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1.
Introduction: More than 90% of amyotrophic lateral sclerosis (ALS) patients have muscle cramps, but evidence‐based treatments have not been available. Methods: A multicenter, double‐blind, placebo‐controlled crossover trial of mexiletine 150 mg twice daily was conducted in ALS patients requesting treatment of symptomatic muscle cramps. Results: Muscle cramp frequency was reduced in 18 of 20 patients; 13 reductions were attributed to treatment (P < 0.05). The average reduction, based on t tests, was 1.8 cramps per day (a reduction from 5.3 with placebo to 3.5 with mexiletine). The estimated reduction of cramp severity was 15 units on a 100‐unit scale (P = 0.01) from a baseline average of 46. No effect on fasciculations was noted. One patient discontinued the study because of dizziness, and another patient discontinued the study to start open‐label mexiletine therapy. No serious adverse event occurred. Discussion: Mexiletine is a well tolerated and effective medication for controlling the symptom of muscle cramps in ALS. Muscle Nerve 58 : 42–48, 2018  相似文献   

2.
Background: The aim of the study was to assess autoimmune involvement in amyotrophic lateral sclerosis (ALS). Methods: We measured IgG antibodies against light (NFL) and medium (NFM) subunits of neurofilaments using ELISA in paired cerebrospinal fluid (CSF) and serum samples from 38 ALS patients and 20 controls. Results: Serum levels of anti‐NFL were higher in ALS patients than in controls (P < 0.005). Serum anti‐NFL antibodies and intrathecal anti‐NFM antibodies were related to patient disability (serum anti‐NFL: P < 0.05; intrathecal anti‐NFM: P < 0.05). Anti‐NFL levels were significantly correlated with anti‐NFM levels in ALS (P < 0.001) and the control group (P < 0.0001) in the CSF, but not in serum. Anti‐NFL and anti‐NFM antibodies significantly correlated between serum and CSF in the ALS group (anti‐NFL: P < 0.0001; anti‐NFM: P < 0.001) and in the control group (anti‐NFL: P < 0.05; anti‐NFM: P < 0.05). Conclusions: Autoimmune humoral response to neurocytoskeletal proteins is associated with ALS.  相似文献   

3.
To explore the efficacy of muscle motor point stimulation in eliciting muscle cramps, 11 subjects underwent eight sessions of electrical stimulation of the following muscles bilaterally: abductor hallucis flexor hallucis brevis, and both heads of the gastrocnemius muscles. Bursts of 150 square wave stimuli (duration: 152 μs; current intensity: 30% supramaximal) were applied. The stimulation frequency was increased from 4 pulses per second (pps) at increments of 2 pps until a cramp was induced. The number of cramps that could be elicited was smaller in flexor hallucis brevis than in abductor hallucis (16 vs. 22 out of 22 trials each; P < 0.05) and in the lateral gastrocnemius than in the medial gastrocnemius (5 vs. 20 out of 22 trials each; P < 0.0001). We show that leg and foot muscles have different cramp susceptibility, and the intermuscle variability in the elicitability profile for electrically induced cramps supports the use of the proposed method for cramp research. Muscle Nerve, 2009  相似文献   

4.
Introduction: In this study we compared the electrodiagnostic (EDX) yield of limb muscles in revealing lower motor neuron (LMN) dysfunction by electromyography (EMG) in early‐stage amyotrophic lateral sclerosis (ALS). Methods: This investigation was undertaken as a retrospective review at a single center. Results: Our study included 122 consecutive patients with possible ALS, as defined by revised El Escorial criteria. Distal limb muscles showed more frequent EMG abnormalities than proximal muscles. EDX yield was found to be higher in the limb where weakness began and when clinical signs of LMN dysfunction were evident. Adoption of the Awaji criteria significantly increased the yield of EMG‐positive segments in the cervical (P < 0.0005) and lumbosacral (P < 0.0001) regions, and upgraded 19 patients into the probable category and 1 patient into the definite category. Discussion: EMG abnormalities are predominant in the distal limb in early‐stage ALS. A redefinition of an EDX‐positive cervical or lumbosacral segment, with an emphasis on distal limb muscles, may result in an earlier ALS diagnosis. Muscle Nerve 58 : 389–395, 2018  相似文献   

5.
By determining the usefulness of motor unit number estimate (MUNE) and compound muscle action potential (CMAP) amplitude in patients with amyotrophic lateral sclerosis (ALS), we tried to find an effective way to stratify the disease stages. In all, 112 consecutive ALS patients were enrolled, among whom 73 were elicited in a longitudinal study. MUNE by the standard incremental technique, the average CMAP amplitude, total Medical Research Council (MRC) score, ALS‐functional rating score (ALS‐FRS), Appel ALS rating scale (AARS), and forced vital capacity (FVC) were performed at baseline and months 3, 6, and 12 after study entry. We found MUNE correlated with CMAP amplitude (P < 0.01) as well as MRC score (P < 0.01) in regionally concordant distal muscles. Both MUNE and CMAP amplitude correlated significantly with ALS‐FRS (P < 0.05) and AARS (P < 0.01). A MUNE decrease was observed at months 3, 6, and 12 compared with baseline, and the rate of change at month 3 was 50.47%. The decrease in MUNE over the first 3 months was significantly greater than other measurements. We arbitrarily divided the patients into three stages: (1) rapid progression: the rate of change of MUNE and CMAP amplitude during the first 3 months exceeded 50%; (2) moderate progression: the rate of change of MUNE was greater than 50% but CMAP amplitude was less than 50%; (3) slow progression: the rate of change of both MUNE and CMAP amplitude were less than 50%. Comparing the rate of ALS‐FRS descent per year using one‐way ANOVA showed a significant difference among the three groups (P < 0.01). Muscle Nerve 39: 304–309, 2009  相似文献   

