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Recent evidence indicates that turning difficulty may correlate with trunk control; however, surface electromyography has not been used to explore trunk muscle activity during turning after stroke. This study investigated trunk muscle activation patterns during standing turns in healthy controls (HCs) and patients with stroke with turning difficulty (TD) and no TD (NTD). The participants with stroke were divided into two groups according to the 180° turning duration and number of steps to determine the presence of TD. The activation patterns of the bilateral external oblique and erector spinae muscles of all the participants were recorded during 90° standing turns. A total of 14 HCs, 14 patients with TD, and 14 patients with NTD were recruited. The duration and number of steps in the turning of the TD group were greater than those of the HCs, independent of the turning direction. However, the NTD group had a significantly longer turning duration than did the HC group only toward the paretic side. Their performance was similar when turning toward the non-paretic side; this result is consistent with electromyographic findings. Both TD and NTD groups demonstrated increased amplitudes of trunk muscles compared with the HC groups. Their trunk muscles failed to maintain consistent amplitudes during the entire movement of standing turns in the direction that they required more time or steps to turn toward (i.e., turning in either direction for the TD group and turning toward the paretic side for the NTD group). Patients with stroke had augmented activation of trunk muscles during turning. When patients with TD turned toward either direction and when patients with NTD turned toward the paretic side, the flexible adaptations and selective actions of trunk muscles observed in the HCs were absent. Such distinct activation patterns during turning may contribute to poor turning performance and elevate the risk of falling. Our findings provide insights into the contribution and importance of trunk muscles during turning and the association with TD after stroke. These findings may help guide the development of more effective rehabilitation therapies that target specific muscles for those with TD.

Aim:The etiology of mastalgia is not fully understood. We evaluated the relationship between electromyography (EMG) measurements of the pectoralis major (PM) muscle and mastalgia in patients with non-cyclic unilateral mastalgia.Methods:Thirty-one patients who were admitted to the breast surgery outpatient clinic due to the complaints of intermittent or continuous unilateral non-cyclic mastalgia for at least one month were included in the study. Data on age, family history, age of menarche, body mass index (BMI), physical examination, breast ultrasonography and mammography findings and amplitudes, latency and cutaneous silent period (CSP) in EMG of the right and left PM muscles were noted.Results:The mean age of the patients was 43.3 (20-65) years; mastalgia affected the left breast and the right breast in 22 (71%) and nine (29%), respectively. Twenty-three patients (74.1%) had a BMI of ≥25 kg/m2. There was a size difference between the two breasts in eleven patients. The EMG results of PM muscles in healthy and mastalgia sides were compared and there was no significant difference observed between the amplitude and resistance parameters. The mastalgia side had significantly higher CSP values.Conclusion:Prolonged CSP suggests that unilateral mastalgia may be associated with pain modulating mechanisms of the central nervous system.

To characterize electromyographic (EMG) findings in patients with primary lateral sclerosis (PLS) during the disease course.In PLS patients we scored spontaneous activity and motor unit action potential (MUP) pattern on EMG. We compared patients according to lower (group A) and higher (group B) EMG scores. EMG studies were repeated at intervals longer than 11 months; two or three repeat studies were required for inclusion in the analysis.We studied 22 patients. Fasciculation potentials were found in 13 and fibrillations/positive sharp waves (fibs/sw) in 3 patients. Both were stable over time. Most patients had MUP abnormalities (n = 17), with worsening in the lower limbs in patients with three evaluations (p = 0.010). Compared to group A (n = 12), patients of group B (n = 10) had a significant shorter disease duration (median 10.9 vs 15.2 years, p 0.001), lower functional score at both first (39 vs 45, p = 0.034) and last (29 vs 38, p = 0.003) evaluations, and had a faster functional decline (0.19 vs 0.08, p = 0.004).Most PLS patients showed minor and stable EMG abnormalities, without progression to ALS. Patients with more EMG abnormalities have a faster progression.EMG abnormalities in most PLS patients are minor and stable.

Guillain–Barre Syndrome and JEV Infection Japanese encephalitis virus infection was confirmed in 161 patients in a northern area of Ningxia, China. Signs and symptoms and electromyographic findings...

