Motor Neuron Lesions Research Articles

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.

Amyotrophic lateral sclerosis with respiratory failure and dysautonomia: a case report.

A 58-year-old male presented with left lower limb limping, difficulty climbing stairs, and left foot weakness, followed by right upper limb weakness and was diagnosed with ALS and received edaravone and riluzole treatment. He presented again with right lower limb weakness, shortness of breath, and wide fluctuations in blood pressure, leading to ICU admission with new diagnosis of ALS with dysautonomia with respiratory failure and was managed with non-invasive ventilation, physiotherapy, and gait training exercises. ALS is a progressive neurodegenerative disease affecting motor neurons but non-motor symptoms can also occur, including dysautonomia, which can result in blood pressure fluctuations. Dysautonomia in ALS is caused by several mechanisms such as severe muscle atrophy, prolonged ventilatory support, and upper and lower motor neuron lesions. Management of ALS involves giving a definitive diagnosis, providing nutritional support, using disease-modifying drugs such as riluzole and non-invasive ventilation to improve survival and quality of life. Early diagnosis is essential for effective management of the disease. Early diagnosis, use of disease-modifying drugs, non-invasive ventilation, and maintaining the patient's nutritional status are crucial for managing ALS which can have non-motor symptoms as well.

A -2- month old child with Spinal Muscular Atrophy Type 1 with Pneumonia: A case report

Spinal muscular atrophy (SMA) is a autosomal recessive disease characterized by muscular weakness, hypotonia and atrophy. SMA is caused by mutation or deletion of the survivor motor neuron gene (SMN1), which is located in the telomere region of chromosome 5q13. Incidence of the disease is 1:6000-10000 newborn. Confirmatory diagnosis can be established by molecular genetic analysis. Here a 2- month- old female baby was admitted into the department of pediatrics of Sylhet Women’s Medical College Hospital with because of less movement of whole body for 1 month and repeated cough with respiratory distress for same duration. Baby was dyspneic, chest in drawing and crepitation was present over both lung fields. Signs of lower motor neuron lesions were present. It was confirmed by Polymerase chain reaction (PCR) for SMN gene. Final diagnosis was Spinal muscular atrophy with pneumonia. Symptomatic, Supportive and antibiotic treatment were given. Genetic counseling, regular respiratory follow up was advised.

Electrical stimulation in lower motoneuron lesions, from scientific evidence to clinical practice: a successful transition.

Long pulse stimulation in its application in everyday clinical practice still represents a challenge for many therapists and clinicians. It is often unclear how the intervention setup, in particular the parameters pulse width, frequency and amplitude, can influence muscle morphology. In addition, the cause of damage to the lower motoneuron can have multiple reasons and is not anatomically located at the same site. Given the large heterogeneity, it is essential to know the current options and limitations in order to carry out a targeted treatment. A retrospective data analysis of n=128 patients, seen at the Swiss Paraplegic Centre (SPC) in 2022, shows a broad variability in manifestation of lower motoneuron damage. Treatment examples based on different causes of lower motoneuron damage are shown and corresponding stimulation programmes are assigned, as well as the expected results in terms of stimulation duration, volume and configuration.

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

<p><strong>Background</strong>: 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.</p><p><strong>Objectives</strong>: To discuss a case of polycythemia vera with multiple episodes of ischemic stroke.</p><p><strong>Case description</strong>: 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.</p><p><strong>Conclusion</strong>: 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<strong> </strong>follow up are in need to increase awareness of future thrombotic events or leukemic transformations.</p>

Etiology of spastic foot drop among 16 patients undergoing electrodiagnostic studies: patient series.

Differentiating foot drop due to upper motor neuron (UMN) lesions from that due to lower motor neuron lesions is crucial to avoid unnecessary surgery or surgery at the wrong location. Electrodiagnostic (EDX) studies are useful in evaluating patients with spastic foot drop (SFD). Among 16 patients with SFD, the cause was cervical myelopathy in 5 patients (31%), cerebrovascular accident in 3 (18%), hereditary spastic paraplegia in 2 (12%), multiple sclerosis in 2 (12%), chronic cerebral small vessel disease in 2 (12%), intracranial meningioma in 1 (6%), and diffuse brain injury in 1 (6%). Twelve patients (75%) had weakness of a single leg, whereas 2 others (12%) had bilateral weakness. Eleven patients (69%) had difficulty walking. The deep tendon reflexes of the legs were hyperactive in 15 patients (94%), with an extensor plantar response in 9 patients (56%). Twelve patients (75%) had normal motor and sensory conduction, 11 of whom had no denervation changes of the legs. This study is intended to raise awareness among surgeons about the clinical features of SFD. EDX studies are valuable in ruling out peripheral causes of foot drop, which encourages diagnostic investigation into a UMN source for the foot drop.

