Upper Motor Neuron Syndrome Research Articles

Dorsal intramedullary giant dermoid tumor

Intramedullary dermoid tumors are rare benign neoplasms that correspond to 1 to 2% of all intramedullary tumors, affecting specially the lumbosacral region. Those tumors are composed of remnants of embryonic tissue derived from the ectoderm, whose walls secretions cause slow growth rate. Despite its benign character, neurological injury comes from the tumor's expansive process and the collateral damage derived from the complete resection of the cyst wall, which can cause hypoesthesia and radicular pain, besides other effects as neuromuscular scoliosis. The primary treatment for intramedullary tumor is resection surgery. The case reported involves an infant with an extensive dorsal intramedullary dermoid tumor without association with spinal dysraphism, presenting clinically complete paraplegia, lower limbs atrophy, hypoesthesia from the T4 level and urinary incontinence. The physical examination revealed upper motor neuron syndrome in the lower limbs, mild cognitive delay and a significant scoliosis. Image exams showed a Cobb angle from T3 to L2 with 115º sitting and 68º with traction, besides a massive expansive intramedullary formation extending from C5 to the T9 plane. The patient underwent extensive cervical and thoracic laminotomy followed by median myelotomy and the resection of the lesion. An arthrodesis treated the secondary vertebral deformity. The postoperative period showed good surgical recovery and the control exams revealed ample resection of the lesion and adequate control of scoliosis with partial maintenance of rotational deformity.

Presenilin-1 Mutations Are a Cause of Primary Lateral Sclerosis-Like Syndrome.

Background and PurposePrimary lateral sclerosis (PLS) is a progressive upper motor neuron (UMN) disorder. It is debated whether PLS is part of the amyotrophic lateral sclerosis (ALS) spectrum, or a syndrome encompassing different neurodegenerative diseases. Recently, new diagnostic criteria for PLS have been proposed. We describe four patients of two pedigrees, meeting definite PLS criteria and harboring two different mutations in presenilin 1 (PSEN1).MethodsPatients underwent neurological and neuropsychological examination, MRI, 18F-fluorodeoxyglucose positron emission tomography (FDG-PET), amyloid-related biomarkers, and next-generation sequencing (NGS) testing.ResultsFour patients, aged 25–45 years old, presented with a progressive UMN syndrome meeting clinical criteria of definite PLS. Cognitive symptoms and signs were mild or absent during the first year of the disease but appeared or progressed later in the disease course. Brain MRI showed microbleeds in two siblings, but iron-related hypointensities in the motor cortex were absent. Brain FDG-PET showed variable areas of hypometabolism, including the motor cortex and frontotemporal lobes. Amyloid deposition was confirmed with either cerebrospinal fluid (CSF) or imaging biomarkers. Two heterozygous likely pathogenic mutations in PSEN1 (p.Pro88Leu and p.Leu166Pro) were found in the NGS testing.ConclusionClinically defined PLS is a syndrome encompassing different neurodegenerative diseases. The NGS testing should be part of the diagnostic workup in patients with PLS, at least in those with red flags, such as early-onset, cognitive impairment, and/or family history of neurodegenerative diseases.

Ergonomic Recommendations in Ultrasound-Guided Botulinum Neurotoxin Chemodenervation for Spasticity: An International Expert Group Opinion.

Ultrasound (US)-guided botulinum neurotoxin (BoNT) injections are becoming a mainstay in the treatment of muscle spasticity in upper motor neuron syndromes. As a result, there has been a commensurate increase in US-guided BoNT injection for spasticity training courses. However, many of these courses do not emphasize the importance of ergonomics. This paper aims to highlight the importance of ultrasound ergonomics and presents ergonomic recommendations to optimize US-guided BoNT injection techniques in spasticity management. Expert consensus opinion of 11 physicians (4 different continents; representing 8 countries, with an average of 12.6 years of practice using US guidance for BoNT chemodenervation (range 3 to 22 years)). A search using PubMed, College of Physicians and Surgeons of British Columbia database, EMbase was conducted and found no publications relating the importance of ergonomics in US-guided chemodenervation. Therefore, recommendations and consensus discussions were generated from the distribution of a 20-question survey to a panel of 11 ultrasound experts. All 11 surveyed physicians considered ergonomics to be important in reducing physician injury. There was complete agreement that physician positioning was important; 91% agreement that patient positioning was important; and 82% that ultrasound machine positioning was important. Factors that did not reach our 80% threshold for consensus were further discussed. Four categories were identified as being important when implementing ultrasound ergonomics for BoNT chemodenervation for spasticity; workstation, physician, patient and visual ergonomics. Optimizing ergonomics is paramount when performing US-guided BoNT chemodenervation for spasticity management. This includes proper preparation of the workspace and allowing for sufficient pre-injection time to optimally position both the patient and the physician. Lack of awareness of ergonomics for US-guided BoNT chemodenervation for spasticity may lead to suboptimal patient outcomes, increase work-related injuries, and patient discomfort. We propose key elements for optimal positioning of physicians and patients, as well as the optimal setup of the workspace and provide clinical pearls in visual identification of spastic muscles for chemodenervation.

