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Introduction/aims: It is unclear whether the revised European Academy of Neurology/Peripheral Nerve Society diagnostic criteria (EAN/PNS 2021 criteria) improved the diagnostic yield for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) compared with the previous version. Therefore, this study aimed to compare the sensitivity and specificity of the EAN/PNS 2021 criteria and the European Federation of Neurological Societies/Peripheral Nerve Society 2010 diagnostic criteria (EFNS/PNS 2010 criteria), with a specific focus on the electrodiagnostic criteria., Methods: Data of patients with clinically suspected CIDP who exhibited objective treatment response, and of those with chronic axonal neuropathies, obtained between 2009 and 2021, were extracted retrospectively from our database. Patients who underwent nerve conduction studies in at least unilateral upper and lower extremities were enrolled. We compared the sensitivity and specificity of the EAN/PNS 2021 and EFNS/PNS 2010 criteria., Results: In total, 55 patients with clinically suspected CIDP and 36 patients with chronic axonal neuropathies were enrolled. When considering the "possible CIDP" category, the EAN/PNS 2021 criteria showed lower sensitivity than the EFNS/PNS 2010 criteria (78% vs. 93%, p < .05), whereas its specificity was higher (78% vs. 61%, p < .05). The lower sensitivity was caused mainly by the failure to fulfill the sensory nerve conduction criteria. The revised definition of abnormal temporal dispersion of the tibial nerve contributed markedly to the improved specificity., Discussion: To improve the sensitivity of the EAN/PNS 2021 criteria, increasing the number of tested sensory nerves may be necessary., (© 2023 Wiley Periodicals LLC.)

Introduction: In chronic inflammatory demyelinating polyneuropathy (CIDP), exclusion of secondary axonal degeneration is challenging with conventional methods such as nerve conduction study (NCS), needle electromyography, and nerve biopsy. Increased echo intensity (EI) and decreased muscle thickness (MT) identified on muscle ultrasound (MUS) examination represent muscle denervation due to axonal degeneration in neurogenic disorders, suggesting MUS as a new tool to detect secondary axonal degeneration in patients with CIDP., Methods: EI and MT of abductor pollicis brevis, abductor digiti minimi, and first dorsal interosseous muscles were measured in 16 CIDP patients. Raw values were converted into z -scores using data from 60 normal controls (NCs)., Results: Six of 45 muscles showed abnormally high EI and low MT, suggesting denervation following secondary axonal degeneration. These six muscles belonged to two patients with long disease history, unresponsiveness to treatment, and long interval from onset to initial therapy. There were no significant differences in EI and MT ( p  = .23 and .67, respectively) between the CIDP and NC groups, although NCS results revealed obvious demyelinating abnormalities in all CIDP patients, suggesting the fact that muscle structures will be preserved, and EI and MT will not change unless secondary axonal degeneration occurs in CIDP., Conclusion: MUS is a promising tool for evaluating secondary axonal degeneration in patients with CIDP.

Introduction: The aim of this study was to formulate diagnostic hallmarks of axonal degeneration and demyelination in sensory nerve conduction studies (NCS)., Methods: We compared nerve conduction data obtained with surface electrode (SE) NCS and on-nerve needle (ONN) NCS in 50 cases of demyelination and 22 cases of axonal degeneration as assessed by sural nerve biopsy., Results: The overall diagnostic sensitivities of sensory nerve conduction were 26% by SE-NCS and 69% by ONN-NCS. The most helpful marker for demyelination was negative-peak nerve conduction velocity (NP-NCV), using a 36% decrease from the means in both techniques. Dispersion was also helpful in identifying demyelination. Low amplitude and absence of compound nerve action potential were indicative of general pathology in SE-NCS but of axonal degeneration in ONN-NCS., Conclusion: Although diagnostic sensitivity is low, NP-NCV and dispersion can be used for diagnosis of demyelination in sensory NCS. Muscle Nerve 53: 866-871, 2016., (© 2015 Wiley Periodicals, Inc.)

Introduction: We describe a previously unreported pitfall, spread of the stimulus at the elbow to the radial nerve, in an antidromic sensory nerve conduction study of the lateral antebrachial cutaneous (LAC) nerve., Methods: Subjects consisted of 80 healthy volunteers, and both sides were examined for each subject. Besides routine recording of the LAC nerve, sensory nerve action potentials (SNAPs) of the radial nerve were recorded distally., Results: The spread phenomenon occurred in 73 of 160 arms (46%), and the SNAP amplitude increased due to contamination of the radial SNAP up to 6.7 times the genuine LAC SNAP. In 10 arms (6%), the spread started before the LAC SNAP was saturated, and the genuine LAC SNAP was unknown due to an anatomical variation in at least 1 arm., Conclusions: Without monitoring distal radial SNAPs, the spread phenomenon will remain unrecognized. This pitfall undermines the reliability of the test., (Copyright © 2013 Wiley Periodicals, Inc.)