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.

Spastic dystonia: Still a valid term.

Hypertonia in childhood may arise because of a variable combination of neuronal and non-neuronal factors. Involuntary muscle contraction may be due to spasticity or dystonia, which represent disorders of the spinal reflex arch and of central motor output respectively. Whilst consensus definitions for dystonia have been established, definitions of spasticity vary, highlighting the lack of a single unifying nomenclature in the field of clinical movement science. The term spastic dystonia refers to involuntary tonic muscle contraction in the context of an upper motor neuron (UMN) lesion. This review considers the utility of the term spastic dystonia, exploring our understanding of the pathophysiology of dystonia and the UMN syndrome. An argument is advanced that spastic dystonia is a valid construct that warrants further exploration. WHAT THIS PAPER ADDS: There is no single universally accepted definitions for terms commonly used to describe motor disorders. Spasticity and dystonia are phenomenologically and pathophysiologically distinct entities. Spastic dystonia represents a subset of dystonia, but with pathophysiological mechanisms more in common with spasticity.

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.

Abstract Number ‐ 226: Pontine Stroke Presenting as 1.5 Syndrome Evolving to 9 Syndrome over 72 Hours.

Introduction A 75 year old woman with a past medical history of hypertension developed sudden‐onset vertigo, nausea and vomiting. She did not immediately seek medical care, thinking she was suffering from food poisoning. The following day her symptoms improved however she developed new right sided weakness and gait imbalance, prompting her presentation to the ED. Her neurologic exam noted complete loss of adduction of the right eye and loss of horizontal extraocular movements of the left eye. She also displayed right upper extremity paresis, right upper extremity dysmetria and mild dysarthria. MRI of the brain disclosed acute pontine infarcts in the ventral and dorsal left paramedian regions. Over the next three days, her horizontal gaze palsy improved with some return of adduction of the right eye and horizontal movements of the left eye. However right sided paresis worsened, and in addition, progressed to include complete left facial palsy (lower motor neuron lesion). She was able to be discharged to rehab, placed on Aspirin 81 mg daily and Ezetimibe (due to elevated liver function enzymes) and 30 day Cardiac Event Monitor follow up. Methods Case report and review of literature. Results Due to the combination of multiple cranial nuclei and white matter tracts in the brainstem, ischemia can lead to several clinical manifestations. There are various presenting clinical syndromes, named numerically by structures involved and/or the clinical deficit. One‐and‐a‐half (1.5) syndrome, first reported by Fischer in 1967, is an uncommon entity caused by a lesion (most commonly infarction) in the pontine tegmentum [1]. It consists of complete ipsilateral horizontal gaze palsy (due to involvement of ipsilateral Paramedian Pontine Reticular Formation/Abducens Nucleus and Medial Longitudinal Fasciculus) and loss of adduction of the contralateral eye (due to involvement of the contralateral Medial Longitudinal Fasciculus). Extension of the lesion to the facial nucleus can produce ipsilateral lower motor neuron type facial palsy, leading to an even more uncommon 8.5 syndrome (7 + 1.5), described by Eggenberger in 1998 [2]. When the lesion involves the corticospinal tracts and/or the medial lemniscus, it can lead to contralateral hemiparesis and/or hemisensory deficits; the so called “9 syndrome” first proposed by Rosini et al in 2013 [3]. This rare entity has been reported in only a handful of cases. Conclusions The awareness of complex brainstem neuroanatomy enables precise localization of the clinical deficits. The noted slow progression of the clinical syndrome from 1.5 to 8.5 and finally 9 syndrome over 72 hours in this case highlights the complexity of small vessel brainstem ischemia.

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).

Spinal Stenosis with Sacral Osseous Deformity Mimicking Chronic Inflammatory Demyelinating Polyneuropathy