Diagnosis of amyotrophic lateral sclerosis (ALS) is based on combination of clinical signs and electrophysiological correlates of pathological process which takes place in general. New electrophysiological criteria Awaji-Shima (AS) additionally qualify the complex fasciculations and neurogenically modified potentials of motor units as signs of active lesions of peripheral motor neuron, contrary to previously valid revised El Escorial criteria (rEE). The objective of this research was to determine the clinical significance and advantages of using the AS criteria in patients with ALS. Methods. Thirty patients (59.2 ± 10.9 years, 57% of them with spinal form of the disease) with clinically suspected ALS were monitored from the time of diagnosis until reaching the category of definitive diagnosis or death. The clinical evaluation and electromyographic (EMG) examinations were carried out at 3-month intervals. Results. By applying the AS criteria, the category of probable or definite diagnosis was achieved in all patients with ALS, except in one (96.6%), as contrary to the rEE (33.3%), after 6 months of the follow-up period. The subclinical affection in more than two body regions has been defined through detection of denervation potentials (80% of the patients by using the AS, or 67% by the rEE criteria). The complex fasciculations were registered particularly often in small muscles of the feet (37- 40%). Conclusion. Application of the AS criteria improve the achievment of category of probable or definite diagnosis of ALS by 2.7 months earlier compared to the rEE. This outcome is particularly affected by a higher frequency of positive EMG findings, when the AS criteria were employed. Early determination of diagnosis provides the better perspective and more frequent participation of the ALS patients in pharmacotherapy studies intended to establish new therapeutic options. Dijagnoza amiotrofične lateralne skleroze (LS) zasniva se na kombinaciji kliničkih znakova i elektrofizioloških korelata patološkog procesa koji se odvija u osnovi. Novi elektrofiziološki kriterijumi, Awaji-Shima (AS), dodatno kvalifikuju kompleksne fascikulacije i neurogeno izmenjene potencijale motornih jedinica kao znakove aktivnih lezija perifernog motornog neurona, nasuprot ranije važećim revidiranim El Escorial kriterijumima (rEE). Cilj ovog istraživanja bio je da se utvrdi klinički značaj i prednosti primene AS kriterijuma, kod obolelih od ALS. Metode. 30 bolesnika (59,2 ± 10,9 godina, od kojih 57% sa spinalnom formom bolesti) sa klinički suspektnom ALS, praćeno je od vremena postavljanja dijagnoze do postizanja kategorije definitivne dijagnoze ili smrtnog ishoda. Kod bolesnika su obavljane klinička evaluacija i elektromiografski (EMG) pregledi u tromesečnim intervalima. Rezultati. Primjenom AS kriterijuma, nakon 6 meseci praćenja, kategorija verovatne ili pouzdane dijagnoze postignuta je kod svih bolesnika sa ALS, izuzev jednog (96,6%), nasuprot rEE kriterijuma (33.3%). EMG nalazi bili su pozitivni u ≥ 2 telesna regiona kod 80% bolesnika primenom AS, odnosno 67% primenom rEE. Kompleksne fascikulacije zabeležene su posebno učestalo u malim mišićima stopala (37-40%). Zaključak. Primenom AS kriterijuma kategorija vjerovatne ili pouzdane dijagnoze ALS postiže se za 2,7 meseca ranije, u poređenju sa rEE, na šta posebno utiče veća učestalost pozitivnih EMG nalaza. Ranije utvrđivanje dijagnoze donosi perspektivu veće zastupljenosti obolelih u farmakoterapijskim studijama sa novim terapeutskim agensima.

Objective The detailed characteristics and prognosis of nonsurgery-related unilateral vocal fold paralysis (NSUVFP) are currently unclear. This study evaluated the extent of laryngeal nerve lesions and the individual characteristics for patients with NSUVFP. Study Design Retrospective, case series. Methods Patients with unilateral vocal fold paralysis (UVFP) were evaluated using videolaryngostroboscopy and quantitative laryngeal electromyography. The side of nerve lesions, involvement of the external branch of the superior laryngeal nerve (eSLN), and complete vocal fold motion recovery were evaluated after ≥ 6 month follow-up. Results A total of 207 UVFP patients were recruited, including 153 surgery-related UVFP and 54 NSUVFP patients. Thirty-four (63%) and 20 (37%) NSUVFP patients were further assigned to idiopathic and nonsurgery-related–nonidiopathic (NSNI) groups, respectively. In the idiopathic group, eSLN lesions occurred in all six (100%) patients with right-side paralysis, but in only six of 28 (21%) patients with left-side paralysis (P 0.05). Conclusion Idiopathic and NSNI UVFP have different clinical presentations defined by laryngeal electromyography. NSNI UVFP had more severe denervation changes compared with idiopathic UVFP. These results may support two pathogenic mechanisms for idiopathic UVFP: 1) neuropathy specifically involving left recurrent laryngeal nerve (RLN), and 2) neuropathy affecting nerves proximal to the RLN. Level of Evidence 4. Laryngoscope, 2016

Calf pseudohypertrophy due to radiculopathy is an exceptional phenomenon rarely described. We report a 67 year old woman with a previous history of lumbar disc surgery consulting by progressive increase for more than a year of evolution painless right calf associated loss of strength. Electromyographic findings showed chronic S1 radiculopathy and radiologically was appreciated in the medial gastrocnemius and soleus rights substitution of normal muscle tissue by adipose tissue without evidence of myopathy or sarcomatous degeneration.