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

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. 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. 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). 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 IV.

Long-term course of anterior spinal cord herniation presenting with an upper motor neuron syndrome: case report illustrating diagnostic and therapeutic implications

BackgroundAnterior spinal cord herniation (aSCH) is a rare cause of myelopathy which may present as pure motor syndrome and mimic other degenerative diseases of the spinal cord. In slowly progressive cases, diagnosis may be impeded by equivocal imaging results and mistaken for evolving upper motor neuron disease. As early imaging studies are lacking, we aimed to provide a detailed description of imaging and neurophysiology findings in a patient with aSCH, focusing on the early symptomatic stages.Case presentationWe here present the case of a 51-year old male patient with an episode of pain in the right trunk and a normal spinal MRI. After a symptom-free interval of 8 years, spasticity and paresis evolved in the right leg. There was subtle ventral displacement and posterior indentation of the thoracic spinal cord on MRI which, in retrospect, was missed as an early sign of aSCH. After another 3 years, symptoms spread to the left leg and a sensory deficit of the trunk became evident. Follow-up MRI now clearly showed an aSCH. Neurosurgical intervention consisted of remobilization of the herniated spinal cord and patch closure of the dura defect. Over the following years, motor and sensory symptoms partially improved.ConclusionsThe history of this patient with aSCH illustrates the importance of careful longitudinal clinical follow-up with repeated imaging studies in progressive upper motor neuron syndromes. Specific attention should be paid to a history of truncal pain and to MRI findings of a ventrally displaced spinal cord. Neurosurgical intervention may halt the progression of herniation.

PHYSIOTHERAPISTS’ APPROACH REGARDING SPASTICITY MEASUREMENT SCALES IN PATIENTS WITH UPPER MOTOR NEURON SYNDROME

OBJECTIVE: To determine the physiotherapists’ approach regarding spasticity measurement scales in patients with upper motor neuron syndrome.METHODS: This cross-sectional study was conducted in different hospitals of Karachi from January 2018 to June 2018. One hundred physiotherapists with an interest in the neurological rehabilitation were enrolled by using convenience-sampling technique. After taking written informed consent, a questionnaire comprising of close-ended questions was distributed among the participants. Extraction sheet was used to extract the recorded response; later was analyzed using SPSS v 21.0.RESULTS: Out of 100 physiotherapists, 90 responded (response rate of 90%), where 60 (66.6%) participants were males and 30 (33.3%) were female. Fifty-five (61.11%) participants responded that they use assessment scale of tone for shoulder complex, seven (7.78%) used biomechanical approaches, five (5.56%) used neurophysiological, three (3.33%) use both biomechanical as well as neurophysiological approach for the assessment of tone in shoulder complex and twenty (22.22%) did not use any measurement scale. Out of 55 participants using any assessment scale, twenty (36.4%) used Modified Ashworth Scale (MAS), while rest of thirty-five (63.6%) used three grade muscle tone scale with different combination: mild/moderate/severe] by twenty (36.4%); flaccid/normal/spastic by five (9.1%), and +/++/+++ by ten (18.1%) participants.CONCLUSION: Majority of participants in this study use unreliable assessment tools for the assessment of tone in upper motor neuron syndrome. They need to be encouraged to use valid and reliable tools for assessment.

Recovery after traumatic thoracic- and lumbar spinal cord injury: the neurological level of injury matters.