BACKGROUND: Differential diagnoses of neurosurgical spinal disorders and polyneuropathies have been recognized to cause clinical perplexity, occasionally misdiagnosing chronic inflammatory demyelinating polyneuropathy (CIDP). When nerve conduction studies and cerebrospinal fluid (CSF) analyses reinforce a certain clinical presentation, the importance of imaging studies, conservative treatment response, and interdisciplinary clinical approach should be highly emphasized. CASE PRESENTATION: We report a 51-year-old patient who presented with a 16-week history of neurogenic claudication and right-sided lower extremity monoparesis, with low back pain syndrome dating from 10 years ago. He was initially evaluated by a neurologist under the suspicion of CIDP, supported by nerve conduction studies and CSF analyses, without any subjective or objective improvements after systemic corticosteroid therapy. After performing magnetic resonance imaging (MRI) of the lumbosacral spine, he was referred to a neurosurgeon. Neurological examination revealed features of lower motor neuron lesion, consistent with the MRI findings of L4-L5 and L5-S1 stenosis with right-sided S1 vertebra osseous deformity, without any radiographic evidence of CIDP. The patient underwent surgery and improvements were noted early in the post-operative recovery phase and continuously throughout the regular monthly follow-ups, without any clinical features of CIDP. Histopathology results confirm sacral osseous deformity. No evidence of CIDP, osseous deformity residue, or recurrence was evident on the post-operative MRI control performed 11-month post-surgery. CONCLUSIONS: Degenerative spinal stenosis compromising spinal canal dimensions can mimic CIDP due to sharing multiple clinical similarities. That scenario is especially highlighted when age-related spinal degenerative disease is unexpected and seldom aggravated by spinal osseous lesions. Avoiding misdiagnosis and providing adequate treatment can pose a serious challenge for neurosurgeons and neurologists, demonstrating the importance of an interdisciplinary approach toward diverse spinal disorders.

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.

Вопросы ранней диагностики и своевременной коррекции дыхательных нарушений при боковом амиотрофическом склерозе (обзор литературы)

Amyotrophic lateral sclerosis (ALS) is a rare severe progressive neurodegenerative disease with upper and lower motor neuron lesions, manifested by weakness of somatic muscle tissue with disorder of vital functions and leading to early disability and fatal outcome of patients. Regardless of the disease form, patients with ALS experience breathing and swallowing disorders. Hypercapnic respiratory failure (HRF) most commonly develop in patients as a consequence of respiratory muscle weakness. Instrumental diagnostic methods are currently used along with the assessment of the HRF clinical picture in ALS. Pulse oximetry and spirometry are the basic means of assessing the respiratory system function. Cardio-respiratory monitoring and arterial blood gas test play an auxiliary role in the diagnosis. To correct respiratory disorders, respiratory support is used with the help of artificial ventilation devices connected through various masks (non-invasive) or through a tracheostomy (invasive). In recent years, it has been proven that early diagnosis and correction of respiratory disorders contribute to prolonging the life of patients with ALS and improving its quality. The review presents the main development mechanisms of respiratory disorders in ALS, clinical and instrumental methods of their diagnosis, methods of correction, as well as criteria for the start of respiratory support. KEYWORDS: amyotrophic lateral sclerosis, respiratory disorders, lung ventilation, pulmonary support, neurodegeneration, NIV, ALV. FOR CITATION: Ermilov E.A., Isaeva N.V. Issues concerning early diagnosis and timely correction of respiratory disorders in amyotrophic lateral sclerosis (literature review). Russian Medical Inquiry. 2023;7(8). (in Russ.). DOI: 10.32364/2587-6821-2023-7-8-4.

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.

Urological Aspects of Spinal Dysraphism.

Micturition requires complex interplay involving bladder, peripheral neural network, spinal cord, and brain. Spinal dysraphism presents variety of neural lesions that may affect this interplay leading to neurogenic bladder. However, the diagnosis of neurogenic bladder in those with spinal dysraphism is often difficult and contrasted to other types of neurogenic bladder caused by complete neural lesions such as spinal cord injury or brain tumor. Typically, neurogenic bladder caused by spinal dysraphism shows lower motor neuron lesion and partial neural injury. However, upper motor signs can be seen with the occurrence of tethered cord syndrome and developmental immaturity of bladder control often complicated by fecal impaction. Thus, the diagnosis of tethered cord syndrome should be made cautiously. Several invasive and noninvasive diagnostic modalities could be applied for accurate diagnosis, preventing renal damage and controlling urinary incontinence. However, it should be borne in mind that no single study is definitive for accurate diagnosis, so it requires careful scrutiny in interpretation. The follow-up scheme of these patients should be determined to detect urological deterioration due to the development of tethered cord syndrome. Since the development of tethered cord syndrome shows diverse nature implicating urological and orthopedic issues, multidisciplinary collaboration is essential for comprehensive care.

