Electrodiagnostic methods to verify Guillain-Barré syndrome subtypes in Istanbul: A prospective multicenter study.

BACKGROUND AND AIMS: This study aimed to identify the clinical characteristics and electrodiagnostic subtypes of Guillain-Barré syndrome (GBS) in Istanbul. METHODS: Patients with GBS were prospectively recruited between April 2019 and March 2022 and two electrodiagnostic examinations were performed on each patient. The criteria of Ho et al., Hadden et al., Rajabally et al., and Uncini et al. were compared for the differentiation of demyelinating and axonal subtypes, and their relations with anti-ganglioside antibodies were analyzed. RESULTS: One hundred seventy-seven patients were included, 69 before the coronavirus disease 2019 pandemic (April 2019-February 2020) and 108 during the pandemic (March 2020-March 2022), without substantial changes in monthly frequencies. As compared with the criteria of Uncini et al., demyelinating GBS subtype diagnosis was more frequent according to the Ho et al. and Hadden et al. criteria (95/162, 58.6% vs. 110/174, 63.2% and 121/174, 69.5%, respectively), and less frequent according to Rajabally et al.'s criteria (76/174, 43.7%). Fourteen patients' diagnoses made using Rajabally et al.'s criteria were shifted to the other subtype with the second electrodiagnostic examination. Of the 106 analyzed patients, 22 had immunoglobulin G anti-ganglioside antibodies (14 with the axonal subtype). They had less frequent sensory symptoms (54.5% vs. 83.1%, p = 0.009), a more frequent history of previous gastroenteritis (54.5% vs. 22.9%, p = 0.007), and a more severe disease as compared with those without antibodies. INTERPRETATION: Serial electrodiagnostic examinations are more helpful for accurate subtype diagnosis of GBS because of the dynamic pathophysiology of the disease. We observed no significant increase in GBS frequency during the pandemic in this metropolis.

Investigation of Ongoing Denervation and Reinnervation in Amyotrophic Lateral Sclerosis by Using Concentric Needle Electrode with Single Fiber Electromyography Method.

Introduction: The aim of this study is to demonstrate the conduction disturbance at the neuromuscular junction in a cranial muscle by measuring jitter with a concentric needle (CN) electrode in the diagnosis of Amyotrophic Lateral Sclerosis (ALS) and to investigate the utility of evaluating the peak number as an ongoing reinnervation marker. Method: Twelve patients diagnosed with ALS were included in this study. Single fiber electromyography (SFEMG) was performed using a CN electrode during the voluntary contraction of the right extensor digitorum communis (EDC) and left frontalis muscles. Results: In SFEMG from the right EDC muscle, the mean jitter value was high in all of them. The average jitter calculated in EDC muscles was 57.76±24.17 µs. The mean jitter value in the frontal muscles was 28.91±10.21 µs. In all patients, the number of CN electrode peaks was more than 4 in the EDC muscle and above 4 in 91.67% of the frontal muscle. Conclusion: Detection of high jitter in SFEMG examination indicates that the examined muscle undergoes a denervation-reinnervation process in the case of increased peak number values. When such a determination is made in the extremity muscles, it becomes important for the diagnosis of ALS.

Long-Term Evaluation of Ulnar Nerve Function in Patients with Distal Humerus Fractures Treated with Open Reduction and Internal Fixation: A Nerve Conduction Study.

