Upper Motor Neuron Lesion Research Articles

Dyke-Davidoff-Masson Syndrome in a Tanzanian patient: a case report

BackgroundDyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological disorder resulting from an insult to the developing brain in utero or during early life which is characterized by refractory seizures and a wide variety of deficits. Due to its rarity, the condition is often overlooked although a detailed history and imaging can help distinguish it from other similar conditions and provide early diagnosis.Case presentationA 30-year-old African female presented with a history of recurrent seizures and intellectual disability. She was born via vaginal delivery, and her post-natal history was unremarkable. Her developmental milestones were normal until the age of three, when she was hospitalized for two weeks due to a febrile illness accompanied by generalized seizures, from which she fully recovered. On cognitive assessment, she scored 14 out of 60 on the Ravens Standard Progressive Matrices (RSPM), indicating an intellectual disability. Neurological examination revealed constructional apraxia, an upper motor neuron lesion (UMNL) affecting the facial nerve, and left-sided spastic paresis. Additionally, a slow, sinuous writhing movement involving all the fingers of her left hand was observed. A computed tomography (CT) scan of her head confirmed a diagnosis of Dyke-Davidoff-Masson syndrome (DDMS). The scan showed significant volume loss in the right cerebral hemisphere, gross dilation of the right lateral ventricle with ex-vacuo dilatation, right calvarial thickening, and hyperpneumatization of the right frontal sinus. She was managed conservatively with anticonvulsants, which effectively controlled her seizures.ConclusionIn the setting of recurrent seizures, intellectual disability, hemiparesis, or facial asymmetry, a clinician should have a high index of suspicion for DDMS. Late diagnosis is often related to intractable seizures to anticonvulsants, necessitating hemispherectomy, which is not a readily available option in limited resource settings.

The Efficiency of Binaural Beats on Anxiety and Depression—A Systematic Review

Anxiety and depression are two of the most impactful diseases on quality of life and cause significant disability to patients. It burdens the medical system even as a stand-alone pathology or a secondary condition. These conditions can occur secondarily after a patient suffers from other illnesses, such as upper motor neuron lesions. Binaural beats are a new and emerging type of technology that can be used as an adjunct therapy for anxiety and depression as well as in neurologic conditions to some extent. We searched through the MEDLINE, PsychINFO, EMBASE, CENTRAL, ISRCTN, and ICTRP databases to identify studies using binaural beats as therapy for anxiety and depression. Twelve articles were declared eligible to be included in this review. Binaural beats, whether used in the form of pure beats or masked by another sound, have shown better results in alleviating symptoms of anxiety and depression compared to control conditions such as no music or the use of noise-canceling headphones alone. The results suggest that using binaural beats could be a promising and easy-to-use method to help alleviate the symptoms of anxiety and depression.

Frequency and Clinical Outcomes of Facial Nerve Palsy at District Level Teaching Hospital, Pakistan