Aims. In this study we analyzed the degree of genetic homozygosity among spina bifida patients with different degrees of neurogenic lesion (N = 82), as well as their clinical and neurological characteristics, compared to healthy control individuals (N = 100). Methods. According to clinical and electromyographic findings, we separately assessed the type of neurogenic lesion (paresis or paralysis). Regarding the degree of neurogenic lesion, patients were classified into three groups: mild, moderate and severe. We analyzed six muscles. For assessing the degree of individual genetic homozygosity, we tested the presence and distribution of 15 homozygous recessive characteristics (HRC). Results. The predominant type of neurogenic lesion was paresis. Every third evaluated muscle was affected in the group with mild neurogenic lesion, while more than half were affected in the group with severe neurogenic lesion. The average values of HRCs among different groups of patients and the control showed the population-genetic differences that exist among them (control HRC/15=3.0&plusmn, 0.2, mild HRC/15=3.6&plusmn, moderate HRC/15=4.8&plusmn, 0.3, severe neurogenic lesion HRC/15=5.0&plusmn, 0.3). Conclusions. Spina bifida patients have a significant increase of recessive homozygosity and a decreased variability compared to the control group. As neurogenic lesions are more severe, more affected muscles are present, as well as the increase of individual recessive homozygosity.

We sought to investigate electromyographic characteristics of Guillain-Barré syndrome (GBS) patients in the recovery phase by using serial electromyography (EMG). We included seven GBS patients and assessed their neurologic function at admission and 2, 3 and 6 months post onset using Hughes Functional Grading Scale scores. All patients underwent serial electromyographic assessment of compound muscle action potentials (CMAPs), mean conduction velocity (MCV), and distal motor latency (DML) of peripheral nerves. F wave was recorded of the median nerve and ulnar nerve. All seven patients had a Hughes Functional Grading Scale score between 3 and 6 at admission, while three patients at 2 months, one patient at 3 months, and no patient at 6 months post GBS onset had a Hughes Functional Grading Scale score between 3 and 6 ( P

Introduction Clinically oriented diagnostic criteria can be as specific for diagnosis of sporadic inclusion body myositis (sIBM) as pathological criteria, especially at the time of presentation. EMG may provide an convincing proof that a muscle biopsy should be performed. Aims To compare the EMG results in patients with sIBM divided into subgroups based on the newest ENMC criteria for sIBM and to obtain the utility of EMG in the diagnostic process at the time of presentation. Methods We retrospectively analysed 16 patients with sIBM for motor unit action potential (MUAP) morphology as well as occurrence and distribution of abnormal spontaneous activity (SA) in muscles. Results Abnormal SA was recorded in 62.5% of sIBM patients. We found statistically significant differences between subgroups in the incidence of polyphasic MUAPs and high amplitude outliers which were more commonly seen in the “clinico-pathologically defined sIBM”. Duration of MUAP in the tibialis anterior was significantly shorter in “probable sIBM”. Discussion “Pseudo-neurogenic” MUAPs, mainly in lower limb muscles, are more commonly seen in “clinico-pathologically defined sIBM” while myopathic MUAPs with prominent abnormal SA are recorded in patients diagnosed with “probable sIBM”. Both EMG patterns may be suggestive of sIBM and be an indication for further diagnosis.

The objective of this study was to improve the evaluation of unilateral vocal fold paralyses (uVFP) by means of an area measurement of the glottic plane, which describes the position of the paralysed vocal fold. The area measurements were related to electromyographic findings and clinical outcome (recovery, voice quality). In 56 patients (33 women and 23 men), uVFP were confirmed by endolaryngeal electromyography (EMG) of the paralysed vocal fold and cricothyroid muscles (CT). The EMG response was classified on a 4-point scale (from 0 to 3). Vocal fold position was divided into 'paramedian' and 'intermediate' and additionally quantified by measurement of the glottic area. An 'area quotient' (AQ) was calculated and related to the EMG findings and clinical outcome. Voice qualities were objectified regarding their additive noise (breathiness) and irregularity (roughness) using the 'Göttingen Hoarseness Diagram'. The majority of uVFP was due to iatrogenic lesions. The AQ of classically graduated 'paramedian' and 'intermediate' vocal fold positions was significantly different but did not correlate with objective voice quality values. There were no significant correlations regarding EMG findings, duration or recovery from paralyses. Laryngeal EMG remains the gold standard for verifying uVFP. But EMG did not correlate significantly with AQ or functional outcome of uVFP. The measurement of an AQ is suitable for obtaining continuous data describing the position of paralysed vocal folds beyond the terms 'paramedian' or 'intermediate' and provides the basis for clinical evaluations of diagnostic tools and therapeutic interventions.

Flail arm syndrome (FAS), an atypical presentation of amyotrophic lateral sclerosis (ALS), is characterized by progressive, predominantly proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or respiratory muscles. When encountering a patient who presents with this symptomatic profile, possible diagnoses include upper limb onset ALS (UL-ALS), and FAS. The lack of information regarding FAS may make differential diagnosis between FAS and UL-ALS difficult in clinical settings. The aim of this study was to compare clinical and electromyographic findings from patients diagnosed with FAS with those from patients diagnosed with UL-ALS. To accomplish this, 18 patients with FAS and 56 patients with UL-ALS were examined. Significant differences were observed between the 2 groups pertaining to the rate of fasciculation, patterns of predominantly affected muscles, and the Medical Research Council scale of the weakest muscle. The presence of upper motor neuron signs and lower motor neuron involvement evidenced through electromyography showed no significant between-group differences.