OBJECTIVES: To investigate the long-term evaluation with electromyography of ulnar nerve function in patients with distal humerus fractures (DHFs) treated with open reduction and internal fixation. DESIGN: Retrospective cohort study. SETTING: Level 1 trauma center. PATIENTS: Fifty-two patients (20 men and 32 women) with DHFs. INTERVENTIONS: All patients underwent open reduction and internal fixation between 2002 and 2017 with a minimum five years' follow-up. MAIN OUTCOME MEASUREMENTS: The nerve conduction test was done for evaluation ulnar nerve function. Secondary outcomes were modified McGowan grading system for symptoms of ulnar neuropathy. RESULTS: The mean follow-up time was 112.7 ± 39 months after surgery. 28.8% (15) of the patients scored in Grade I, 30 (57.6%) in Grade II, and 7 (13.6%) in Grade III on the affected side according to McGowan grading scale. According to EMG results, 40.1% (21/52) of patients had abnormal results. There was significant difference between fracture and unaffected side regarding ulnar nerve motor wrist CMAP amplitude, motor below elbow CMAP amplitude, above elbow CMAP amplitude, above elbow MNCV, and sensory wrist amplitude. Ulnar nerve motor wrist-ADM latency (p = 0.01; r = 0.446) and ulnar nerve sensory conduction velocity (p < 0.001, r = -0.504) were significant correlation with McGowan grading scale. CONCLUSION: There were significant difference ulnar nerve motor amplitude and sensory wrist amplitude between fracture and unaffected side. A mean decrease in sensory amplitude of fracture elbow was found 25% compared to the unaffected side in our result. LEVEL OF EVIDENCE: Diagnostic Level III. See Instructions for Authors for a complete description of levels of evidence.

MYO1H is a novel candidate gene for autosomal dominant pure hereditary spastic paraplegia.

In this study, we aimed to determine the genetic basis of a Turkish family related to hereditary spastic paraplegia (HSP) by exome sequencing. HSP is a progressive neurodegenerative disorder and displays genetic and clinical heterogeneity. The major symptoms are muscle weakness and spasticity, especially in the lower extremities. We studied seven affected and seven unaffected family members, as well as a clinically undetermined member, to identify the disease-causing gene. Exome sequencing was performed for four affected and two unaffected individuals. The variants were firstly filtered for HSP-associated genes, and we found a common variant in the ZFYVE27 gene, which has been previously implied for association with HSP. Due to the incompletely penetrant segregation pattern of the ZFYVE27 variant, revealed by Sanger sequencing, with the disease in this family, filtering was re-performed according to the mode of inheritance and allelic frequencies. The resulting 14 rare variants were further evaluated in terms of their cellular functions, and three candidate variants in ATAD3C, VPS16, and MYO1H genes were selected as possible causative variants, which were analyzed for their familial segregation. ATAD3C and VPS16 variants were eliminated due to incomplete penetrance. Eventually, the MYO1H variant NM_001101421.3:c.2972_2974del (p.Glu992del, rs372231088) was found as the possible disease-causing deletion for HSP in this family. This is the first study reporting the possible role of a MYO1H variant in HSP pathogenesis. Further studies on the cellular roles of Myo1h protein are needed to validate the causality of MYO1H gene at the onset of HSP.

Near-tendon single-fiber electromyography in patients with myopathy.

We investigated how properties of single-fiber action potentials (SFAP) and jitter changed in different recording sites in patients with myopathy and controls. SFAP and jitter were recorded using a disposable single-fiber electrode in the biceps brachii muscle of three patients and controls in two sites: i) near the end-plate zone (EPZ), ii) near the tendon. SFAP and spike duration were longer in patients than in controls. Moving the needle away from the EPZ did not change the electrophysiologic parameters. Longer SFAP duration may reflect slow velocity in muscle fibers and serve as an indicator for small fiber size in myopathy.

Automatic detection of F waves and F-MUNE in two types of motor neuron diseases.

INTRODUCTION/AIMS: Motor unit number estimation by F waves (F-MUNE) is an uncommonly used MUNE technique. Our aim in this study was to assess the sensitivity of F-MUNE values elicited with newly developed software in motor neuron diseases. METHODS: F waves were recorded by 300 submaximal stimuli from abductor digiti minimi and abductor pollicis brevis muscles of 35 patients with amyotrophic lateral sclerosis, 18 with previous poliomyelitis, and 20 controls. The software determined the surface motor unit action potentials (sMUAPs) and calculated the F-MUNE values. Compound muscle action potential scans were also recorded to obtain MScanFit. RESULTS: The sMUAP amplitudes were higher and F-MUNE values were lower in both muscles of the patients when compared with controls. F-MUNE values could distinguish patients from controls. Significant correlations were found between F-MUNE and MScanFit in the patient groups. DISCUSSION: The new F-MUNE software offered promising results in revealing motor unit loss caused by motor neuron diseases.