Multicenter prospective cohort. To discern neurological- and functional recovery in patients with a traumatic thoracic spinal cord injury (TSCI), conus medullaris syndrome (CMS), and cauda equina syndrome (CES). Specialized spinal cord injury centers in Europe. Lower extremity motor score (LEMS) and spinal cord independent measure (SCIM) scores from patients with traumatic TSCI, CMS, and CES were extracted from the EMSCI database. Scores from admittance and during rehabilitation at 1, 3, 6, and 12 months were compared. Linear mixed models were used to statistically analyse differences in outcome, which were corrected for the ASIA Impairment Scale (AIS) in the acute phase. Data from 1573 individuals were analysed. Except for the LEMS in patients with a CES AIS A, LEMS, and SCIM significantly improved over time for patients with a TSCI, CMS, and CES. Irrespectively of the AIS score, recovery in 12 months after trauma as measured by the LEMS showed a statistically significant difference between patients with a TSCI, CMS, and CES. Analysis of SCIM score showed no difference between patients with TSCI, CMS, or CES. Difference in recovery between patients with a traumatic paraplegia is based on neurological (motor) recovery. Regardless the ceiling effect in CES patients, patients with a mixed upper and lower motor neuron syndrome (CMS) showed a better recovery compared with patients with a upper motor neuron syndrome (TSCI). These findings enable stratifications of patients with paraplegia according to the level and severity of SCI.

Rate of Information Flow Across Layered Neuro-Spike Network in the Spinal Cord.

Spinal Cord Injury (SCI) is a severe condition that can result in loss of motor and sensory functions by disrupting communication among neurons, i.e., neuro-spike communication. Future information and communication technology (ICT) based treatment techniques for SCI are expected to rely on nano networks, deployed inside the body. In this respect, modeling neuro-spike communication channels in the spinal cord and revealing the relationship between channel metrics and SCI are required to realize these treatment techniques and diagnosis tools such as replacement neural implants, high-performance diagnosis tools, which are based on ICT metrics instead of large medical data. Therefore, in this study, we focus on a spinal cord network, namely the descending spinal cord pathway, which is responsible for the transmission of brain motor signals to the spinal cord. We aim to analyze the rate of motor information flow to the corresponding muscle. To this end, we model the spinal cord motor network as a layered network consisting of a cascade of two independent neuro-spike channels, which are brain-spinal cord network and spinal cord interneuron-spinal cord motoneuron network. We derive upper and lower bounds for the total rate across the brain-spinal cord network and interneuron-spinal cord network. Our evaluations demonstrate that the total rate in the case of upper motor neuron syndrome (UMNS), which manifests itself with muscle weakness, approaches zero, where the brain-spinal cord network becomes a bottleneck. In lower motor neuron syndrome (LMNS), which results in muscle atrophy, the total rate again approaches zero with the loss of spinal cord motoneurons (MN).

Ghost spasticity in multiple sclerosis

Spasticity is the velocity-dependent hypertonia frequently encountered in patients affected by Upper Motor Neuron Syndrome. It is due to a tonic stretch reflex, which is evoked in patients at rest. The aim of this study, performed using surface electromyography (EMG), was to investigate stretch reflex excitability in the hamstrings muscles of patients affected by progressive Multiple Sclerosis (MS) and to correlate EMG results with clinical findings. Thirty patients and 20 age-matched healthy controls were investigated. EMG activity was recorded from biceps femoris muscle with the patient at rest. To stretch hamstrings muscles, the patient’s leg was manually moved from maximal flexion to maximal extension at 3 different velocities to investigate both phasic and tonic stretch reflex. Only 7 patients were affected by hypertonia of the hamstrings; 4 of them showed muscle contracture. A tonic stretch reflex was present in the vast majority of the recruited patients, whether they presented hypertonia of the hamstrings or not. Tonic stretch reflex is often present in the hamstrings muscles of progressive MS patients without producing increased muscle tone. This “ghost spasticity” is likely to be, for its intrinsic features, an important risk factor for the development of contractures in the hamstrings muscles.

Δ1 -Pyrroline-5-carboxylate synthetase deficiency: An emergent multifaceted urea cycle-related disorder.