Introduction Congenital myopathies (CM) followed by Congenital muscular dystrophies (CMDs) are the most common muscular cause of floppy infant syndrome. It is well known that EMG pattern in both groups is similar, but it is recently described a characteristic association between peripheral neuropathy and some congenital muscular dystrophies. The objective of this presentation is to describe the EMG findings in our cohort. Material and methods We reviewed retrospectively charts and EMG studies of patients with genetically confirmed Cand CM followed in our Hospital in order to evaluate the pattern of EMG involvement. Results All patients showed proximal myopathic changes at EMG. Only one patient with DMC had spontaneous activity at rest (LAMA2). Three patients shared a mixed pattern: First patient with col VI myopathy showed a distal motor axonal neuropathy (similar to distal motoneuron disease), the second one with col VI myopathy displayed minor motor conduction abnormalities but with loss of motor unit at distal muscles and a third patient with LAMA2 Cexhibited isolated sensory motor desmyelinating findings. Conclusions In our cohort only 37% of Cpatients show association of neuropathy and myopathy at EMG. In patients with floppy infant syndrome we suggest to perform nerve conduction studies and needle EMG at proximal and distal muscles to evaluate the association between myopathy and motor neuropathy to achieve a correct electrodiagnosis. It will be interesting to perform longitudinal studies to know if neuropathy is a finding that develops during the follow up and if it depends on the type of genetic abnormality.

Emel Oguz Akarsu1, Hurtan Acar2, Feriha Ozer3, Sefer Gunaydin2, Ozger Akarsu4, Tuba Aydemir Ozcan3, Serkan Ozben5, Aytul Mutlu2, Mithat Bedir6, Gulsun Cinarli Gul2, Ozlem Cokar2, Mehmet Burak Aktuglu7 1Umraniye Training and Research Hospital, Neurology Clinic, Istanbul, Turkey 2Haseki Training and Research Hospital, Neurology Clinic, Istanbul, Turkey 3Ordu University Faculty of Medicine, Department of Neurology, Ordu, Turkey 4Kartal Training and Research Hospital, Nephrology Clinic, Istanbul, Turkey 5Kafkas University Faculty of Medicine, Department of Neurology, Kars, Turkey 6Mardin State Hospital, Neurology Clinic, Mardin, Turkey 7Haseki Training and Research Hospital, Family Medicine Clinic, Istanbul, Turkey Original Article / Ozgun Arastirma DO I:10.4274/Tnd.13471

Rippling muscle disease (RMD) is a myopathy with hyperirritability, the pathophysiology for which is uncertain.We report electromyographic findings in a 30-year-old man with RMD. Clinical features included muscle rippling and percussion-induced rapid muscle contractions. Both were associated with bursts of short-duration, low-amplitude spikes, which resembled single muscle fiber discharges. Our case stands in contrast to previously reported cases, which showed either electrical silence or motor unit potential discharges associated with rippling, and may represent muscle fiber hyperexcitability.

Introduction: Patients who require prolonged endotracheal intubation (>48 hours) are at risk of dysphagia. Speech-language pathologists should perform swallowing exercises after extubation due to the high probability of developing aspiration pneumonia. There are no studies describing the use of swallowing techniques employed in post-extubation therapy aided by surface electromyography. Objectives: To evaluate the effects of swallowing function therapy in extubated patients after prolonged orotracheal intubation by means of clinical and electromyographic evaluation. Methods: A total of 15 patients were enrolled in this study (average age 48.6 ± 16.5 years). The study was carried out in three phases: (1) Clinical and electromyographic evaluation using the Dysphagia Risk Assessment Protocol following dysphagia scores criteria, and the measurement of the suprahyoid muscles amplitude (μV) expressed by root mean square (RMS), respectively; (2) swallowing rehabilitation program; and (3) reevaluation of patients after therapy. The Wilcoxon paired test assuming a significance level of 5% was used for statistical analysis. Results: By means of the swallowing scale, it was verified that patients suffered from severe oropharyngeal dysphagia at the first evaluation (80%), but the rehabilitation therapy reduced clinical signs, persistent only in one patient (6.7%) post-therapy, thus, improving swallowing. Significant differences, pre- and post-therapy, for suprahyoid muscles during maximal voluntary isometric contractions of right ( P = .0067) and left ( P = .0215), saliva swallowing by right ( P = .0413) and left ( P = .0151), and liquid swallowing by right ( P = .0479) and left ( P = .0215) sides, were found, as shown by electromyography. Conclusions: Swallowing exercises carried out by extubated patients after prolonged orotracheal intubation increased neuromuscular recruitment of suprahyoid muscles involved with swallowing and reduced dysphagia levels.

