stroke (30 patients), traumatic brain injury (11 patients), and cerebral palsy (1 patient). Average duration between injury and surgery was 6.6 years. At an average follow-up of 14 months, improvements were noted in active extension (42° to 20°; P < .001). In addition, active arc of motion increased from 77° (range of motion [ROM]: 42° to 119°) to 113° (ROM: 20° to 133°) (P < .001) and passive arc of motion increased from 103° (ROM: 24°-127°) to 131° (ROM: 8°-139°) (P < .001). Significant improvement in MAS was also noted after surgery (2.7 to 1.9; P < .001). Superficial wound dehiscence occurred in 2 patients and was successfully treated nonoperatively. In patients with spastic elbow flexion deformities and active motor control, fractional myotendinous lengthening of the elbow flexors safely improves active extension and the overall arc of motion while affording immediate postoperative elbow motion. Level IV, Case Series, Treatment Study.

Toward an epidemiology of poststroke spasticity

Poststroke spasticity (PSS)-related disability is emerging as a significant health issue for stroke survivors. There is a need for predictors and early identification of PSS in order to minimize complications and maladaptation from spasticity. Reviewing the literature on stroke and upper motor neuron syndrome, spasticity, contracture, and increased muscle tone measured with the Modified Ashworth Scale and the Tone Assessment Scale provided data on the dynamic time course of PSS. Prevalence estimates of PSS were highly variable, ranging from 4% to 42.6%, with the prevalence of disabling spasticity ranging from 2% to 13%. Data on phases of the PSS continuum revealed evidence of PSS in 4% to 27% of those in the early time course (1-4 weeks poststroke), 19% to 26.7% of those in the postacute phase (1-3 months poststroke), and 17% to 42.6% of those in the chronic phase (>3 months poststroke). Data also identified key risk factors associated with the development of spasticity, including lower Barthel Index scores, severe degree of paresis, stroke-related pain, and sensory deficits. Although such indices could be regarded as predictors of PSS and thus enable early identification and treatment, the different measures of PSS used in those studies limit the strength of the findings. To optimize evaluation in the different phases of care, the best possible assessment of PSS would make use of a combination of indicators for clinical impairment, motor performance, activity level, quality of life, and patient-reported outcome measures. Applying these recommended measures, as well as increasing our knowledge of the physiologic predictors of PSS, will enable us to perform clinical and epidemiologic studies that will facilitate identification and early, multimodal treatment.