Background: Facial nerve palsy, characterized by the dysfunction of the seventh cranial nerve, is a common neurological disorder that significantly impacts patients' quality of life. It manifests as unilateral facial weakness or paralysis, with Bell's palsy being the most prevalent cause. This condition requires thorough clinical evaluation and appropriate management to ensure optimal recovery outcomes. Objective: The objective of this study was to determine the frequency and clinical outcomes of facial nerve palsy in patients presenting to a district-level teaching hospital in Lakki Marwat, Pakistan. Methods: A cross-sectional study was conducted from May 2023 to October 2023 at the Physiotherapy Department of District Headquarter Hospital, Lakki Marwat. Patients with a confirmed diagnosis of facial nerve palsy by a neurophysician were included, while those with congenital facial nerve palsy or upper motor neuron lesions were excluded. Ethical approval was obtained, and written informed consent was collected from all participants. Clinical assessments were performed using the House-Brackmann scale to evaluate the severity of facial nerve dysfunction. Data were collected through a semi-structured questionnaire and analyzed using SPSS version 25. Descriptive statistics, including frequencies, percentages, means, and standard deviations, were used to present the data. Results: Out of 102 patients, 59 (57.9%) were male, and 43 (42.1%) were female, with a mean age of 38.2 ± 10.36 years. The majority of patients (41.1%) were in the 31-40 years age group. Right-side facial palsy was more prevalent, affecting 64 (62.7%) patients. The House-Brackmann grading revealed that 49 (48.2%) patients were in Grade IV, 24 (23.5%) in Grade V, 15 (14.7%) in Grade III, 8 (7.8%) in Grade VI, and 6 (5.8%) in Grade II. Bell’s palsy was the most common cause, accounting for 52 (51%) cases, followed by external trauma in 22 (21.6%) patients, infections in 18 (17.6%) patients, and tumors in 10 (9.8%) patients. Treatment outcomes showed complete recovery in 65 (63.7%) patients, incomplete recovery in 28 (27.5%), and no recovery in 9 (8.8%). Conclusion: Facial nerve palsy was more common in male patients, with the right side of the face being predominantly affected. Bell’s palsy was identified as the leading cause. The study highlights the importance of early diagnosis and intervention, particularly physiotherapy, in managing facial nerve palsy. Further research is recommended to explore long-term outcomes and optimize treatment strategies.

Are tactile function and body awareness of the foot related to motor outcomes in children with upper motor neuron lesions?

Somatosensory function can be reduced in children with Upper Motor Neuron (UMN) lesions. Therefore, we investigated relationships between somatosensory functions of the foot and motor outcomes in children with UMN lesions. In this cross-sectional study, we assessed the Tactile Threshold (TT) with monofilaments and body awareness with Tactile Localisation Tasks for spatial-related action (TLTaction) and structural-related perception (TLTperception) body representation at the foot sole. Furthermore, we assessed four motor outcomes: the Selective Control Assessment of the Lower Extremity (SCALE), the modified Timed Up and Go test (mTUG), the Gillette Functional Assessment Questionnaire (FAQ), and the Functional Mobility Scale (FMS). Spearman's correlations (ρ) were applied to assess relationships between the somatosensory function of the foot sole and the applied motor outcomes. Thirty-five children with UMN lesions, on average 11.7 ± 3.4 years old, participated. TLTperception correlated significantly with all lower limb motor outcomes (|ρ|=0.36-0.57; p < 0.05), but TLTaction (|ρ|=0.00-0.27; p = 0.15-0.97, and TT did not (|ρ|=0.01-0.83; p = 0.73-0.94). TLTperception correlated strongly with the Gross Motor Function Classification System (|ρ|=0.62; p = 0.001) in children with cerebral palsy (n = 24). Assessing structural body representation of the foot sole should be considered when addressing lower limb motor impairments, including gait, in children with upper motor neuron lesions. Our results suggest that the assessment of tactile function and spatial body representation may be less related to lower limb motor function.

Electromyographic findings directing the diagnosis of an upper motor neuron lesion of the hypoglossal nerve

A 45-year-old woman presented with a subacute, intermittent deviation of the tongue to the right side, and dysarthria. Symptoms worsen and become more evident when the patient attempts to speak for a prolonged period of time. Neurological examination

Assessing proprioception in children with upper motor neuron lesions: feasibility, validity, and reliability of the proprioception measurement tool.

To investigate the feasibility, discriminative and convergent validity, and reliability of a lower limb sensor-based proprioception measure in children with upper motor neuron (UMN) lesions. We assessed three proprioception modalities (joint movement, joint position, and dynamic position sense) of the lower limbs in 49 children with UMN lesions and 50 typically developing (TD) peers (5-19 years). Forty-three children with UMN lesion had a congenital and six an acquired brain lesion and 82% were able to walk without a walking aid. We evaluated the feasibility, compared the test results between children with UMN lesions and TD peers, and calculated Spearman correlations (rs) between the modalities. We quantified relative reliability with Intra-Class Correlation Coefficients (ICC) and absolute reliability with Smallest Detectable Changes (SDC). Most children with UMN lesions (>88%) found the tests easy to perform. The children with UMN lesions had significantly (p < 0.001) lower proprioceptive function than the TD children. The correlation between the three proprioceptive modalities was moderate to high (0.50 ≤ rs ≤ 0.79). The relative reliability for test-retest and the inter-rater reliability was moderate to high (ICCs = 0.65-0.97), and SDC was between 2° and 15°. The three tests are feasible, and discriminative and convergent validity and reliability were confirmed. Further studies should investigate the influence on motor function and performance in children with UMN lesions.