Nerve compression and entrapment syndromes are classic examples of the integrated role of radiologists and clinicians in achieving difficult but accurate diagnoses. The pathology of nerves can only be understood after evaluating the normal architecture and imaging characteristics of nerves. Understanding the correlation of the pathophysiology of nerve compromise with electromyographic findings and imaging findings allows for greater comprehension of a difficult topic. Particularly in the elbow, there are multiple potential areas of nerve entrapment and compression that can be evaluated perhaps best with magnetic resonance imaging.

ObjectivesTo describe clinicopathologic features of muscular sarcoidosis and the associated sarcoidosis phenotype through a nationwide multicenter study.MethodsPatients were included if they had histologically proven sarcoidosis and symptomatic muscular involvement confirmed by biological, imaging, or histologic examinations.ResultsForty-eight patients (20 males) were studied, with a median age at muscular symptoms onset of 45 years (range 18–71). Four patterns were identified: a nodular pattern (27%); smoldering phenotype (29%); acute, subacute, or progressive myopathic type (35%); and combined myopathic and neurogenic pattern (10%). In all patterns, sarcoidosis was multivisceral with a median of 3 extramuscular organs involved (mostly lungs, lymph nodes, eyes, and skin) and a prolonged course with long-term use of corticosteroids and immunosuppressive drugs. Muscular patterns differed according to clinical presentation (myalgia, nodules, or weakness), electromyographic findings, muscular MRI, and response to sarcoidosis treatment. The myopathic and neuromuscular patterns were more severe.ConclusionThis nationwide study of muscular sarcoidosis allowed the identification of 4 patterns of granulomatous myositis, which differed by phenotypes and the clinical course.

The post-polio syndrome (PPS) is an entity characterized for an episode of muscular weakness and/or abnormal muscular fatigue in individuals that had presented acute polio years before. We report the case of PPS in a patient, 40 years, that thirty-five years after had had paralytic poliomyelitis, developed new symptoms of fatigue, muscular atrophy, dyspnea, difficulties in deambulation and muscular and joint pain. The electromyographic findings revealed injuried neurons of the anterior horn of the marrow and reinnervation after muscular tests.

An 83-year-old man with multiple medical problems, including gout, pseudogout, and renal insufficiency, presented with more than a year of proximal weakness. He had an extensive previous medical workup, including a normal creatinine kinase. His weakness persisted despite endurance and strength training. Electrodiagnostic findings were consistent with a myopathy, although without abnormal spontaneous activity and a length-dependent neuropathy. On the basis of these findings, colchicine was discontinued. The patient experienced marked symptomatic improvement within a week. Myopathies with neuropathies may be found with the use of colchicine. This case was unusual because of the absence of abnormal spontaneous activity and increased creatinine kinase, as typically reported with colchicine myopathy. Level of Evidence V

Objective: To study a series of patients with traumatic peripheral nerve injury during the past 10 years in Cerrahpasa Medical Faculty/Istanbul/Turkey. Methods: The chart review of 802 patients was evaluated and we explored the type(s) and cause(s) of injury, and electromyographic findings. The study included 171 children and 631 adults and we excluded the patients who suffered from injuries due to the Marmara earthquakes that occurred in 1999. Results: Injury was most common in the upper extremities in both children (78.36%) and adults (63.54%). The common causes of nerve injury in children were as follows: obstetric lesions (46.78%), iatrogenic lesions (16.95%), traffic accidents (15.7%), and sharp lacerations (12.8%), whereas the commonest cause of nerve injury in adults was due to sharp lacerations (27.57%), followed by iatrogenic lesions (25.67%), and traffic accidents (23.77%). The most commonly injured nerves were the brachial plexus and ulnar nerve in children and adults, respectively. Electromyography demonstrated that complete nerve injury predominated in both groups. Conclusions: If preventive measures are taken into consideration satisfactorily, the incidence of disabling peripheral nerve injury may decrease, as such injuries are often treatable.

Glycogen storage disease II (GSD II), also known as Pompe disease is an autosomal recessive disorder caused by mutations in the GAA gene encoding the enzyme acid alpha-glucosidase. Two different clinical forms have been described: infantile (IOPD) and late-onset (LOPD). Aim of the study is to evaluate EMG findings, in particular distal muscle involvement, in a cohort of LOPD patients. 15 LOPD patients (9 Male–6 Female) underwent to ENG and EMG evaluation looking at spontaneous activity, MUP analysis recruitment. EMG evidenced the presence of myotonic discharges (MD) in paraspinal muscles in 6 patients and in distal limb muscles in 1 patient. Fibrillation potentials occurred in all muscles where MD were found. Short duration, MUPs were found in the paraspinal (6/12), proximal (6/12) and distal limb muscles (5/12). Our results confirm that myopathic EMG pattern is usually observed in proximal muscles, but we also find a similar pattern in distal muscles revealing a subclinical involvement. Moreover, myotonic discharges and denervation activity are present in both paraspinal and distal muscles. We can conclude that it is useful to extend the EMG study also to distal muscles even if not clinically compromised.