Introduction: Far-field potentials (FFPs) from muscles other than the abductor digiti minimi (ADM) may interfere with motor unit number estimation (MUNE) from that muscle., Methods: We identified the origin of each surface motor unit potential (SMUP) during hypothenar MUNE using the multiple point stimulation method in 20 control subjects by recording from individual ulnar-innervated muscles with a common proximal reference (pref)., Results: ADM SMUPs comprised 39.0% of the accepted SMUPs, followed by those from the fourth dorsal interosseous muscle (14.0%), the fourth lumbrical muscle (9.2%), and the second and third palmar interosseous muscles (8.8% each). The percentage of ADM SMUPs varied from 18% to 73% of accepted SMUPs among individual subjects. Accepted non-ADM SMUPs were usually much smaller than ADM SMUPs, and many more non-ADM SMUPs were excluded due to their small size., Conclusions: A large contribution from non-ADM or non-hypothenar SMUPs obscures the meaning of the MUNE value., (© 2013 Wiley Periodicals, Inc.)

Introduction: The repetitive nerve stimulation (RNS) test in the trapezius muscle is used widely for the evaluation of myasthenia gravis. However, pseudofacilitation is often difficult to avoid in this muscle and may compromise the detection of small decremental responses. We have devised a new maneuver to reduce pseudofacilitation., Methods: Using our maneuver, the shoulder of a supine subject is elevated passively and is held firmly by the examiner. Four conventional maneuvers as well as ours were compared with regard to pseudofacilitation that was maximal at the second wave in 14 control subjects., Results: Pseudofacilitation at the second and fourth waves was the smallest using our maneuver. Up to 15% pseudofacilitation was observed using the other maneuvers., Conclusion: Pseudofacilitation in the trapezius muscle is mainly due to shortening of the muscle belly. It can be reduced greatly by shortening the muscle in advance., (Copyright © 2012 Wiley Periodicals, Inc.)

Introduction: In this investigation we report on the clinical utility of sensory nerve conduction studies of the medial femoral cutaneous (MFC) nerve., Methods: Sensory nerve conduction of the MFC nerve was assessed in 22 patients for whom this test was considered clinically necessary., Results: MFC nerve conduction was abnormal in 4 cases of MFC neuropathy. The most common cause was iatrogenic in 14 femoral neuropathy cases. MFC nerve conduction showed absent or low-amplitude sensory nerve action potential (SNAP) in 13 cases, with femoral motor nerve conduction abnormal in 5 cases. In 2 cases with acute lumbar plexopathy, the MFC SNAP was absent unilaterally. MFC nerve conduction was normal in 1 case with diabetic lumbar radiculopathy and in another case with postpolio syndrome., Conclusion: Assessment of MFC nerve conduction is extremely useful in the diagnosis of femoral neuropathy, medial femoral cutaneous neuropathy, and lumbar plexopathy., (Copyright © 2011 Wiley Periodicals, Inc.)

Introduction: The role of fasciculation potentials (FPs) in the diagnosis of amyotrophic lateral sclerosis (ALS) has been underrated. The Awaji algorithm has restored the value of FPs. Our aim was to test the diagnostic yield of the Awaji algorithm, with consideration of FPs., Methods: Subjects consisted of 139 consecutive ALS patients retrospectively enrolled over 5 years. At presentation we evaluated the diagnostic categories using the revised El Escorial Criteria (R-EEC) and the Awaji algorithm., Results: The percentage of patients classified as confirmed ALS, clinically probable (laboratory-supported), or higher was 43% using the R-EEC and 37% using the Awaji algorithm. Thirteen patients with upper motor neuron signs only in one body region showed a decrease in their category using the Awaji algorithm. FPs were observed in 89% of ALS patients and were frequent in proximal muscles., Conclusion: The sensitivity of the Awaji algorithm is lower than that of the R-EEC., (Copyright © 2011 Wiley Periodicals, Inc.)

Introduction: The compound muscle action potential (CMAP) following ulnar nerve stimulation receives a considerable contribution from far-field potentials (FFPs), although their origin is not entirely clear. We investigated this issue using voluntary contractions., Methods: In 7 control subjects, we placed multiple recording electrodes over the motor points of ulnar-innervated muscles. We asked the subjects to perform a weak movement corresponding to the action of each muscle, and identified the single motor unit potentials (MUPs) from that muscle. We summed the MUPs from each individual muscle and reconstructed the CMAPs., Results: The reconstructed CMAPs coincided well with the actual ones. The N1, P1, and early N2 components of the FFPs were generated primarily by palmar, but also by dorsal, interosseous muscles. The abductor digiti minimi muscle usually generated positive-negative biphasic FFPs, and the negative FFP generated the late N2 component., Conclusions: These results should prompt a revision of the theory of FFP generation., (Copyright © 2010 Wiley Periodicals, Inc.)

