No abstract available.
Amyotrophic Lateral Sclerosis* ; Charcot-Marie-Tooth Disease* ; China* ; Pedigree*

BACKGROUND AND OBJECTIVES: Amyotropic lateral sclerosis (ALS) is a progressive neuromuscular disease that involves degeneration of upper motor neurons in the cortex and lower motor neurons in the brain stem and spinal cord. Although there is a decline in the articulatory dysfunction, respiratory, and phonatory dysfunctions are known to occur in ALS. However, the pattern of disease progression and early identification of phonatory changes are not well documented. The purpose of this study was to compare selected acoustic parameters of voice in the normal control group to the ALS group who were perceptually free of vocal symptoms. Significant differences between the two groups could indicate that signs of bulbar degeneration in ALS may be present even when the voice is clinically perceived as normal. MATERIALS AND METHODS: The authors used a computerized acoustic analysis program (CSL-MDVP) to measure mean fundamental frequency (Fo), jitter, shimmer, and noise to harmonics ratio (NHR) from voice samples of ALS patients and compared this results with the acoustic parameters of 60 control subjects (men: 30, women: 30) who have no history of neurologic disease and were over 60 years old. RESULTS: There were significant group differences for jitter, shimmer, and NHR in male ALS. There was significant group difference for jitter in female ALS. CONCLUSION: Certain acoustic measures of voice may be sensitive indicators of early laryngeal deterioration in ALS. Acoustic analysis of voice may be one way of detecting aberrant vocal features before the human ear detects dysphonia, and jitter may be the most sensitive acoustic variable.
Acoustics* ; Amyotrophic Lateral Sclerosis* ; Brain Stem ; Disease Progression ; Dysphonia ; Ear ; Female ; Humans ; Male ; Middle Aged ; Motor Neuron Disease ; Motor Neurons ; Neuromuscular Diseases ; Noise ; Spinal Cord ; Voice*

No abstract available.
Carpal Tunnel Syndrome* ; Diabetic Neuropathies*

BACKGROUND AND PURPOSE: Evaluating respiratory function is important in neuromuscular diseases. This study explored the reference ranges of the maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and sniff nasal inspiratory pressure (SNIP) in healthy adults, and applied them to amyotrophic lateral sclerosis (ALS) patients. METHODS: MIP, MEP, and SNIP were measured in 67 healthy volunteers aged from 21 to 82 years. Reference ranges were evaluated by multivariate regression analysis using the generalized additive modeling of location, scale, and shape method. Thirty-six ALS patients were reviewed retrospectively, and abnormal values of MIP, MEP, and SNIP were determined according to the reference ranges. RESULTS: MIP, MEP, and SNIP were abnormal in 57.1%, 51.4%, and 25.7% of the ALS patients, respectively. MIP and SNIP were significantly correlated with the degree of restrictive pattern and respiratory symptoms. The ALS Functional Rating Scale-Revised score was correlated with SNIP. CONCLUSIONS: This study has provided the reference range of respiratory muscle strength in healthy adults. This range is suitable for evaluating respiratory function in ALS patients.
Adult ; Amyotrophic Lateral Sclerosis* ; Healthy Volunteers ; Humans ; Methods ; Neuromuscular Diseases ; Reference Values* ; Respiratory Muscles* ; Retrospective Studies

OBJECTIVE: To evaluate the difference in vital capacity (VC) between sitting and supine position in patients with amyotrophic lateral sclerosis (ALS) and Duchenne muscular dystrophy (DMD). METHOD: VC was measured in the sitting and supine position for 30 patients with DMD and for 30 patients with ALS. The highest value in three or more attempts in each position was chosen. RESULTS: VCs measured in ALS patients in the sitting and supine position were 1591.7+/-634.6 ml and 1290.0+/-580.3 ml respectively. The VC in the sitting position showed significantly higher value than the VC in the supine position (p<0.05). VCs measured in DMD patients were 903.7+/-518.1 ml and 795.3+/-505.6 ml respectively. There was no statistically significant difference between the measurements in both positions. CONCLUSION: Difference in postural change of VC was observed in patients with different types of neuromuscular disorders. Such difference in VC suggest that postural change of VC should be considered in management of neuromuscular disease with respiratory muscle weakness.
Amyotrophic Lateral Sclerosis ; Humans ; Muscular Dystrophy, Duchenne ; Neuromuscular Diseases* ; Respiratory Muscles ; Supine Position ; Vital Capacity*