This study assessed lumbago and its radicular propagation to the lower extremities by CT myelography, interpreting possible etiopathogenetic factors. We diagnosed swelling of the cauda equina roots on the basis of clinical features, CT myelography and electromyographic findings.

We reviewed electrodiagnostic findings in patients whose electromyograph pointed toward a disorder of the central nervous system. Of the 34 patients identified, 17 did not have a clinical diagnosis of a central nervous system disorder before electromyogram. Signs suggesting a central nervous system disorder included poor activation, poor relaxation, extensor plantar responses, and flexor spasms on needle electromyogram. Ultimate central nervous system diagnoses included myelopathy, demyelinating disease, parkinsonism, tumor, and stroke.

Recently characterized as an immune-mediated channelopaty, Isaacs' syndrome (also known as acquired neuromyotonia) was first described in 1961 in two men with persistent, generalized muscle stiffness, in addition to spontaneous, rapid discharges of motor-unit potentials on electromyography. In the peripheral nervous system, antibodies targeted to voltage-gated potassium channels induce hyperexcitability of motor axons, resulting in signs of muscle stiffness or of pseudomyotonia. A spontaneous burst of single motor-unit activity, and myokymic and neuromyotonic discharges, are the most characteristic features found in electromyography studies. This report describes Isaacs' syndrome in a child, in whom the diagnosis was made by clinical features of acquired, spontaneous muscle overactivity and typical electromyographic findings.

To develop an index of gait instability from electromyographic information, we made observations on infants from the time they first began to walk independently at about one year of age until around three years of age. From our findings we obtained the following criteria. (1) Very unstable gait: As seen in a child within the first month of learning to walk the vastus medialis is active in the latter half of swing phase, the tibialis anterior and rectus femoris are active during stance phase, and activity of the vastus medialis is continuous. These electromyographic characteristics are not usually seen in subsequent childhood gait or in adult gait, and they serve as markers of very unstable gait. (2) Unstable gait: Activity of the gastrocnemius in the latter half of swing phase is generally noted only within the first three months after a child learns to walk, and that activity is interpreted as a sign of unstable gait. (3) Slightly unstable gait: Activity of the gastrocnemius in the first half of stance phase and continuous activities of the biceps femoris and gluteus maximus from initial contact with the floor until push off are found in children until three years of age. These activities are considered electromyographic markers of slightly unstable gait.

Understanding the cause of adolescent idiopathic scoliosis is important for elucidating its pathogenesis. Epidemiological investigations have demonstrated that the incidence of idiopathic scoliosis is approximately 2% in patients with a Cobb angle of 20°. The proportion of patients with adolescent idiopathic scoliosis who are need of treatment is only 0.1% to 0.3%. The wide range of curve magnitudes in patients with idiopathic scoliosis suggests the presence of multiple genetic and environmental factors1-3. Various theories have been proposed regarding the cause of idiopathic scoliosis, but none have been widely accepted3. Based on historical and recent findings regarding the cause of idiopathic scoliosis, the present report reviews the muscular theory, neurological theory, connective tissue theory, bone growth mismatch theory, genetic theory, and experimental animal models of this disorder. The most representative and important report on the muscular theory of adolescent idiopathic scoliosis was based on the electromyographic findings of Le Febvre et al.4. That study revealed potential asymmetry of the erector …

This study was designed to investigate masticatory disturbances in patients with occupational maxillofacial injuries. During the 3 -year 3 -month period from April 1992 through June 1995, 51 patients were examined on the basis of (1) dental findings, (2) mandibular mobility, (3) electromyograms (EMG), (4) occlusal findings by Dental Prescale®, and (5) masticatory efficiency rate calculated from the results of a questionnaire that assessed the ability to chew 173 foods.The following results were obtained:1. Masticatory efficiency rate could be estimated on the basis of occlusal variables assessed with Dental Prescale®. The most important occlusal variable with this method was maximal force.2. At a value of 40% or more, masticatory efficiency rate was correlated with dental findings and mandibular mobility.3. In this same group of patients, masticatory efficiency rate was correlated with electromyographic findings (silent period rate, etc.).4. Variables of the occlusion evaluated with Dental Prescale® were positively correlated with masticatory efficiency rate.5. In patients with a masticatory efficiency rate of 40% or more, masticatory disturbances could be generally estimated from masticatory efficiency rate.6. Many patients with a masticatory efficiency rate of less than 40% had lack of consistency between objective and subjective findings. Masticatory disturbances could therefore not be evaluated correctly by analyzing items (1) to (5) in this group of patients.