Does transcutaneous electrical nerve stimulation affect pain, neuropathic pain, and sympathetic skin responses in the treatment of chronic low back pain? A randomized, placebo-controlled study.

BACKGROUND: The purpose of this study was to assess the effectiveness of transcutaneous electrical nerve stimulation (TENS) in chronic low back pain and neuropathic pain. METHODS: Seventy-four patients aged 18-65 with chronic low back pain were included in the study. Baseline measurements were performed, and patients were randomized into three groups. The first group received burst TENS (bTENS), the second group conventional TENS (cTENS), and the third group placebo TENS (pTENS), all over 15 sessions. Patients' visual analogue scale (VAS) scores were evaluated before treatment (preT), immediately after treatment (postT), and in the third month after treatment (postT3). Douleur Neuropathique 4 Questions (DN4), the Modified Oswestry Low Back Pain Disability Questionnaire (MOS), the Beck Depression Inventory (BDI), and sympathetic skin response (SSR) values were also evaluated preT and postT3. RESULTS: A statistically significant improvement was observed in mean VAS scores postT compared to preT in all three groups. Intergroup comparison revealed a significant difference between preT and postT values, that difference being assessed in favor of bTENS at multiple comparison analysis. Although significant improvement was determined in neuropathic pain DN4 scores measured at postT3 compared to preT in all groups, there was no significant difference between the groups. No statistically significant difference was also observed between the groups in terms of MOS, BDI, or SSR values at postT3 (P > 0.05). CONCLUSIONS: bTENS therapy in patients with low back pain is an effective and safe method that can be employed in short-term pain control.

Corticomotor Excitability in Two Kinds of Motor Neuron Diseases: A Study on the Patients With Amyotrophic Lateral Sclerosis and Poliomyelitis Survivors.

PURPOSE: To examine upper motor neuron functions comparatively in patients with amyotrophic lateral sclerosis (ALS) and poliomyelitis survivors using transcranial magnetic stimulation (TMS) methods. METHODS: Single- and paired-pulse TMS with conventional methods and the triple-stimulation technique were performed by recording from the abductor digiti minimi and abductor pollicis brevis muscles in 31 patients with ALS, 18 patients with poliomyelitis survivors, and 21 controls. Nine patients were diagnosed as having postpoliomyelitis syndrome after a 6-month follow-up. RESULTS: Triple-stimulation technique and some of conventional TMS studies were able to distinguish ALS from both poliomyelitis survivors and controls. A reduced ipsilateral silent period in abductor pollicis brevis muscles was the only parameter to show a significant difference when comparing thenar and hypothenar muscles in ALS. No significant difference was present in any TMS parameters between the postpoliomyelitis syndrome and non-postpoliomyelitis syndrome groups. CONCLUSIONS: Conventional TMS and particularly triple-stimulation technique studies are helpful in disclosing upper motor neuron dysfunction in ALS. The results of this study might favor the cortical hypothesis for split hand in ALS, but they revealed no significant indication for upper motor neuron dysfunction in postpoliomyelitis syndrome.

Another brick in the wall: is hexane neuropathy a 'nodo-paranodopathy'?

n-Hexane gives cause to one of the most common toxic polyneuropathies seen in poorly ventilated factories. It is a sensory-motor polyneuropathy ending up with axonal degeneration. Nerve biopsy reveals paranodal axonal swelling and secondary myelin retraction in early stages. Myelin retraction imitates demyelination causing focal conduction block and failure before axonal degeneration emerges. This brings to mind the new category of nodo-paranodopathy described first for anti-ganglioside antibody-mediated neuropathies, which can be proved by electrophysiological re-evaluations. We, herein, discuss the clinical and electrophysiological follow-up of three patients with n-hexane neuropathy and remark overlaps with new concept nodo-paranodopathy.

Electrophysiological Findings of Subclinical Lower Motor Neuron Involvement in Degenerative Upper Motor Neuron Diseases.