To determine the conversion factor (CF) of the sural nerve the correlation between the maximum nerve conduction velocity (NCV) and the diameter of the largest fibers was studied in 30 patients suspected of having neuropathy. Sensory nerve action potentials were obtained by on-nerve needle nerve conduction study using needle electrodes placed on the exposed sural nerve during biopsy. The CF was 4.3 (n = 2) in normal sural nerves and close to the normal value (3.85, n = 4) in axonal neuropathy. The CF in demyelinating neuropathy was smaller than the normal value (2.77, n = 24), indicating disproportionately slower conduction than expected from the diameter of nerve fibers. The CF was helpful in differentiating between demyelinating and axonal neuropathies. We propose that a 36% decrease from the mean value of NCV is a reasonable criterion for demyelination of the nerve.

The purpose of this study was to investigate the clinical and electrophysiological efficacy of 3,4-diaminopyridine (DAP) in patients with Lambert-Eaton myasthenic syndrome (LEMS) in a randomized, double-blind, cross-over drug trial. The diagnosis of LEMS was made based on the combination of fluctuating muscle weakness, diminished or absent reflexes, and more than 60% increment of the compound muscle action potential (CMAP) amplitude after brief exercise or 50-HZ stimulation on a repetitive nerve stimulation (RNS) test. Evaluations were done at baseline, with placebo, and with 3,4-DAP (up to 75-80 mg/day). Assignment of placebo or 3,4-DAP was done in a double-blinded manner. Measurements included subjective symptoms score, objective clinical measurements [LEMS classification, muscle strength score, quantitative myasthenia gravis (QMG) score] and RNS test and single-fiber electromyography (SFEMG). The differences between placebo and baseline values (placebo change) were compared with the differences between 3,4-DAP and baseline or placebo values (DAP change). Seven patients with LEMS (QMG score >9) participated in the study. One patient had major side-effects with 3,4-DAP and withdrew from the study. Statistically significant efficacy was noted with DAP change (N = 13) compared with placebo change (N = 7) according to the subjective symptoms score (P = 0.01), LEMS classification (P < 0.001), muscle strength score (P < 0.006), QMG score (P = 0.02), and CMAP (P = 0.03). For long-term treatment, 2 patients preferred 3,4-DAP, 1 chose guanidine hydrochloride, 1 preferred pyridostigmine, and 2 chose no treatment. A randomized, double-blind, cross-over drug trial of 3,4-DAP showed significant efficacy over placebo in patients with LEMS. As a long-term treatment, however, not all patients preferred this drug.

Demographic, clinical, and laboratory features were compared in 235 white and African-American (AA) patients with myasthenia gravis (MG) at the University of Alabama at Birmingham Neuromuscular Disease Clinic from May 2003 to January 2008. Seventy nine percent of patients were white. Acetylcholine receptor antibody was positive in 71% of white patients and in 59% of AA. In patients with seronegative generalized MG, the rate of positive muscle-specific tyrosine kinase antibody (MuSK-Ab) was significantly higher in AA than it was in whites (50% in AA vs. 17% in whites). Ocular MG was seronegative in 75% of AA patients. In AA, MG occurred earlier and more frequently in females, whereas, in whites, disease onset was later and more common in males. Another significant difference was a higher percentage of abnormality on repetitive nerve stimulation in AA. There was also a tendency for more severe forms of MG in AA. There are racial differences in MG between whites and AA in Alabama. These racial differences highlight the need to study biological factors in the pathogenesis of MG and to assess different approaches in diagnosis and treatment.

Needle electromyography (EMG) of the tongue is traditionally used as a key to the diagnosis of amyotrophic lateral sclerosis (ALS), although relaxation of the tongue is often difficult to achieve. Recently, frequent abnormalities in the EMGs of the sternocleidomastoid (SCM) and upper trapezius muscles in ALS have been reported. To elucidate the diagnostic utility of these muscles we performed a multicenter prospective study to examine EMGs of the tongue (genioglossus), SCM, and trapezius in 104 ALS or suspected ALS patients. We also examined EMGs of the SCM and trapezius in 32 cervical spondylosis (CS) patients. We mainly evaluated fibrillation potentials/positive sharp waves (Fib/PSWs) and fasciculation potentials. Complete relaxation was achieved in 85% of ALS patients in the trapezius, but in only 6% of patients in the tongue. Fib/PSWs were observed in 8%, 13%, and 45% of ALS patients in the tongue, SCM, and trapezius, respectively, whereas fasciculation potentials were observed in 1%, 7%, and 39%, respectively. Abnormal spontaneous activity of any type was found in 9%, 17%, and 63% of patients, respectively. The high frequency of abnormal spontaneous activity in the trapezius was similar among the different diagnostic categories, and even 72% of clinically suspected ALS (progressive muscular atrophy) patients showed them in their trapezius. We did not observe Fib/PSWs or fasciculation potentials in any of our CS patients, thus these findings have excellent specificity. Tongue EMG added little utility over the clinical sign of tongue atrophy. Abnormal spontaneous activity in the trapezius would be more useful for the early diagnosis of ALS.