BACKGROUND: Motor conduction slowing in diabetic distal symmetrical polyneuropathy (DSP) generally exceeds that in distal axonal polyneuropathy. Additional mechanisms secondary to axonal injury may contribute towards this slowing. However, clinical and pathophysiological significances of motor conduction slowing have been rarely discussed. The purpose of this study is to evaluate the clinical and pathophysiological significance of conduction slowing in DSP. METHODS: We analyzed motor conduction studies of 39 patients with symptomatic painful DSP and 24 patients with asymptomatic painless DSP. Motor conduction studies of 39 patients with amyotrophic lateral sclerosis (ALS) were used as controls for the amplitude-dependent slowing of conduction. Percentages of normal limits were calculated for the compound muscle action potential amplitude (CMAP), distal motor latency (DL), and conduction velocity (CV), and converted to a square root (SQRT) form. The changes of SQRT-DL or SQRT-CV according to SQRT-CMAP changes were plotted and analyzed. RESULTS: Regression analysis showed that DL and CV were amplitude-dependent in both painless DSP and ALS. The changes of DL and CV in painful DSP did not show amplitude-dependency except DL in the lower extremities. CONCLUSIONS: This data supports the hypothesis that the mechanism of slowing is similar in both painless DSP and ALS and results from the loss of large, fast-conducting fibers. Lack of amplitude-dependency of conduction slowing in painful DSP may reflect the combined axonal and demyelinating changes, possibly due to inflammation.
Action Potentials ; Amyotrophic Lateral Sclerosis ; Axons ; Diabetic Neuropathies* ; Electrophysiology ; Humans ; Inflammation ; Lower Extremity ; Neural Conduction ; Polyneuropathies

OBJECTIVE: The purpose of this study was to evaluate therapeutic effect of local steroid injection in carpal tunnel syndrome, and to make a comparison between therapeutic effect in patients with and without diabetic neuropathy. METHOD: 30 patients (40 hands) with carpal tunnel syndrome diagnosed clinically and electrophysiologically were injected with 40mg of methylprednisone. Patients were evaluated with the visual analogue scale after 4 weeks and 8 weeks. According to the therapeutic responses, the patients were grouped into: excellent; good; poor; failed; recurrent. RESULTS: After 4 weeks, symptom relief was noted in the 95% of all cases: 100% of the patients without diabetic neuropathy; 82% of the patients with diabetic neuropathy. After 8 weeks, symptom relief was noted in the 82.5% of all cases: 86% of the patients without diabetic neuropathy; 73% of the patients with diabetic neuropathy. There was no statistically significant difference between the patients with and without diabetic neuropathy (p>0.05). CONCLUSION: We concluded that local steroid injection in carpal tunnel syndrome was an effective therapeutic modality for a short term and local steroid injection in the carpal tunnel syndrome with diabetic neuropathy diagnosed by palmar test also had a good effect.
Carpal Tunnel Syndrome* ; Diabetic Neuropathies ; Humans

No abstract available.
Botulism*

No abstract available.
Botulism*

OBJECTIVE: To evaluate the difference in forced vital capacity (FVC) between sitting and supine position in patients with amyotrophic lateral sclerosis (ALS), cervical spinal cord injury (SCI) and Duchenne muscular dystrophy (DMD). METHOD: FVC was measured in sitting and supine position for 32 patients with DMD, 32 patients with cervical SCI and for 28 patients with ALS. The highest value in three or more attempts in each position was chosen. RESULTS: FVCs measured in cervical SCI and ALS patients in the sitting and supine position were 1612.8+/-291.0 ml, 1393.2+/-286.7 ml and 2054.7+/-545.8 ml, 1104.3+/-425.4 ml respectively. Cervical SCI patients showed significantly higher value in the supine position (p<0.05). And ALS patients showed significantly higher value in the sitting position (p<0.05). FVCs measured in DMD patients were 1311.6+/-260.7 ml and 1213.8+/-378.9 ml respectively. There was no statistically significant difference between the measurements in both positions. CONCLUSION: Difference in postural change of FVC was observed in patients with different types of neuromuscular disorders. Such difference in FVC suggest that postural change of FVC should be considered in management of neuromuscular disease with respiratory muscle weakness.
Amyotrophic Lateral Sclerosis ; Humans ; Muscular Dystrophy, Duchenne ; Neuromuscular Diseases* ; Posture* ; Respiratory Muscles ; Spinal Cord Injuries ; Supine Position ; Vital Capacity*