INTRODUCTION: The present study is an examination of possible subclinical involvement of lower motor neuron (LMN) in patients with primary lateral sclerosis (PLS) and hereditary spastic paraparesis (HSP) electrophysiologically. METHODS: Nine PLS patients and 5 HSP patients were prospectively analyzed. Jitter measurement with concentric needle electrode (25 mm, 30 G) (CN-jitter) recorded from right extensor digitorum muscle during voluntary contraction with 1 kHz high-pass frequency filter set. European Myelopathy Score (EMS) was used to evaluate disability. The relationship between disability score and jitter values was investigated. RESULTS: HSP patients had suffered from the disease for longer period of time (p<0.001). Mean jitter values of patients with PLS and HSP were 26.5±12.1 µs and 30.8±34.8 µs, and the number of individual high jitters (>43 microseconds) observed in the PLS and HSP groups was 16/180 and 9/100, respectively without a significant intergroup difference. The ratio of patients with an abnormal jitter study were higher in HSP group (60%) compared to PLS (22%) (p<0.05). Potential pairs with blocking were present in HSP group (7 of 100 potential pairs) but not seen in PLS patients. EMS values were significantly lower in patients having potential pairs with high jitter and blocking compared to those without high jitter and blocking. CONCLUSION: The present study has demonstrated that early signs of LMN dysfunction can be detected electrophysiologically by CN-jitter in patients with UMN involvement. These electrophysiological findings in these patients with longer disease duration and lower clinical scores may be explained by spreading of the disease to LMNs or transsynaptic degeneration and its contribution in disease progression.

Compound muscle action potential scan and MScanFit motor unit number estimation during Wallerian degeneration after nerve transections.

BACKGROUND: Compound muscle action potential (CMAP) scan and MScanFit have been used to understand the consequences of denervation and reinnervation. This study aimed to monitor these parameters during Wallerian degeneration (WD) after acute nerve transections (ANT). METHODS: Beginning after urgent surgery, CMAP scans were recorded at 1-2 day intervals in 12 patients with ANT of the ulnar or median nerves, by stimulating the distal stump (DS). Stimulus intensities (SI), steps, returners, and MScanFit were calculated. Studies were grouped according to the examination time after ANT. Results were compared with those of 27 controls. RESULTS: CMAP amplitudes and MScanFit progressively declined, revealing a positive correlation with one another. SIs were higher in WD groups than controls. Steps appeared or disappeared in follow-up scans. The late WD group had higher returner% than the early WD and control groups. CONCLUSIONS: MScanFit can monitor neuromuscular dysfunction during WD. SIs revealed excitability changes in DS.

Changes in motor unit bioelectrical activity recorded at two different sites in a muscle.

INTRODUCTION: The objective of this study was to compare the properties of bioelectrical signals of motor units recorded at different sites in the muscles of controls, patients with myopathy and patients with motor neuron disease (MND). METHODS: Five controls, 10 patients with myopathy and 11 patients with MND were included. Electrophysiologic tests were performed in the biceps brachii (BB) muscle from two recording sites. Site 1 was near the belly of the muscle and Site 2 was 5cm distal from Site 1, near the tendon. Multi-motor unit potential (MUP) analysis, jitter analysis, and peak number count were calculated from the signals recorded using a concentric needle electrode (CN). RESULTS: At Site 2, duration was longer, number of phases was higher and amplitudes were smaller in MUPs compared with those recorded at Site 1. This significant difference between recording site and patient groups was related to neurogenic muscles. Jitter analysis showed no significant difference except an intergroup difference between the patient groups and controls. The peak number calculated using the CN was greater when recorded from Site 1 in concordance with MUP analysis. CONCLUSION: Duration of MUP was longer and amplitude was smaller when the recording electrode was placed distally along the muscle near the tendon in neurogenic muscles, probably related to increased temporal dispersion. However, changing the position of the needle did not provide further information in distinguishing myogenic muscles.

Repeater F-waves in amyotrophic lateral sclerosis: Electrophysiologic indicators of upper or lower motor neuron involvement?