An incremental response after brief exercise or high-rate stimulation on the repetitive nerve stimulation (RNS) test is a critical diagnostic criterion for Lambert-Eaton myasthenic syndrome (LEMS). This prospective study was performed to determine what duration of exercise shows the highest diagnostic sensitivity for LEMS. The compound muscle action potential amplitude in the abductor digiti quinti muscle was obtained at rest and after 5 s, 10 s, 15 s, 20 s, 25 s, and 30 s of exercise. Incremental responses were compared for the different exercise durations in 24 studies performed in nine LEMS patients. The increment was highest with 5-s and 10-s exercises (244%-243%) and lowest with 30-s exercise (84%). A gradual decrease in the increment was noted from 5- to 30-s exercise. A significant difference in the increment was noted between 5- to 10-s and 20-s to 30-s exercise. There was significantly higher diagnostic sensitivity with the 10-s exercise compared with 30-s exercise at 100% increment and 60% increment levels. Higher increment and diagnostic sensitivity were achieved with 10-s exercise than with 30-s exercise. Thus, 10-s exercise should be the standard protocol for the RNS test for LEMS.

Previous studies suggest that in amyotrophic lateral sclerosis (ALS) the abductor pollicis brevis (APB) and first dorsal interosseous (FDI) are more severely involved than abductor digiti minimi (ADM). To elucidate the pattern, frequency, extent, and specificity of such dissociated muscle atrophy in ALS, compound muscle action potentials recorded from APB, FDI, and ADM were analyzed in 77 ALS patients, 171 normal controls, and 196 disease controls. Compared with normal controls, ALS patients had a reduced APB/ADM amplitude ratio (P < 0.001) and FDI/ADM ratio (P < 0.001), whereas patients with other anterior horn diseases showed similar APB/ADM and FDI/ADM ratios to normal values. Decreased APB/ADM ratio was found in 41% of ALS patients, 5% of normal controls, and 4% of disease controls. Prominent muscle atrophy in APB and FDI, with relatively preserved ADM, appears to be specific to ALS. Dissociated hand muscle atrophy presumably reflects part of the pathophysiology and supports the diagnosis of ALS.

In order to determine whether there is any difference between voltage-gated calcium-channel antibody (VGCC-Ab)-positive and -negative groups in Lambert-Eaton myasthenic syndrome (LEMS), we compared the clinical and electrophysiological features between 13 patients with VGCC-Ab and 6 VGCC-Ab-negative patients. No obvious difference was observed in the various clinical features or findings on single-fiber electromyography between seropositive and seronegative cases. In seropositive cases, the compound muscle action potential (CMAP) amplitude was lower but the increment on post-exercise facilitation (PEF) and high-rate stimulation (HRS) was significantly higher than in the seronegative group, indicating that the repetitive nerve stimulation (RNS) test in the seropositive group is more typical of LEMS and more severe. A 100% increment as the diagnostic criterion in the routine RNS test was satisfied in all seropositive cases but in only three seronegative cases, whereas a 60% increment as the diagnostic criterion was found in all seronegative cases. The classic triad (low CMAP amplitude, decrement at low rate of stimulation, and increment at PEF or HRS) of RNS is rare, adding to the difficulty in diagnosing LEMS in the seronegative group, and making a 60% increment criterion more critical for the diagnosis of this disorder.

To better define electrophysiological abnormalities in myasthenia gravis (MG) patients with muscle-specific tyrosine kinase (MuSK) antibodies (Ab), we compared electrophysiological features of 14 MuSK Ab-positive, 73 acetylcholine receptor antibody (AChR Ab)-positive, and 22 MuSK and AChR Ab-negative (seronegative) patients with generalized disease. Repetitive nerve stimulation (RNS) abnormalities were observed in 86% of MuSK Ab-positive and 82% of AChR Ab-positive patients but in only 55% of seronegative patients. RNS decrements in the orbicularis oculi were more common and severe in the MuSK Ab-positive patients than the other two groups. Single-fiber electromyography (SFEMG) of the extensor digitorum communis was abnormal in 90% of MuSK Ab-positive patients. The high frequency of RNS abnormalities in facial muscles in the MuSK Ab-positive population reflects the propensity for facial muscle involvement in this form of MG and emphasizes the importance of including facial muscles in RNS protocols when evaluating these patients.