Myasthenia gravis is a neuromuscular transmission function disorder characterized by fatigue and weakness of voluntary muscles. This muscular weakness is intensified by activity and stress, and improved by the use of anticholinesterase compounds. It was initially described by Erb in 1879 and later named myasthenia gravis by Jolly in 1895. Although the pathogenesis is Known to be an autoimmune related reduction in the number of available acetylcholine receptors at neuromuscular junctions, the role of thymus in myasthenia gravis is still unclear and under investigation. Thymectomy in the management of myasthenia gravis has become increasingly important since Dr. Blalock observed in 1939 that some patients with thymic tumors and myasthenia gravis improved following thymectomy. A clinical study of 102 cases of myasthenia gravis was performed at Yonsei University College of Medicine. Seoul, Korea from Jan. 1976 to Jun. 1986. In order to determine which factors are of prognostic significance, attention is focused upon pre-operative patient evaluation, problems in operative and post-operative care, and long-term follow-up observations. The results were as follows: 1. The sex distribution was 67 females and 35 males, the mean age of onset was 28.95*1.69 years, and the maximal incidence occurred between 21 and 40 years of age [56 cases: 54.9%]. 2. Clinical manifestations of ocular symptoms were seen to 70 patients [68.6%] extremities weakness in 33 [32.3%], bulbar weakness in 29 [28.4%], and dyspnea in 13 [12.7%]. 3. Study cases more than two thirds were classified as mild types [MG 1 and MG 11A] and 6 cases as grave [MG 1V] based on the modified Osserman`s classification system, 4. Thymectomy was performed in 19 cases which presented in severe myasthenia symptoms and showed no improvement with cholinergic drugs. Histologic examination of the excised thymus glands revealed no abnormalities in 4 cases, thymic hyperplasia in 5, benign thymoma in 5, and malignant thymoma in 5. 5. Immediate post-operative complications included 2 cases of pneumothorax which were treated by tube thoracostomies, there was no operative mortality. 6. The response to cholinergic drugs in 36 cases younger than 20 years old and in 27 cases older than 40 years was relatively poor, while that in 35 cases between the ages of 21 and 40 years old was good. 7. Thirty of 39 cases in groups IIB, III & IV improved markedly with medical or surgical management while only 16 of 59 cases in the mild groups [I and IIA] improved, almost all surgical cases improved in all categories. 8. There were 5 deaths. occurring between 7 months and 3 years 3 months of treatment of myasthenia gravis. The causes of death were myasthenic crisis in 2 cases, respiratory failure due to candidiasis & radiation pneumonitis in one case, cerebral hemorrhage due to high blood pressure in two case.
The Journal of Korean Academy of Orthopedic Manual Physical Therapy
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v.11
no.1
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pp.11-28
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2005
Spasticity has been defined as "a motor disorder characterized by a velocity-dependent increased in tonic stretch reflexes with exaggerated tendon jerks, resulting from hyperexcitability of the stretch reflex, as one components of the upper motorneuron syndrome". Spasticity is one of the common symptoms of stroke patients and frequently interferes with the motor functions such as gait, posture and activities of daily living. Therefore, its management is becoming a major issue in physical therapy. The purpose of this study was to determined the effects of reciprocal inhibition by isometric contraction of pre-tibia muscle on spasticity in hemiplegic patients through Hoffmann reflex. The subjects were consisted 45 patients who had hemiplegia due to stroke. All subjects randomly assigned to 3 group: manual reciprocal inhibition program group(manual group), neuromuscular electrical stimulation group(NMES group) and control group. The manual group received voluntary isometric contraction of pre-tibia muscle. The NMES group received neuromuscular electrical stimulation on tibialis anterior. The control group was not received any therapeutic intervention. Before and after experiments, Hoffmann reflex, M-wave and Modified Ashworth scale was measure in all patients. The data of 30 patients who complete experimental course were statistically analysed. Modified Ashworth scale were significantly decreased after experiment in manual group(p<.01). The Hmax/Mmax ratios were significantly decreased after experiment in manual group(p<.o1). There were no statistical difference between pre-test and post-test with modified Ashworth scale in NMES group(p>.01). There were no statistical difference between pre-test and post-test with Hmax/Mmax ratios in NMES group(p>.01). There were no statistical difference between pre-test and post-test with modified Ashworth scale in control group(p>.01). There were no statistical difference between pre-test and post-test with Hmax/Mmax ratios in control group(p>.01). The present results revealed that reciprocal inhibition which produced by voluntary isometric contraction of pre-tibia muscle can be reduce spasticity of gastrocnemius. Therefore, reciprocal inhibition is useful to improve functional activities in hemiplegic patient. Further study should be done to analyse the effects of intervention duration of reciprocal inhibition, appropriate muscle contraction, optimal time to apply the reciprocal inhibition in more long period.