Objective In electromyography (EMG) the presence of fibrillation potentials or positive sharp-waves is often interpreted as a sign of acute ongoing denervation activity (DA). Large motor unit potentials and discrete interference pattern with large amplitude may indicate more chronic changes. Our hypothesis was that the proportion between acute and chronic EMG changes may be related to the disease course in amyotrophic lateral sclerosis (ALS). Methods Fifty-five consecutively patients referred for motor neuron disease at our department were included. Diagnosis according to the revised El Escorial criteria was confirmed during the course of the disease. Acute and chronic EMG changes in individual muscles were dichotomised and related to survival time after EMG by means of Kaplan-Meier statistics. Results For the anterior tibial muscle survival was shorter in patients with DA ⩾ 7/10 sites (log-rank test p Conclusion The amount of DA in distal limb muscles relates to survival in ALS. Key message EMG findings may predict prognosis in ALS.

Three cases of acquired demyelinating multifocal neuropathy with persistent conduction blocks are reported. In one of them protyreline tartrate (TRH-T) had an evident deblocking effect; similar but milder effects were seen in the other two cases. The course and consistency of this effect is analyzed on the grounds of clinical and electromyographic findings. The nature of the THR-T responsive conduction block is analysed as a mild variant of CIDP with regard to its occurrence in a late and chronic phase without signs of active immune processes.

Electrophysiologic findings in thallium intoxication are usually untimely, limited in extent, and often uninformative. This report documents serial conduction and electromyographic findings in a case of thallium poisoning, beginning 10 days after symptom onset and ending 24 months later. Initially, the plantar nerves in the foot demonstrated profound axonal loss while the sural and peroneal nerves were essentially normal. The latter two nerves subsequently underwent axonal loss. Two years were required for the sural and peroneal nerves to display recovery. At 24 months, the plantar nerves continue to remain absent. A primarily distal axonopathy, significantly worse in the lower than upper extremities and requiring more than 2 years for recovery, now documents what was previously speculated: the electrophysiologic course of thallium intoxication. Additionally, this case emphasizes the need to examine the plantar nerves of the foot to avoid missing distal axonopathies during the early course of the disease process. The clinical course and pathophysiology of thallium poisoning are also reviewed.

Parsonage–Turner syndrome is the term used to describe a neuritis involving the brachial plexus. It may present with symptoms of an isolated peripheral nerve lesion, although the pathology is thought to lie more proximally. A case describing an isolated anterior interosseus nerve palsy due to an acute brachial neuritis is presented where the electromyographic findings confirmed the diagnosis, but also demonstrated the coexistence of a dual pathology in the form of a cervical radiculopathy. The literature is reviewed regarding etiology, treatment, and prognosis.

The subsequent clinical course of patients examined in the EMG laboratory has not been adequately studied before. It is not known how EMG findings in practice affect patients' final diagnosis, treatment and outcome. The aim of the present study was to test the hypothesis that EMG results have an important impact on the choice of patients' treatment and on outcome. Three years after EMG examination, a short questionnaire asking about initial symptoms, the final diagnosis, treatment and current symptoms was sent to 300 consecutive EMG patients. Responses concerning 186 of them (39% men) were received either from patients and/or from their referring physicians. Information obtained during EMG examination was also added. Data was analyzed using methods of bivariate and multivariate statistics. We found that patients with a pathologic EMG had a better clinical outcome 3 years later. Pathologic EMG has an independent effect, pointing to more favorable disease courses, which was further improved by more active treatment approaches received by these patients. The study demonstrated the important impact of EMG findings on the treatment choice and clinical outcome in population consisting mainly of patients with carpal tunnel syndrome and radiculopathy. Studies in other patient populations are needed.

Background/Aims: Little is known about the distribution of electromyographic (EMG) abnormalities in myositis even though this is relevant in daily practice. Methods: A retrospective semiquantitative analysis of needle EMG findings was performed in a group of 98 patients with myositis. The frequency, type, and distribution of abnormalities were studied. The influence of the use of corticosteroids and the stage of the disease were evaluated. Results: In most patients, a myopathic pattern with spontaneous activity was found, although several clinically relevant exceptions were noted. Long-duration motor unit potentials were found in all three diagnostic groups and were not associated with disease duration. In the lower extremity a distal to proximal gradient was present, adding to the diagnostic confusion with neurogenic diseases, and spontaneous activity was absent in a relatively large group although none of the patients in the acute stage of the disease had a normal EMG. The use of corticosteroids reduced the number of abnormal findings in dermatomyositis and polymyositis, but not in inclusion body myositis. Conclusion: A myopathic pattern with spontaneous activity was most frequently found, although several clinically relevant exceptions were noted. These results illustrate the spectrum of EMG findings in myositis, and may aid the clinician in the interpretation of the EMG in these patients.