Chapter 2 - Upper and lower motor neuron neurophysiology and motor control

This chapter considers the principles that underlie neurophysiological studies of upper motor neuron or lower motor neuron lesions, based on an understanding of the normal structure and function of the motor system. Human motor neurophysiology consists of an evaluation of the active components of the motor system that are relevant to volitional movements. Relatively primitive motor skills include locomotion, much dependent on the spinal cord central pattern generator, reaching, involving proximal and distal muscles activation, and grasping. Humans are well prepared to perform complex movements like writing. The role of motor cortex is critical for the motor activity, very dependent on the continuous sensory feedback, and this is essential for adapting the force and speed control, which contributes to motor learning. Most corticospinal neurons in the brain project to brainstem and spinal cord, many with polysynaptic inhibitory rather than excitatory connections. The monosynaptic connections observed in humans and primates constitute a specialized pathway implicated in fractional finger movements. Spinal cord has a complex physiology, and local reflexes and sensory feedback are essential to control adapted muscular contraction during movement. The cerebellum has a major role in motor coordination, but also consistent roles in sensory activities, speech, and language, in motor and spatial memory, and in psychological activity. The motor unit is the final effector of the motor drive. The complex interplay between the lower motor neuron, its axon, motor end-plates, and muscle fibers allows a relevant plasticity in the movement output.

Neural mechanisms underlying peripheral facial nerve palsy: A protocol for systematic review and meta-analysis of neuroimaging studies

Peripheral facial nerve palsy is a general term used to describe diseases that cause lower motor neuron lesions in the facial nerve. The most common cause of peripheral facial nerve palsy is idiopathic Bell's palsy (BP), accounting for 60% to 70% of all peripheral facial nerve palsy cases. Idiopathic BP is found in various medical conditions, such as ischemia, viral infection, and neuroinflammation; however, the specific pathophysiology remains unclear. Neuroimaging techniques help understand the neural mechanisms underlying various neurological disorders. In recent years, several studies have applied functional neuroimaging to investigate alterations of brain function in BP patients. However, the results have been inconsistent due to differences in methodology and quality. Moreover, systematic reviews of studies on functional neuroimaging for BP are insufficient. Therefore, the present study aims to verify the neural mechanisms of brain alterations and draw conclusions about the functional reorganization in patients with BP. This review includes all suitable studies published on or before October 31, 2022. A thorough search will be conducted using the following databases: MEDLINE, Cochrane Library, Excerpta Medica Database (EMBASE), China Knowledge Resource Integrated Database (CNKI), Korean Medical database (KMBASE), Korean Studies Information Service System (KISS). Clinical studies of BP using functional neuroimaging will be selected. We will apply a coordinate-based meta-analysis because most individual neuroimaging studies provide their results as coordinates in the standard space. The primary outcomes will include the types of functional neuroimaging methods and alterations of brain function in BP patients. The secondary outcomes will include the information about clinical measurement of the disease. Study selection, data extraction, and risk of bias assessment will be conducted. If possible, heterogeneity tests, data synthesis, and subgroup analyses will be conducted. The study will analyze the alterations in brain activity and worsening of clinical symptoms caused by idiopathic BP. The aim of this study is to investigate functional reorganization of brain alterations in patients with BP. This review will improve the understanding of the neural mechanisms of BP based on the most recent publications through extensive data retrieval. If sufficient data are collected, a sensitivity analysis is performed to verify the robustness of the conclusions.

Surgical quadriceps lengthening can reduce quadriceps spasticity in chronic stroke patients. A case-control study.

BackgroundMuscle overactivity is one of the positive signs of upper motor neuron lesions. In these patients, the loss of muscle length and extensibility resulting from soft tissue rearrangement has been suggested as a contributing cause of muscle overactivity in response to stretching.ObjectiveTo assess the effects of surgical lengthening of the quadriceps femoris (QF) muscle-tendon unit by aponeurectomy on muscle spasticity.MethodsThis is a case-control study on chronic stroke patients with hemiparesis that have undergone lower limb functional surgery over a 8-year period. CASEs underwent corrective surgery for both the foot and knee deviations, inclusive of a QF aponeurectomy. Controls (CTRLs) underwent corrective surgery for foot deviations only. QF spasticity was assessed with the Modified Tardieu Scale (MTS) before and 1 month after surgery. The Wilcoxon test was used to assess MTS variations over time and the Mann–Whitney test was used to verify the presence of group differences at the 1 month mark.ResultsNinety-three patients were included: 57 cases (30F, 1–34 years from lesion) and 36 controls (12F, 1–35 years from lesion). Before surgery, both CASEs and CTRLs had similar MTS scores (median MTS = 3) and functional characteristics. One month after surgery, QF spasticity was significantly lower in the CASEs compared to CTRLs (p = 0.033) due to a significant reduction of the median MTS score from 3 to 0 in the CASE group (p < 0.001) and no variations in the CTRL group (p = 0.468). About half of the cases attained clinically significant MTS reductions and complete symptom relief even many years from the stroke.ConclusionsFunctional surgery inclusive of QF aponeurectomy can be effective in reducing or suppressing spasticity in chronic stroke patients. This is possibly a result of the reduction in neuromuscular spindle activation due to a decrease in muscle shortening, passive tension, and stiffness.