Purpose: The neurodynamic test used to implicate symptoms arising from the nerve is proposed to selectively increase the strain of the nerve without increasing the strain of adjacent tissue, although this has not yet been established in the time of nerve tension application. This study aimed to investigate the acute effects of nerve stretching time on nerve excitability using compound nerve action potential (CNAP) analysis. Methods: Thirty healthy young adults (mean age=23.10 years) with no medical history of neurological or musculoskeletal disorder voluntarily participated in this study. Nerve excitability was assessed using the median nerve conduction velocity test. The amplitude of the CNAP was measured under three conditions: resting phase (supra-maximal stimulus, without nerve stretching), baseline phase (two-thirds of the supra-maximal stimulus, without nerve stretching), and stretch phase (two-thirds of the supra-maximal stimulus, with 1-5 minutes nerve stretching). One-way repeated measures ANOVA was conducted to compare the latency and amplitude of CNAP. A post-hoc test was analyzed using the contrast test. Results: The latency was significantly delayed after 1 min. of nerve stretching in comparison with the baseline test. However, no significant difference was found during the nerve stretching (1-5 min.). The amplitude was significantly increased by nerve stretching. Conclusion: Nerve stretching can induce nerve excitability without any nerve injury. Based on the results, more than 1 min. of nerve stretching as a neurodynamic test can be a useful method in the clinical setting.
Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant neuromuscular disorder which has been clinically shown to cause progressive weakness and result in atrophy of the facial muscles, shoulder girdle and upper arm muscles. The responsible gene for the FSHD has been located on chromosome 4q35-qter. The probes p13E-11 and pFR-1 detect DNA rearrangements associated with FSHD as under 28 kb DNA fragment in genomic southern analysis digested with EcoRI and the fragment contains 3.3 kb Kpn I tandem repeats. In this study, 4 fetuses with a family history of FSHD were analysed by genomic southern hybridization analysis with probes to determine whether they carried the deleted region. Of the 4 fetuses, three of them had mothers who were FSHD patients and the other one had a father affected with FSHD. After 10-11 weeks of gestation, we performed chorionic villi sampling and extracted DNA from uncultured and cultured tissue cells for the direct DNA analysis. The result of the southern analysis showed two fetuses having received about 15-18 kb of deleted genes from the father and the mother respectively, and found to be FSHD patients. The other two fetuses were shown to have two normal alleles from the parents and found to be normal. Two pregnancies which were determined to be normal were carried to term delivering two healthy babies.
Amyotrophic lateral sclerosis (ALS) is a progressive disorder that causes degeneration of motor neurons of the brain and spinal cord. ALS is a progressive, fatal neuromuscular disease characterized by loss of motor neurons leading to muscle weakness. Sensation and mental function stay intact during the course of the disease. ALS is characterized by both upper and lower motor neuron damage. Diagnosis includes magnetic response imaging (MRI) electromyogram (EMG), muscle biopsy, and blood test. There is no cure for ALS. We recently observed three cases of ALS. The patients were diagnosis with ALS by EMG and symptoms. This report was conducted to evaluate how oriental medical treatment can affect ALS. We report the change of their symptoms through oriental medical treatment compared with taking riluzole.
The prevalence of pediatric obstructive sleep apnea syndrome (OSAS) is approximately 3% in children. Adenotonsillar hypertrophy is the most common cause of OSAS in children, and obesity, hypotonic neuromuscular diseases, and craniofacial anomalies are other major risk factors. Snoring is the most common presenting complaint in children with OSAS, but the clinical presentation varies according to age. Agitated sleep with frequent postural changes, excessive sweating, or abnormal sleep positions such as hyperextension of neck or abnormal prone position may suggest a sleep-disordered breathing. Night terror, sleepwalking, and enuresis are frequently associated, during slow-wave sleep, with sleep-disordered breathing. Excessive daytime sleepiness becomes apparent in older children, whereas hyperactivity or inattention is usually predominant in younger children. Morning headache and poor appetite may also be present. As the cortical arousal threshold is higher in children, arousals are not easily developed and their sleep architectures are usually more conserved than those of adults. Untreated OSAS in children may result in various problems such as cognitive deficits, attention deficit/hyperactivity disorder, poor academic achievement, and emotional instability. Mild pulmonary hypertension is not uncommon. Rarely, cardiovascular complications such as cor pulmonale, heart failure, and systemic hypertension may develop in untreated cases. Failure to thrive and delayed development are serious problems in younger children with OSAS. Diagnosis of pediatric OSAS should be based on snoring, relevant history of sleep disruption, findings of any narrow or collapsible portions of upper airway, and confirmed by polysomnography. Early diagnosis of pediatric OSAS is critical to prevent complications with appropriate interventions.