6.
Introduction: In this study we investigated the prognostic significance of cough peak flow (CPF) decline rate in patients with amyotrophic lateral sclerosis (ALS). Methods: Thirty-four patients with ALS participated in our investigation. We examined CPF, forced vital capacity (FVC), body mass index, and the revised ALS Functional Rating Scale (ALSFRS-R), and followed patients to death, tracheostomy, or non-invasive ventilator dependency. We analyzed the relationship between the rate of decline of each parameter and survival prognosis. Results: The CPF decline rate significantly correlated with the decline rates of the ALSFRS-R bulbar score (P < 0.0001) and FVC (P < 0.0001). Patients with a CPF decline rate ≥25% had shorter overall survival (P < 0.0001). Cox model multivariate analyses showed that the CPF decline rate was an independent prognostic factor for survival (P = 0.021). Discussion: The CPF decline rate reflects the progression of bulbar and respiratory dysfunction and predicts survival. Muscle Nerve 59 :168–173, 2019  相似文献   

7.
Introduction: Patients with myasthenia gravis often experience fatigue, but its effect on quality of life (QoL) is underestimated, and fatigue is rarely measured in clinical trials. Methods: Two hundred fifty‐seven myasthenic patients completed the Neuro‐QoL‐Fatigue and measures of disease severity and QoL. We studied the relationship between fatigue and clinical and demographic variables. Finally, we studied the responsiveness of the Neuro‐QoL‐Fatigue in 95 patients receiving treatments for myasthenia and estimated the minimal important difference (MID). Results: Fatigue correlated with greater disease severity (r = 0.52–0.69, P < 0.0001) and worse QoL (r = 0.65–0.75, P < 0.0001). Patients in remission, with minimal manifestations, and pure ocular symptoms reported minimal fatigue. Regression modeling showed that, in addition to its relationship with disease severity, fatigue was worse in females, patients with generalized disease, and those with anxiety/depression. Fatigue improved after immunomodulation (P < 0.0001), and the MID was 5.3 points. Discussion: Fatigue in myasthenia correlates with disease severity, affects QoL, and can improve after treatment. Muscle Nerve 58 : 197–203, 2018  相似文献   

8.
Introduction: PLS is defined as pure upper motor neuron disease/dysfunction (PUMND) beyond 48 months after symptom onset. We know little about its early stages, but such knowledge would help to identify the mechanisms underlying PLS and ALS and determine why PLS patients seem to be protected against lower MND (LMND). Methods: We reviewed 622 MND cases during a 4‐year period and identified 34 patients with PUMND (5.4%). Results: Among 23 cases with follow‐up data/electromyograms (EMGs; 2 had only 1 EMG), 13 (57%) remained classified as PUMND, and 8 (35%) developed LMND (mean, 51.4 months after onset). Of these 8, LMND developed in 3 after 48 months from symptom onset. Patients with PUMND and LMND were more functionally impaired (P = 0.02). Separately, we identified 5 patients with PUMND who developed LMND long after 48 months (range, 50–127 months). Conclusions: PLS belongs to the ALS spectrum, and perhaps all cases eventually develop LMND. Muscle Nerve, 2013  相似文献   

9.
Introduction: We investigated the functional and structural integrity of the corticospinal tract (CST) using diffusion tensor imaging (DTI) and the triple stimulation technique (TST) in patients with amyotrophic lateral sclerosis (ALS). Methods: Fourteen patients with ALS, 13 healthy controls (HCs), and 6 patients with lower motor neuron (LMN) syndrome underwent DTI and TST. Results: The mean diffusivity was higher in ALS patients than HCs (P < 0.01). The TST ratio was lower in ALS patients compared with HCs (P < 0.001) and in LMN patients compared with HCs (P < 0.05). The increase in the mean diffusivity was correlated with the decrease in the TST ratio (P < 0.01). Conclusions: Significant correlations exist between the DTI and TST results, indicating both structural and functional involvement of the CST in patients with ALS. Muscle Nerve 49:551–557, 2014  相似文献   

10.
Introduction: The distribution of clinical and neurophysiological abnormalities in patients with early amyotrophic lateral sclerosis (ALS) was investigated in an attempt to delineate patterns of disease spread. Methods: Clinical and electrodiagnostic data were collected from 150 ALS patients and analyzed based on the clinical region of onset. Results: Asymmetry of clinical and neurophysiological abnormalities was more marked in upper limb‐onset than lower limb‐onset disease. Significant rostral–caudal gradients of clinical weakness were identified in bulbar‐ and lower limb‐onset disease. Neurophysiological evidence of the ALS “split‐hand” pattern was evident irrespective of the region of disease onset. Limbs with and without evidence of clinical weakness demonstrated similar rates of abnormality on electromyography. Conclusions: These findings suggest a pattern of disease spread in ALS. This study may serve to guide ongoing development of disease quantitation biomarkers and the targeting of future neuroprotective strategies. Muscle Nerve 50: 894–899, 2014  相似文献   

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