Évaluation du tonus musculaire chez les patients ayant subi une lésion cérébrale acquise : une revue systématique

ObjectiveThis study determined which muscle tone assessment scale has the best psychometric properties in patients with acquired brain injury. MethodsThe systematic review included cross-sectional studies of diagnostic accuracy in which the sample consisted of adult patients with acquired brain injury of various etiologies, and the outcomes were the psychometric properties of muscle tone rating scales. The main databases consulted were PubMed, Cochrane Central Register of Controlled Trials and PEDro. Risk of bias and applicability were assessed using QUADAS-2, a specific tool for diagnostic studies. The characteristics of each study were presented in a synaptic table, and the results were described schematically and narratively. ResultsThe search yielded 1056 results, of which only 13 were included in the systematic review after full-text analysis. The population of nine studies involved adult patients with upper motor neuron lesions of various etiologies, while in four studies the sample analyzed consisted of children with cerebral palsy. With regard to spasticity, the Ashworth scale and the modified Ashworth scale demonstrated low to moderate inter-operator reliability and low validity. The modified Ashworth scale, on the other hand, demonstrated high inter-operator reliability in the assessment of upper limb districts. The modified Tardieu scale was found to have low to moderate inter-operator reliability and moderate to high intra-operator reliability, with greater concordance of scores than the modified Ashworth scale. The Hypertonia Assessment Tool, the Barry-Albright Dystonia Scale, the Unified Dystonia Assessment Scale and the Burke-Fahn-Marsden Movement Scale, studied on samples of children with cerebral palsy, showed moderate to good inter-operator reliability and good internal consistency. ConclusionThe systematic review identified a series of muscle tone assessment scales and analyzed the psychometric properties of each tool.

An interesting case of crossed syndrome: ipsilateral facial paralysis with contralateral glossoplegia

BackgroundStroke is rarely accompanied with peripheral facial paralysis and supranuclear palsy of the hypoglossal nerve. Both sides of the motor cortex innervate the hypoglossal nucleus; therefore, unilateral lesions of the upper motor neurons rarely result in contralateral lingual paresis. We report a rare case of crossed syndrome with associated hyperacute peripheral hemifacial paralysis and contralateral lingual paresis after a lower pontine tegmentum ischemic stroke.Case presentation: A 73-year-old man presented with symptoms of hyperacute peripheral hemifacial paralysis. Upon protrusion, the patient’s tongue deviated to the contralateral side, without fasciculation or atrophy. Brain imaging showed focal ischemic stroke in the pontine tegmentum. However, lingual hemiparesis and multimodal neuroimaging findings differed.ConclusionsWe suggest that cortico-hypoglossal fibers pass through the dorsal pontine. This case of crossed syndrome is a rare report of a lower pontine tegmentum ischemic stroke resembling an upper motor neuron lesion of the contralateral hypoglossal nerve.

Delphi consensus study and clinical practice guideline development for functional electrical stimulation to support upright mobility in people with an upper motor neuron lesion.