Background Injury to the recurrent laryngeal nerve (RLN) is a rare complication of initial thyroid and parathyroid surgery, but the prevalence is much higher in the reoperative setting. The use of continuous, intraoperative electromyographic monitoring of the RLN has been suggested to improve the safety of cervical explorations. Methods Outcomes of a group of reoperative thyroid and parathyroid cases that used EMG monitoring with endoscopically applied hook-wire electrodes were compared with a group of cervical reoperations without monitoring. Office laryngoscopy (indirect or fiberoptic) was used to evaluate and follow suspected RLN complications. Results Electromyography was used in 52 cervical reexploration procedures. Patients averaged 1.8 previous explorations (range, 1-7 explorations) and underwent procedures for parathyroid (31%) and/or thyroid (77%) disease (overall, 72% malignant). The nonmonitored group had 59 patients with similar characteristics. Only 1 permanent nerve complication in each group was unintended (electromyography, 1.9%; non-electromyography, 1.7%). Seven false-negative and 2 false-positive electromyographic findings occurred. No complications resulted from placement of the electromyography electrodes. Conclusions Intraoperative electromyographic monitoring of the RLN in reoperative neck surgery can be performed safely but did not decrease RLN complications in this study. Experience and routine nerve exposure remain crucial to the minimization of RLN complications.

In some patients with a history of polio, the electromyography is normal, not showing the typical neurogenic signs. The aim of this study was to explain the normal findings in electromyography, especially in paralytic polio.Retrospective study.Concentric needle electromyography, macro electromyography (including single fibre electromyography) and neurography were performed in various combinations in 688 patients with an alleged history of polio.Thirty-five patients with paralytic polio had normal or minimally abnormal neurophysiology. In 6 patients the diagnosis of polio was rejected and was instead found to be other diagnoses. Three patients had a very atypical history. Of the 26 with possible paralytic polio, 17 showed a strong suspicion of previous paralytic polio without any neurophysiological signs of degeneration of the anterior horn cells.If neurophysiological findings are normal in patients with a history of polio, the original diagnosis may be incorrect. However, the absence of electromyography changes does not entirely exclude a previous history of polio with transient functional loss without degeneration of anterior horn cells vulnerable for later functional impairment.

A computerized system is presented to provide expert assistance to a physician in the evaluation of electromyographic findings in the clinical diagnosis of compressive radiculopathies. The system uses an object-oriented, frame-based representation of the nerve-root and muscular structure of the lower back and leg, and uses a rule-based reasoning system to interpret electromyographic findings and relate them to potential pathologies in the nerve roots in the lower spinal column. A novel feature of this research was the development of a hybrid system of Bayesian regulated belief functions and symbolic endorsements for the resolution of uncertainty in the clinical observations. This hybrid system provided quantitative estimates of the nerve pathologies. while simultaneously providing the physician with qualitative information regarding the interrelations of the clinical findings and the diagnosis. >

Both intra-anal sponge electromyography (SEMG) and needle electromyography (NEMG) are used to diagnose paradoxical puborectalis contraction (PPC). The aim of this retrospective study was to assess the correlation among SEMG and NEMG and cinedefecography (CD) in the diagnosis of PPC.Between 1992 and 1999, a total of 261 constipated patients underwent both CD and EMG: 64 had NEMG while 197 had SEMG. PPC was diagnosed by EMG when there was failure to achieve a significant decrease in electrical activity of the puborectalis muscle during attempted evacuation. CD criteria for PPC included lack of straightening of the anorectal angle or persistence of the puborectalis impression during evacuation. CD was considered diagnostic and EMG results were thus compared with those of CD. Agreement was calculated using the kappa statistics (kappa) for concordance.Both NEMG and SEMG had low positive predictive rates (33% in NEMG, 28% in SEMG) and high negative predictive rates (91% in NEMG, and 78% in SEMG) when correlated with CD findings. Agreement between NEMG and CD was noted in 46 of 64 patients (72%, kappa=0.274) while there was agreement between SEMG and CD in 120 of 197 patients (61%; kappa=0.067); p0.05 needle vs. sponge.Although both NEMG and SEMG have a low positive predictive values, they have high negative predictive value for PPC. Therefore, neither NEMG nor SEMG alone are optimal tests for diagnosing the presence of PPC.

We describe a case of carpal tunnel syndrome (CTS) in a hypertensive man on long term treatment with a beta-blocker, propranolol. The clinical and instrumental findings, including MRI at the wrist, excluded all other possible causes of CTS. The improvement in symptoms and electromyographic findings on discontinuation of the drug suggested that the CTS may have been related to the beta-blocker therapy.

Children with spinal muscular atrophy were treated by the administration of thyrotropin-releasing hormone. In three infants with spinal muscular atrophy type I, thyrotropin-releasing hormone showed little efficacy, but in children with types II and III, there was improvement in motor function and electromyographic findings after the thyrotropin-releasing hormone therapy. Thyrotropin-releasing hormone has a neurotrophic effect on the spinal anterior motor neurons of spinal muscular atrophy patients and thus may be warranted for the management of spinal muscular atrophy. (J Child Neurol 1994;9:287-289).

A 20-year-old carpet-layer with compression of the right sural nerve due to the peculiar posture maintained during his work is described, and electromyographic findings are presented.