OBJECTIVE: To extract insight about the mechanism of repeater F-waves (Frep) by exploring their correlation with electrophysiologic markers of upper and lower motor neuron dysfunction in amyotrophic lateral sclerosis (ALS). METHODS: The correlations of Frep parameters with clinical scores and the results of neurophysiological index (NI), MScanfit MUNE, F/M amplitude ratio (F/M%), single and paired-pulse transcranial magnetic stimulation (TMS), and triple stimulation technique (TST) studies, recorded from abductor digiti minimi (ADM) and abductor pollicis brevis (APB) muscles of 35 patients with ALS were investigated. RESULTS: Frep parameters were correlated with NI and MScanfit MUNE in ADM muscle and F/M% in both muscles. None of the Frep parameters were correlated with clinical scores or TST and TMS measures. While the CMAP amplitudes were similar in the two recording muscles, there was a more pronounced decrease of F-wave persistence in APB, probably heralding the subsequent split hand phenomenon. CONCLUSION: Our findings suggest that the presence and density of Freps are primarily related to the degree of lower motor neuron loss and show no correlation with any of the relatively extensive set of parameters for upper motor neuron dysfunction. SIGNIFICANCE: Freps are primarily related to lower motor neuron loss in ALS.

Repetitive nerve stimulation and jitter measurement with disposable concentric needle electrode in newly diagnosed myasthenia gravis patients.

INTRODUCTION: The aim of this study was to define the diagnostic accuracy of concentric needle (CN)-jitter in newly diagnosed myasthenia gravis (MG) patients and to compare CN-jitter with repetitive nerve stimulation. METHODS: In 30 MG patients, repetitive nerve stimulation in 4 muscles (orbicularis oculi, nasalis, trapezius and abductor digiti minimi) and CN-jitter of extensor digitorum (ED) and frontalis muscles were evaluated. RESULTS: Twenty-eight of 30 patients (93%) had high jitter in at least one muscle. Repetitive nerve stimulation was abnormal in 23 of the patients (77%). Eighty-six percent of the patients in whom repetitive nerve stimulation test was negative could be diagnosed with CN-jitter. The most frequent muscle showing abnormal decrement was orbicularis oculi. The results of CN-jitter were similar between patients with different serological groups. Of 13 patients with generalized weakness, all had high jitter in both muscles studied whereas of 17 patients only with ocular weakness, 15 had high jitter in at least one muscle studied. CONCLUSION: Abnormal RNS was present in 77% of newly diagnosed MG patients, being less than CN-jitter (93%) but more than antibody positivity (73.3%).

Jitter Values on Voluntary Active Periocular Muscles of Healthy Subjects with Conventional (37 mm) Concentric Needle Electrode.

INTRODUCTION: The aim of this study was to re-evaluate jitter values of healthy subjects in whom pairs of single-fiber-like potentials were recorded from voluntary activated periocular muscles using a disposable 37-mm concentric needle electrode (CNE) with 2-kHz low-cut filtering. METHODS: We reviewed the recordings of 129 subjects (85 women; 44 men; mean age, 43.8±15.3 years). The m. frontalis group included 116 subjects, and the m. orbicularis oculi group included 18 subjects. Jitter values were expressed as the mean consecutive difference (MCD) of 20 different pairs. RESULTS: The mean MCD (n=2680) was 22.5±9.7 µs (range, 5-121 µs), and the upper 95% confidence limit (CL) was 39 µs. The mean of 134 MCD values for each subject was 22.5±3.7 µs (range, 15-33 µs), and the upper 95% CL was 30 µs. The outer limit of the 18th highest MCD values out of 20 recordings for each subject was 31.3±6.5 µs (range, 18-53 µs), with an upper 95% CL of 43.3 µs. CONCLUSION: Using a conventional 37-mm CNE with 2-kHz low-cut filtering may be a cost effective alternative to a single-fiber electrode in periocular muscles if strict criteria are used for acceptable signals. Jitter values of >44 µs that were calculated from single-fiber-like action potential pairs should alert the physician regarding the possibility of neuromuscular junction disorders and constitute an indication for a further diagnostic investigation.

Jitter analysis with concentric needle electrode in the masseter muscle for the diagnosis of generalised myasthenia gravis.