A Clinical Practice Guideline (CPG) is required to provide guidance on optimal service delivery for Functional Electrical Stimulation (FES) to support upright mobility in people living with mobility difficulties due to an upper motor neuron lesion, such as stroke or multiple sclerosis. A modified Delphi consensus study was used to provide expert consensus on best practice. A Steering Group supported the recruitment of an Expert Panel, which included a range of stakeholders who participated in up to three survey rounds. In each round, panelists were asked to rate their agreement with draft statements about best practice using a 6-point Likert scale and add free text to explain their answer. Statements that achieved over 75% agree/strongly agree on the Likert scale were included in the CPG. Those that did not were revised based on free text comments and proposed in the next survey round. The first round included 82 statements with seven substatements. Sixty-five people (84% response rate) completed survey round 1 leading to 62 statements and four substatements being accepted. Fifty-six people responded to survey round 2, and consensus was achieved for all remaining statements. The accepted statements are included within the CPG and provide recommendations about who can benefit from FES and how they can be optimally supported through FES service provision. As such the CPG will support advocacy for, and optimal design of, FES services.

CHECGAIT: A Functional Electrical Stimulation Clinical Pathway to Reduce Foot Drop during Walking in Adult Patients with Upper Motor Neuron Lesions.

Foot drop during the swing phase of gait and at initial foot contact is a current kinematic abnormality that can occur following an upper motor neuron (UMN) lesion. Functional electrical stimulation (FES) of the common peroneal nerve through an assistive device is often used in neuro-rehabilitation to help patients regain mobility. Although there are FES-specific guideline recommendations, it remains a challenge for clinicians to appropriately select patients eligible for the daily use of FES devices, as very few health insurance systems cover its cost in Europe. In Luxembourg, since 2018, successfully completing an FES clinical pathway called CHECGAIT is a prerequisite to receiving financial coverage for FES devices from the national health fund (Caisse Nationale de Santé-CNS). This study describes the structure and steps of CHECGAIT and reports our experience with a cohort of 100 patients enrolled over a three-year period. The clinical and gait outcomes of all patients were retrospectively quantified, and a specific analysis was performed to highlight differences between patients with and without an FES device prescription at the end of a CHECGAIT. Several significant gait differences were found between these groups. These results and CHECGAIT may help clinicians to better select patients who can most benefit from this technology in their daily lives. In addition, CHECGAIT could provide significant savings to public health systems by avoiding unnecessary deliveries of FES devices.

Unmasking the Mystery of A Hidden Syrinx: Case Report of Progressive Paralysis in A 20-Year-Old Male with Intact Pain and Temperature Sensations

Syringomyelia is a progressive neurological disorder in which a fluid-filled cyst known as syrinx forms within the spinal cord. The case of a 20-year of adult is presented with lower limb stiffness and difficulty in walking for last 3 years. For the last 1 year, he also developed stiffness of upper limbs. Upon examination, he had a spastic gait with no ataxia. There was muscle wasting of both upper and lower limbs, with fasciculation. Tone was increased with reduced power and hyperreflexia bilaterally in all four limbs with positive ankle clonus and upgoing plantars bilaterally. Sensory system, cerebellum and higher motor functions were intact. MRI scan revealed an abnormal intramedullary multifocal T2WI hyper intense signal in cervical and thoracic spinal cord consistent with syrinx and he was diagnosed with Syringomyelia. This case is an uncommon presentation of Syringomyelia presenting with predominantly upper motor neuron lesion without sensory involvement

Quantitative assessment of spasticity: a narrative review of novel approaches and technologies.

Spasticity is a complex neurological disorder, causing significant physical disabilities and affecting patients' independence and quality of daily lives. Current spasticity assessment methods are questioned for their non-standardized measurement protocols, limited reliabilities, and capabilities in distinguishing neuron or non-neuron factors in upper motor neuron lesion. A series of new approaches are developed for improving the effectiveness of current clinical used spasticity assessment methods with the developing technology in biosensors, robotics, medical imaging, biomechanics, telemedicine, and artificial intelligence. We investigated the reliabilities and effectiveness of current spasticity measures employed in clinical environments and the newly developed approaches, published from 2016 to date, which have the potential to be used in clinical environments. The new spasticity scales, taking advantage of quantified information such as torque, or echo intensity, the velocity-dependent feature and patients' self-reported information, grade spasticity semi-quantitatively, have competitive or better reliability than previous spasticity scales. Medical imaging technologies, including near-infrared spectroscopy, magnetic resonance imaging, ultrasound and thermography, can measure muscle hemodynamics and metabolism, muscle tissue properties, or temperature of tissue. Medical imaging-based methods are feasible to provide quantitative information in assessing and monitoring muscle spasticity. Portable devices, robotic based equipment or myotonometry, using information from angular, inertial, torque or surface EMG sensors, can quantify spasticity with the help of machine learning algorithms. However, spasticity measures using those devices are normally not physiological sound. Repetitive peripheral magnetic stimulation can assess patients with severe spasticity, which lost voluntary contractions. Neuromusculoskeletal modeling evaluates the neural and non-neural properties and may gain insights into the underlying pathology of spasticity muscles. Telemedicine technology enables outpatient spasticity assessment. The newly developed spasticity methods aim to standardize experimental protocols and outcome measures and enable quantified, accurate, and intelligent assessment. However, more work is needed to investigate and improve the effectiveness and accuracy of spasticity assessment.