The bifunctional homooligomeric enzyme Δ1 -pyrroline-5-carboxylate synthetase (P5CS) and its encoding gene ALDH18A1 were associated with disease in 1998. Two siblings who presented paradoxical hyperammonemia (alleviated by protein), mental disability, short stature, cataracts, cutis laxa, and joint laxity, were found to carry biallelic ALDH18A1 mutations. They showed biochemical indications of decreased ornithine/proline synthesis, agreeing with the role of P5CS in the biosynthesis of these amino acids. Of 32 patients reported with this neurocutaneous syndrome, 21 familial ones hosted homozygous or compound heterozygous ALDH18A1 mutations, while 11 sporadic ones carried de novo heterozygous ALDH18A1 mutations. In 2015 to 2016, an upper motor neuron syndrome (spastic paraparesis/paraplegia SPG9) complicated with some traits of the neurocutaneous syndrome, although without report of cutis laxa, joint laxity, or herniae, was associated with monoallelic or biallelic ALDH18A1 mutations with, respectively, dominant and recessive inheritance. Of 50 SPG9 patients reported, 14 and 36 (34/2 familial/sporadic) carried, respectively, biallelic and monoallelic mutations. Thus, two neurocutaneous syndromes (recessive and dominant cutis laxa 3, abbreviated ARCL3A and ADCL3, respectively) and two SPG9 syndromes (recessive SPG9B and dominant SPG9A) are caused by essentially different spectra of ALDH18A1 mutations. On the bases of the clinical data (including our own prior patients' reports), the ALDH18A1 mutations spectra, and our knowledge on the P5CS protein, we conclude that the four syndromes share the same pathogenic mechanisms based on decreased P5CS function. Thus, these syndromes represent a continuum of increasing severity (SPG9A < SPG9B < ADCL3 ≤ ARCL3A) of the same disease, P5CS deficiency, in which the dominant mutations cause loss-of-function by dominant-negative mechanisms.

The effectiveness and safety of extracorporeal shock wave therapy (ESWT) on spasticity after upper motor neuron injury: A protocol of systematic review and meta-analysis.

Spasticity is one of the manifestations of motor dysfunction in upper motor neuron syndrome, which is characterized by increased muscle tone. Spasticity seriously affects the motor function and activity of daily life of patients. Some studies have shown that extracorporeal shock wave therapy (ESWT) can relieve spasticity in recent years. However, the effectiveness and safety of ESWT on spasticity after motor neuron injury have not been confirmed. The purpose of this systematic review (SR) is to evaluate the effectiveness and safety of ESWT on spasticity after upper motor neuron injury. We will search China National Knowledge Infrastructure (CNKI), the Chinese Science and Technology Periodical Database (VIP), Wan Fang Data, China Biology Medicine (CBM), PubMed, Embase, The Cochrane Library, and Web of Science systematically from their inception dates through October 2019 to obtain randomized controlled trials (RCTs) using ESWT to relieve spasticity in patients after upper motor neuron injury. The primary outcome will be the Modified Ashworth Scale (MAS). Secondary outcomes will include Composite Spasticity Scale (CSS), Spasm Frequency Scale, Modified Tardieu Scale (MTS), electrophysiological study (ratio of maximum H reflex to maximum M response, root mean square value, integrated electromyogram, co-contraction ratio, etc.), or other spasticity-related outcomes. In addition, adverse events will also be assessed as safety measurement. Study selection, data extraction, and quality assessment will be performed independently by 2 reviewers. Assessment of risk of bias and data synthesis will be performed using Review Manager software (RevMan, version 5.3.5) and R (version 3.6.1) software. We will synthesize current studies to evaluate the effectiveness and safety of ESWT on spasticity after upper motor neuron injury. Our study will provide evidence of ESWT on spasticity after upper motor neuron injury. The ethical approval is not required since SR is based on published studies. The results of this SR will be published in a peer-reviewed scientific journal according to the Preferred Reporting Item for Systematic Review and Meta-analysis (PRISMA) guidelines. CRD42019131059.

Gait-related Changes in Ankle Plantar Flexor Muscle Tone during Movement in Upper Motor Neuron Syndrome

Gait-related Changes in Ankle Plantar Flexor Muscle Tone during Movement in Upper Motor Neuron Syndrome

Upper limb robotic assessment: Pilot study comparing velocity dependent resistance in individuals with acquired brain injury to healthy controls.

IntroductionAssessment of velocity dependent resistance (VDR) can provide insights into spasticity in individuals with upper motor neuron syndrome. This study investigates the relationship between Modified Ashworth scores and a biomechanical based representation of VDR using a rehabilitation robot. Comparisons in VDR are made for the upper limb (UL) between individuals with acquired brain injury and healthy controls for the para-sagittal plane.MethodsThe system manipulates the individual’s limb through five flexion and extension motions at increasing speeds to obtain force profiles at different velocities. An approximation of VDR is calculated and analyzed statistically against clinical scales and tested for interactions.ResultsAll individuals (aged 18–65), including healthy controls exhibited VDR greater than 0 (P < 0.05). MAS scores were found to be related to VDR (P < 0.05) with an interaction found between MAS Bicep and Tricep scores (P < 0.01). Considering this interaction, evidence of differences in VDR were found between several neighboring assessment score combinations.ConclusionThe robot can detect and quantify VDR that captures information relevant to UL spasticity. Results suggests a better categorization of VDR is possible and supports further development of rehabilitation robotics for assisting spasticity assessment.