OBJECTIVES: The purpose of our study was to show neuromuscular transmission abnormality in the masseter muscle of generalised myasthenia gravis (MG) patients and to compare motor end-plate failure of the masseter with the extensor digitorum communis (EDC) and periocular muscles. METHODS: Motor end-plate function was evaluated during voluntary contraction of the masseter muscle of 20 generalised MG patients aged between 16 and 63 years, as well as 20 age-matched healthy volunteers. The mean jitter value was calculated for each group and compared. The upper limit of normal jitter was also calculated and the number of jitters exceeding this cut-off value was counted for each group for comparison. In MG patients, jitter analysis was also performed in periocular and EDC muscles along with the masseter and the number of single fibre-like potentials with abnormal jitter was counted for each muscle. All tests were performed during the same session with a concentric needle electrode (CNE). RESULTS: For the masseter muscle, the mean jitter of all potential pairs was significantly higher in the patient group (24.7 ± 9.6 µs in healthy volunteers, 71.9 ± 41 µs in patients). The calculated mean jitter for the 18th highest value in healthy volunteers was 33.8 ± 5.9 µs (upper 95% confidence limit was 45.6 µs). The number of abnormal jitters (≥ 46 µs) was significantly higher in the patient group (276 out of 402 jitters) compared to healthy volunteers (10 out of 400 jitters). In the patient group, the number of single fibre-like potentials with abnormal jitter was found to be similar for the masseter, periocular and EDC muscles. CONCLUSION: The masseter muscle has diagnostic importance in generalised MG. The ratio of high jitters to all of the calculated jitters in a particular muscle was similar for masseter, periocular and EDC muscles. SIGNIFICANCE: Jitter analysis of the masseter muscle during voluntary contraction is easy to perform and it was found as informative as other muscles in patients with generalised MG.

Prediction of externally applied forces to human hands using frequency content of surface EMG signals.

In this work, a new signal processing method was proposed in order to predict externally applied forces to human hands by deriving a relationship between the surface electromyographic (SEMG) signals and experimentally known forces. This relationship was investigated by analyzing the spectral features of the SEMG signals. SEMG signals were recorded from three subjects during isometric contraction and from another three subjects during anisometric contraction. In order to determine force-SEMG signal relationship, higher order frequency moments (HOFMs) of the signals were calculated and used as characterizing features of SEMG signals. Subsequently, artificial neural networks (ANN) with backpropagation algorithm were trained by using the HOFMs. Root mean square difference (RMSD) between the actual and predicted forces was calculated to evaluate force prediction performance of the ANN. In addition to RMSD, cross-correlation coefficients between actual and predicted force time histories were also calculated for anisometric experiment results. The RMSD values ranged from 0.34 and 0.02 in the isometric contraction experiments. In the anisometric contraction tests, RMSD results were between 0.23 and 0.09 and cross-correlation coefficients ranged from 0.91 to 0.98. In order to compare the performance of the HOFMs with a widely used EMG signal processing technique, root-mean-squared (RMS) values of the EMG signals were also calculated and used to train the ANN as another characterizing feature of the signal. Predicted forces using HOFMs technique were in general closer to the actual forces than those of obtained by using RMS values. The results indicated that the proposed signal processing method showed an encouraging performance for predicting the forces applied to the human hands, and the spectral features of the EMG signal might be used as input parameter for the myoelectric controlled prostheses.

Possible reflex pathway between medial meniscus and semimembranosus muscle: an experimental study in rabbits.

Meniscus is a well innervated tissue with four types of receptors. These receptors are mainly concentrated at the anterior and posterior horns. Although they are intended to be a part in reflex arc, this function has not been thoroughly evaluated. We hypothesized that electrical stimulation of the normal meniscus would elicit electromyographic activity of the hamstring muscle via the reflex arc. Five adult domestic male rabbits were used in this study. Under general anesthesia, knee arthrotomy and thigh dissection were done to expose medial meniscus and semimembranosus muscle. Menisci were stimulated by Teflon-coated bipolar needle electrodes. Needles were placed in the posterior horn of the medial menisci. Two Teflon-coated monopolar needle electrodes were placed in semimembranosus muscle. A four-channel electromyograph was used for recording. Two different potentials were recorded from the target muscle. The first response had a very short distal latency and its amplitude was changing in accordance with the strength of the stimulus, suggesting that this response was being elicited by direct muscle stimulation. The second delayed response with less amplitude also appeared in some traces. The latency and the amplitude of this second response were fairly stable stating that this delayed response was being generated by a reflex pathway and seen in all subjects.