Is functional electrical stimulation effective in improving walking in adults with lower limb impairment due to an upper motor neuron lesion? An umbrella review.

To conduct an umbrella review of systematic reviews on functional electrical stimulation (FES) to improve walking in adults with an upper motor neuron lesion. Five electronic databases were searched, focusing on the effect of FES on walking. The methodological quality of reviews was evaluated using AMSTAR2 and certainty of evidence was established through the GRADE approach. The methodological quality of the 24 eligible reviews (stroke, n = 16; spinal cord injury (SCI), n = 5; multiple sclerosis (MS); n = 2; mixed population, n = 1) ranged from critically low to high. Stroke reviews concluded that FES improved walking speed through an orthotic (immediate) effect and had a therapeutic benefit (i.e., over time) compared to usual care (low certainty evidence). There was low-to-moderate certainty evidence that FES was no better or worse than an Ankle Foot Orthosis regarding walking speed post 6 months. MS reviews concluded that FES had an orthotic but no therapeutic effect on walking. SCI reviews concluded that FES with or without treadmill training improved speed but combined with an orthosis was no better than orthosis alone. FES may improve quality of life and reduce falls in MS and stroke populations. FES has orthotic and therapeutic benefits. Certainty of evidence was low-to-moderate, mostly due to high risk of bias, low sample sizes, and wide variation in outcome measures. Future trials must be of higher quality, use agreed outcome measures, including measures other than walking speed, and examine the effects of FES for adults with cerebral palsy, traumatic and acquired brain injury, and Parkinson's disease.

Recurrent Ischemic Stroke In Elderly With Polycythemia Vera: Case Report

&lt;p&gt;&lt;strong&gt;Background&lt;/strong&gt;: Myeloproliferative disorders can rise a risk of thrombotic events. Ischemic stroke is one of presenting symptoms can be found in such patients. These coexisting conditions can burden patients with more severe complications.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Objectives&lt;/strong&gt;: To discuss a case of polycythemia vera with multiple episodes of ischemic stroke.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Case description&lt;/strong&gt;: The patient was a 70-year-old female with chief complaint of sudden rotatory dizziness with moderate to severe intensity. Nausea, vomiting, slurred speech, and facial muscle weakness were also reported. Onset of symptoms started 3 hours before hospital admission. Medical history including previous ischemic stroke in 2020 and 2015 with remaining right extremity weakness. Physical examination showed weakness, hypertension. Nystagmus horizontal bidirectional and upper motor neuron lesion of left CN VII and XII, weakness in right upper and lower extremities were observed. Physiological reflexes were increased, and pathological reflex was found. Head CT showed hypodense lesion led to her ischemic stroke diagnosis. Blood test showed increased hemoglobin count, leukocytosis but normal platelet count. Increased cell numbers and variations of size, morphology and distribution were found in peripheral smear. This finding was also supported with bone marrow examination which showed hypercellularity, panmyelosis with dysplasia of all hematopoietic lineage. Patient was diagnosed with polycythemia vera and treated accordingly.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion&lt;/strong&gt;: Further examination of hematology, such as peripheral smear or bone marrow examination should be considered in patients with recurrent episodes of stroke to rule in underlying myeloproliferative disorders.Appropriate treatment and routine hematology&lt;strong&gt; &lt;/strong&gt;follow up are in need to increase awareness of future thrombotic events or leukemic transformations.&lt;/p&gt;