Pain and Muscles Properties Modifications After Botulinum Toxin Type A (BTX-A) and Radial Extracorporeal Shock Wave (rESWT) Combined Treatment.

Spasticity (most common disability in upper motor neuron syndrome or UMNS) caused an inability of patients' to perform daily activities and a decrease inquality of life. One of the promising methods nowadays, but still not widely used in everyday practice, for spasticity reduction is extracorporeal shock wave. The aim of this study was to evaluate the objective clinical effects of combined treatment botulinum toxin type A and radial Extracorporeal Shock Wave Therapy in spasticity post stroke. We considered 30 subjects (14 female and 16 male) with post stroke spasticity of Biceps Brachii, Superficial Flexor Digitorum, Gastrocnemius Medialis and Lateralis and we divided patients into two groups (group A received botulinum toxin injection and physiotherapy while group B received botulinum toxin injection, rESWT and physiotherapy). Assessments were performed before treatment (t0), after 1 (t1), 2 (t2) e 3 (t3) months using Modified Ahworth Scale, Visual Analogical Scale for pain and MyotonPro® device (to assessed myometric evaluation of muscles tone and stiffness). Visual Analogical Scale, Modified Ahworth Scale, muscles tone and stiffness statistically decreased until t3 in the group A and in the group B, but the differences between the two groups were significant at the t1 only. Radial Extracorporeal Shock Wave Therapy could be an effective physical treatment aimed at the reduction of upper and lower limbs spasticity and could lead to the improvement of trophic conditions of the spastic muscles in post-stroke.

Spastic dystonia in stroke subjects: prevalence and features of the neglected phenomenon of the upper motor neuron syndrome

ObjectiveSpastic dystonia is one of the positive phenomena of the upper motor neuron syndrome (UMNS). It is characterised by the inability to relax a muscle leading to a spontaneous, although stretch-sensitive, tonic contraction. Although spastic dystonia is a recognized cause of muscle hypertonia, its prevalence among hypertonic muscles of stroke subjects has never been investigated. Differently from spasticity, which is an exaggerated stretch reflex, spastic dystonia is viewed as an efferent phenomenon, due to an abnormal central drive to motoneurons. MethodsIn 23 hemiparetic stroke subjects showing increased muscle tone of wrist flexors, surface EMG was used to investigate the presence of spontaneous, stretch-sensitive EMG activity in flexor carpi radialis. ResultsSpontaneous, stretch-sensitive EMG activity was found in 17 subjects. In the remaining 6 subjects, no spontaneous EMG activity was found. ConclusionsThe majority of stroke subjects is affected by spastic dystonia in their hypertonic wrist flexor muscles. Only a minority of subjects is affected by spasticity. SignificanceTo stop spastic dystonia from being the neglected aspect of UMNS, it is essential to link its definition to increased muscle tone, as occurred for spasticity. Recognizing the real phenomena underling muscle hypertonia could improve its management.

The Use of Botulinum Toxin for Treatment of Spasticity.

Spasticity is one component of the upper motor neuron (UMN) syndrome resulting from a multitude of neurologic conditions, such as stroke, brain injury, spinal cord injury, multiple sclerosis, and cerebral palsy. It is clinically recognized as a phenomenon of velocity-dependent increase in resistance, i.e., hypertonia. Recent advances in the pathophysiology of spasticity improve our understanding of mechanisms underlying this complex phenomenon and its relations to other components of UMN syndrome (weakness and disordered motor control), as well as the resultant clinical problems. This theoretical framework provides a foundation to set up treatment goals and to guide goal-oriented clinical assessment and treatment. Among a spectrum of treatment options, botulinum toxin (BoNT) therapy is the preferred treatment for focal spasticity. The evidence is very robust that BoNT therapy effectively reduces spasticity; however, it does not improve voluntary movement. In this chapter, we highlight a few issues on how to achieve the best clinical outcomes of BoNT therapy, such as dosing, dilution, guidance techniques, adjunctive therapies, early treatment, repeated injections, and central effects, as well as the ways to improve motor function in selected subgroups of patients with spasticity. We also discuss the reasons of poor responses to BoNT therapy and when not to use BoNT therapy.