Purpose: This study was conducted to predict the risks that arise while standing on mediolateral ramps at various ramp angles by identifying the ratio of medial to lateral gastrocnemius muscle activities. Methods: The subjects were 20 healthy adult men. Seven mediolateral ramp angles ($0^{\circ}$, $2^{\circ}$, $5^{\circ}$, $10^{\circ}$, $15^{\circ}$, $20^{\circ}$, and $25^{\circ}$) were applied for the experiment. The ratio of medial to lateral gastrocnemius muscle activities in each condition was measured using electromyography, and the measured data were converted to root mean square values to calculate the activity ratios. Results: The study results showed statistically significant differences in the ratio of mediolateral gastrocnemius muscle activities according to the selected mediolateral ramp angles. The results of a post hoc test confirmed that the multifidus muscles were asymmetrically used on the two sides at mediolateral ramp angles of $5^{\circ}$ or higher. Conclusion: This study found that an asymmetric use of the multifidus muscles began at a mediolateral ramp angle of $5^{\circ}$, and the provision of propulsion using the ankle joints on mediolateral ramps at $5^{\circ}$ or steeper was maneuvered differently from that on flat ground. This suggests that individuals with balance control disorder have the risk of falling due to ankle sprains and unstable balance control. Therefore, patients and elderly people are required to exercise caution when crossing $5^{\circ}$ or steeper ramps.
Churg-Strauss syndrome(CSS) is a systemic vascular disorder that has an unknown cause with multiorgan involvement and diverse presentations. The three main histologically distinct phases were necrotizing vasculitis, tissue eosinophilia and extravascular granulomas. A diagnosis of CSS can be made on four or more of the following six criteria : 1) asthma, 2) peripheral eosinophilia >10% on the differential leukocyte count, 3) mononeuropathy (including multiple) or polyneuropathy, 4) paranasal sinus abnormalities, 5) nonfixed pulmonary infiltrates and 6) biopsy evidence of extravascular eosinophils in the skin, the nerves, or the lungs. CSS has a good prognosis with systemic steroid therapy. The 5 year survival is approximately 70%. We experienced a 66-year-old man who presented with cough, sputum, edema and numbness in both legs. He presented with all of the 6 CSS criteria. A nerve and muscle biopsy confirmed the diagnosis. Here, we report this case with a review of the relevant literatures.
Kim, So Young;Oh, Hyo Jeong;Hwang, Ki Eun;Jung, Jong Hoon;Kim, Hak Ryul;Yang, Sei Hoon;Cho, Kwang Ho;Jeong, Eun Taik
Tuberculosis and Respiratory Diseases
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v.59
no.4
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pp.427-431
/
2005
Lambert-Eaton myasthenic syndrome (LEMS), a rare autoimmune neurological syndrome, is caused by defects in the secretion of acetylcholine from the presynaptic membrane, and is associated with the destruction of voltage gated calcium channels (VGCC) in the neuromuscular junction. LEMS can be confirmed by repetitive nerve stimulation and by the clinical symptoms, which are characterized by proximal muscle weakness in the lower extremities, decreased deep tendon reflexes and autonomic dysfunctions. In about 60% of patients with this disorder, underlying cancer-small cell lung cancer may be detected. Clinical symptoms may precede the diagnosis of malignancy, with the early diagnosis and treatment of the underlying malignancy being possible through the diagnosis of LEMS. A case of LEMS, with positive VGCC antibodies, in a 48-year-old man, which improved after chemotherapy of the underlying small cell lung cancer, is reported.
Purpose: Temporomandibular disorder (TMD) is a common musculoskeletal problem that causes pain in and disability of masticatory muscles, the temporo-mandibular joint (TMJ), and related structures. The purpose of this study was to compare pressure pain thresholds (PPTs) of masticatory muscles, cervical ranges of motion (ROM), and pelvic mobility during gait of subjects with or without TMD. Methods: In this study, pain thresholds and changes in the mobility of the cervical vertebrae and pelvis were measured in 25 patients with TMD and 25 healthy controls. Using a pressure algometer, the pressure pain thresholds (PPTs) of the masseter and temporalis muscles were measured in both groups. A gyroscope sensor with a mobile application was used to determine cervical ROM in the frontal and sagittal planes. A 3D-motion analysis system was used to evaluate pelvic mobility in the sagittal, frontal, and transverse planes during gait. Results: The TMD group showed significantly decreased PPTs of masseter and temporalis muscles compared with the control group (p < 0.05). Cervical ROM in flexion, extension, and lateral bending were significantly decreased in the TMD group compared with the control group (p < 0.05). In addition, antero-posterior pelvic tilt was significantly decreased in the TMD group (p < 0.05). Conclusion: The results of the current study suggest that there are close anatomical and functional relationships between TMD and muscle chains related to the cervical spine and pelvis. Therefore, more comprehensive body posture assessments, especially of painful areas, should be undertaken when studying TMD patients.
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