Posterior mediastinal Ewing sarcoma with multiple metastases on FDG PET/CT: a rare entity

BackgroundEwing sarcoma and peripheral primary neuroendocrine tumors are aggressive neoplasms which consist of small, round, blue cells of neuroectodermal origin. They usually arise from the skeleton and consist of genetic mutations EWSR1 in chromosome 22 and FL1 gene on chromosome 11. Extraskeletal Ewing sarcomas (EES) are rare entities with most common sites of EES being extremities, head and neck region and retroperitoneum. Posterior mediastinal Ewing sarcoma is rare. For its evaluation, 18F-fluorodeoxyglucose positron emission tomography (18F FDG PET/CT) plays significant role in staging, management planning and prognostication.Case presentationWe describe a rare case of EES of posterior mediastinum in a 20-year-old boy who presented with signs of upper motor neuron lesion below D10 level. Contrast-enhanced magnetic resonance imaging (CEMRI) showed a heterogeneously enhancing posterior mediastinal mass in pre- and paravertebral region with intraspinal extension in D2-D4 levels. Fluorodeoxyglucose PET/CT showed a metabolically active mass occupying the superior, middle and posterior mediastinum on the left, displacing the trachea and esophagus toward the right side and causing complete collapse of the left lung. Posteriorly the mass was seen destroying the D2-D5 vertebrae with intraspinal extension at D2-D4 level. Metabolically active metastatic disease was seen in pleura, skull, D12 vertebra, right iliac bone and bilateral proximal femorae. Biopsy obtained from lung and adjacent pleura showed features of a round cell tumor positive for NKX 2.2, weak positive for FLI 1 and negative for PAN CK, LCA, Vimentin and TLE-1, suggestive of Ewing sarcoma. Based on these investigations, a diagnosis of EES of posterior mediastinum was made.ConclusionExtraskeletal Ewing sarcoma of posterior mediastinum is a rare and aggressive entity. Management of metastatic EES comprises radiotherapy and systemic chemotherapy which reduces tumor burden and micrometastasis. However, response to treatment in metastatic EES is poorer than in localized disease with overall 5-year survival rates of less than 30%. Fluorodeoxyglucose PET/CT can be a useful tool to accurately detect the extent of local disease in the presence of atelectasic lung for radiotherapy planning as well as evaluating response to therapy.

Feasibility, Validity, and Reliability of Lower Limb Tactile and Body Awareness Assessments in Children With Upper Motor Neuron Lesions

ObjectiveTo investigate the feasibility, discriminative and convergent validity, and inter-rater reliability of a lower limb tactile function and 2 body awareness assessments in children with upper motor neuron (UMN) lesions. DesignCross-sectional psychometric study. SettingPediatric rehabilitation center. ParticipantsForty individuals with UMN lesions (mean age 11.7 years, SD 3.4 years; 27 girls) and 40 neurotypically developing children of the same age participated (N=80). InterventionsNot applicable. Main Outcome MeasuresWe assessed the tactile threshold (TT) with monofilaments and body awareness with tactile localization tasks (TLTs) for structural (TLTaction) and spatial (TLTperception) body representation at the foot sole. We compared the test outcomes between children with UMN lesions and neurotypically developing children with the Wilcoxon signed-rank test. Furthermore, we quantified the relations between the 3 tests with Spearman correlations (rs) and the interrater reliability with quadratic weighted kappa (κQW). ResultsAbout 80% of the children with UMN lesions perceived the tests easy to perform. The children with UMN lesions had significantly reduced somatosensory function compared with the neurotypically developing children. For the more affected leg, we found good relations between the TT and the TLTaction (rs=0.71; P<.001) and between the 2 TLTs (rs=0.66; P<.001), and a fair relation between the TT and the TLTperception (rs=0.31; P=.06). The inter-rater reliability analyses for the sum scores showed almost perfect agreement for the TT (κQW more affected leg 0.86; less affected leg 0.81), substantial agreement for TLTaction (κQW more affected leg 0.76; less affected leg 0.63), and almost perfect agreement for TLTperception (κQW more affected leg 0.88; less affected leg 0.74). ConclusionThe 3 tests are feasible to assess lower limb somatosensory function in children with UMN lesions. Discriminative and convergent validity and reliability of the 3 tests were confirmed. Further studies should investigate responsiveness and association with motor function of these outcome measures.