Cerebrospinal Fluid Neurofilaments May Discriminate Upper Motor Neuron Syndromes: A Pilot Study

Background: Patients presenting with upper motor neuron (UMN) signs may widely diverge in prognosis, ranging from amyotrophic lateral sclerosis (ALS) to primary lateral sclerosis (PLS) and hereditary spastic paraplegia (hSP). Neurofilaments are emerging as potential diagnostic and prognostic biomarkers for ALS, but the diagnosis of UMN syndromes still relies mostly on clinical long-term observation and on familiarity or genetic confirmation. Objectives: To test whether phosphorylated neurofilament heavy chain (pNfH) may discriminate different UMN syndromes at diagnosis and to test their prognostic role among these diseases. Methods: We measured the cerebrospinal fluid (CSF) and serum pNfH of 30 patients presenting with UMN signs and diagnosed with ALS, hSP, and PLS, plus 9 healthy controls (HC). Results: ALS patients had higher levels of pNfH in CSF and serum compared to HC (p < 0.001 and p < 0.001 in CSF and serum, respectively) and PLS (p = 0.015 and p = 0.038) and hSP (p = 0.003 and p = 0.001) patients. PLS and hSP patients had similar CSF and serum pNfH concentrations, but a higher CSF pNfH concentration, compared to HC (p = 0.002 and p = 0.003 for PLS and hSP, respectively). Receiver operating characteristic curves for discriminating ALS from PLS and hSP showed an area under the curve of 0.79 for CSF and 0.81 for serum. In multivariable survival analysis including relevant clinical factors CSF pNfH represented the strongest variable predicting survival (HR 40.43; 95% CI 3.49–467.79, p = 0.003) independently of clinical group. Conclusions: Despite some statistical instability of the results due to limitations in sample size, our study supports the role of CSF pNfH as a prognostic biomarker for motor neuron diseases presenting with UMN signs. A potential power to discriminate between ALS and other UMN syndromes at presentation, and between all of the examined MND and HC, has been detected for both CSF and serum pNfH.

Upper Limb After Effects of an Upper Motor Neuron Syndrome

Upper Limb After Effects of an Upper Motor Neuron Syndrome

Evaluation of a toolkit for standardizing clinical measures of muscle tone

Objective: To evaluate a new portable toolkit for quantifying upper and lower extremity muscle tone in patients with upper motor neuron syndrome (UMNS). Approach: Cross-sectional, multi-site, observational trial to test and validate a new technology. Setting: Neurorehabilitation clinics at tertiary care hospitals. Participants: Four cohorts UMNS patient, >6 mo post acquired brain injury, spinal cord injury, multiple sclerosis and cerebral palsy, and a sample of healthy age-matched adult controls. Measures: Strength: grip, elbow flexor and extensor, and knee extensor; range of motion (ROM): passive ROM (contracture) and passive-active ROM (paresis); objective spasticity: stretch-reflex test for elbow, and pendulum test for knee; subjective spasticity: modified Ashworth scale scores for elbow and knee flexors and extensors. Results: Measures were acquired for 103 patients from three rehabilitation clinics. Results for patient cohorts were consistent with the literature. Grip strength correlated significantly with elbow muscle strength and all patient populations were significantly weaker in upper- and lower-extremity compared to controls. Strength and paresis were correlated for elbow and knee but neither correlated with contracture. Elbow spasticity correlated with strength and paresis but not contracture. Knee spasticity correlated with strength, and subjective spasticity correlated with contracture. Significance: The BioTone™ toolkit provided comprehensive objective measures for assessing muscle tone in patients with UMNS. The toolkit could be useful for standardizing outcomes measures in clinical trials and for routine practice.

A historical review of the evolution of the Tardieu Scale

ABSTRACTThere are many clinical assessment tools that can be used to quantify spasticity, one feature of the Upper Motor Neurone (UMN) syndrome. The focus of this short paper is on three; the Tardieu Scale, the Modified Tardieu Scale and the Australian Spasticity Assessment Scale, because a fundamental concept of these tests is their velocity dependent nature. Other bedside assessments such as the Modified Ashworth Scale examine hypertonicity, another feature of the UMN syndrome, but in this instance, the stretching movement is not velocity dependent. The Tardieu Scale, while not officially named until 1997, was conceived in the 1950s and since that time it has been revised by multiple authors and it is these additions that will be discussed in this article. The advantages and disadvantages of these assessment tools will be discussed with the ultimate aim of identifying one that has greater clinical utility.