Phenol vs. botulinum toxin A injection for managing lower limb spasticity in adult patients with upper motor lesions: A randomized clinical trial

Background: Phenol and botulinum toxin type A (BTX-A) injections are two options for treating spasticity with the ability to select a specific spastic muscle and determine the dosage based on spasticity degree. This study intends to compare the efficacy of BTX-A vs. phenol blockade in treating lower limb spasticity and to evaluate the performance improvement in gross motor functional outcomes among adult patients with upper motor neuron (UMN) lesions. Methods: This randomized, double-blind clinical trial of 28 spastic lower limb adult patients with UMN was diagnosed between March 1, 2017, to April 30, 2019. Patients were randomized in a 1:1 ratio to a “BTX-A injections” or a “Phenol injections” group. The outcomes were measured through assessment spasticity by the Modified Ashworth Scale (MAS), active range of motion (AROM) of lower limb joint by a goniometer, Verbal Rating Scale (VRS), Visual Analog Scale (VAS), and Penn Spasm Frequency Scale (PSFS) as a baseline and post-injection follow-up at 24 hours, 3 weeks, and 3 months. Results: All 28 randomized patients were analyzed. No significant difference between the two study arms, neither in demographic characteristics nor in MAS, AROM, VRS, VAS, and PSFS parameters prior to the procedures. AROM showed a significant decrease from baseline throughout the study in the phenol group. While in the BTX-A group, they improved significantly at 3 weeks; no more improvement was observed at 3 months, and the differences were statistically significant (p &lt; 0.05). The reduction in MAS, VRS, VAS, and PSFS was statistically significant in each group at 24 hours, 3 weeks, and 3 months after the injection (p &lt; 0.05). However, the differences were not significant between the phenol and BTX-A groups (p &gt; 0.05), except for PSFS at the 3 months of follow-up in the Phenol group (p = 0.01). The need for re-injection at 6 months and 9 months was that 5 patients vs. 0 patients (p = 0.01) and 8 patients vs. 3 patients (p = 0.04) in the BTX-A and phenol groups, respectively, were statistically significant. Conclusion: Phenol injection showed superior treatment effects in AROM, decreased spasm degree based on PSFS at 3 months, and less-frequent re-injection rate compared to BTX-A injections in adult patients with UMN lesions. However, both phenol and BTX-A injections effectively reduce spasticity without significant differences in efficacy and adverse effects. Future studies must be conducted with a longer duration of follow-up, and larger sample sizes better to compare both drugs’ effectiveness and side effects. Trial registration: The study protocol was registered as a clinical trial under registration IRCT20170826035914N2 at the Iranian Registry of Clinical Trials (http://www.irct.ir).

The cutaneous silent period as a measure of upper motor neuron dysfunction in amyotrophic lateral sclerosis

ObjectivesWe investigated the cutaneous silent period (CutSP) as a measure of upper motor neuron (UMN) dysfunction in amyotrophic lateral sclerosis. MethodsThe onset latency, duration, and amount of EMG suppression of the CutSP were compared with clinical UMN signs in 24 patients with amyotrophic lateral sclerosis (ALS). UMN signs were quantified using a clinical index and transcranial magnetic stimulation (TMS). Central motor conduction time (CMCT), cortical motor threshold and motor evoked potential amplitudes were assessed as measures of UMN dysfunction. CutSP was studied in abductor digit minimi (ADM) and tibialis anterior (TA) EMG recordings following stimulation of the 5th finger and sural nerves respectively. Non-parametric tests and binomial logistic regression were applied to evaluate the data. ResultsCutSP onset latency was increased in ALS patients, compared to healthy controls, both for ADM and TA muscles. In limbs with clinical UMN signs or abnormal TMS findings, the CutSP onset latency was particularly increased. There was a significant positive correlation between CutSP onset latency and the UMN score in both upper and lower limbs. In TA muscles there was also a negative correlation between CutSP onset latency and EMG suppression. The logistic regression model based on CutSP parameters correctly classified more than 70% of the cases regarding the presence of clinical signs of UMN lesion, in both upper and lower limbs. The results were not significant for TMS. ConclusionWe conclude that upper limb CutSP changes associates with UMN lesion in ALS. This neurophysiological measurement merits further investigation in ALS.

Automatic Identification of Involuntary Muscle Activity in Subacute Patients with Upper Motor Neuron Lesion at Rest—A Validation Study

Sustained involuntary muscle activity (IMA) is a highly disabling phenomenon that arises in the acute phase of an upper motor neuron lesion (UMNL). Wearable probes for long-lasting surface EMG (sEMG) recordings have been recently recommended to detect IMA insurgence and to quantify its evolution over time, in conjunction with a complex algorithm for IMA automatic identification and classification. In this study, we computed sensitivity (Se), specificity (Sp), and overall accuracy (Acc) of this algorithm by comparing it with the classification provided by two expert assessors. Based on sample size estimation, 6020 10 s-long sEMG epochs were classified by both the algorithm and the assessors. Epochs were randomly extracted from long-lasting sEMG signals collected in-field from 14 biceps brachii (BB) muscles of 10 patients (5F, age range 50-71 years) hospitalized in an acute rehabilitation ward following a stroke or a post-anoxic coma and complete upper limb (UL) paralysis. Among the 14 BB muscles assessed, Se was 85.6% (83.6-87.4%); Sp was 89.7% (88.6-90.7%), and overall Acc was 88.5% (87.6-89.4%) and ranged between 78.6% and 98.7%. The presence of IMA was detected correctly in all patients. These results support the algorithm's use for in-field IMA assessment based on data acquired with wearable sensors. The assessment and monitoring of IMA in acute and subacute patients with UMNL could improve the quality of care needed by triggering early treatments to lessen long-term complications.

Botulinum neurotoxin-A in a patient with post-stroke spasticity: a neurophysiological study.

Post-stroke spasticity (PSS) is a disorder of the sensory-motor control, leading to upper motor neuron lesions manifesting either as intermittent or sustained involuntary activation of muscles. Botulinum neurotoxin-A (BoNT-A) is mostly utilized in a variety of therapeutic indications, and it is effective and safe in the management of focal PSS in the rehabilitation scenario. The study aimed to evaluate the effect of BoNT-A administration on H-reflex of upper and lower limbs following PSS. In addition, the investigation of the association among the degree of spasticity (assessed by the Modified Ashworth Scale [MAS]) and motor neuron pool excitability (assessed by analysing H-reflex excitability) was done. Fifty patients with a stroke of either sex aged 30 to 60 years presented with either upper or lower limb focal spasticity were studied. BoNT-A was given on two occasions to the gastrocnemius, soleus, biceps brachii muscles and flexor carpi radialis (FCR). H-reflex was documented from the FCR and soleus muscles at baseline and 3-4 weeks post BoNT-A injection. Medical Research Council scale and MAS were used to assess the PSS and muscle strength. H-reflex latency and amplitude, H/M ratio recorded from FCR and soleus muscles were significantly different between pre- and post-management. The MRC scale was significantly increased whereas the MAS was significantly reduced post BoNT-A injection. BoNT-A causes obvious improvement in PSS clinically as assessed by MAS and MRC scale as well as neurophysiologically by H-reflex. A negative correlation between H-reflex latency but not the amplitude or H max /M max ratio and MAS was observed.