In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.
Respiratory Insufficiency/etiology/*rehabilitation/*therapy ; Respiration, Artificial ; Neuromuscular Diseases/complications/*rehabilitation ; Humans ; Breathing Exercises

In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.
Respiratory Insufficiency/etiology/*rehabilitation/*therapy ; Respiration, Artificial ; Neuromuscular Diseases/complications/*rehabilitation ; Humans ; Breathing Exercises

PURPOSE: For patients with neuromuscular disease, air stacking, which inflates the lungs to deep volumes, is important for many reasons. However, neuromuscular patients with severe glottic dysfunction or indwelling tracheostomy tubes cannot air stack effectively. For these patients, we developed a device that permits deep lung insufflations substituting for glottic function. MATERIALS AND METHODS: Thirty-seven patients with bulbar-innervated muscle weakness and/or tracheostomies were recruited. Twenty-three had amyotrophic lateral sclerosis, and 14 were tetraplegic patients due to cervical spinal cord injury. An artificial external glottic device (AEGD) was used to permit passive deep lung insufflation. In order to confirm the utility of AEGD, vital capacity, maximum insufflation capacity (MIC), and lung insufflation capacity (LIC) with AEGD (LICA) were measured. RESULTS: For 30 patients, MICs were initially zero. However, with the use of the AEGD, LICA was measurable for all patients. The mean LICA was 1,622.7+/-526.8 mL. Although MIC was measurable for the remaining 7 patients without utilizing the AEGD, it was significantly less than LICA, which was 1,084.3+/-259.9 mL and 1,862.9+/-248 mL, respectively (p<0.05). CONCLUSION: The AEGD permits lung insufflation by providing deeper lung volumes than possible by air stacking.
Adult ; Aged ; Female ; Forced Expiratory Flow Rates/physiology ; Humans ; Insufflation/*instrumentation/methods ; Male ; Middle Aged ; Neuromuscular Diseases/*physiopathology/therapy ; *Ventilators, Mechanical ; Vital Capacity/physiology ; Young Adult

Myasthenia gravis is an autoimmune disease involving neuromuscular junction. It can be associated with thymic hyperplasia, thymoma and various tumors involving thymus, but extrathymic malignant lymphoma associated with myasthenia gravis has been rarely reported. We experienced a 62-year-old woman diagnosed with ocular myasthenia gravis associated with small lymphocytic lymphoma without thymic involvement. She had complete remission after 6 cycles of chemotherapy, and symptoms of myasthenia gravis were completely resolved.
Autoimmune Diseases ; Drug Therapy ; Female ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lymphoma* ; Middle Aged ; Myasthenia Gravis* ; Neuromuscular Junction ; Thymoma ; Thymus Gland ; Thymus Hyperplasia

Hypothyroidism can cause a variety of signs and symptoms of the neuromuscular system. However, ptosis in a patient with hypothyroidism is very rare. We report here on a case of central hypothyroidism that was due to Sheehan's syndrome and it manifested as bilateral ptosis in a 51-yr-old woman. She complained of exertional dyspnea and weakness. About 25-yr ago, she had a history of severe postpartum vaginal bleeding. The laboratory studies demonstrated hypopituitarism with secondary hypothyroidism. The ptosis was improved by replacement of thyroid hormone. Hypothyroidism should be considered in the differential diagnosis of patients who manifest with ptosis and that prompt replacement of hormone can lead to a complete recovery.
Blepharoptosis/complications/*diagnosis/drug therapy ; Electromyography ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hypopituitarism/complications/*diagnosis/drug therapy ; Hypothyroidism/complications ; Magnetic Resonance Imaging ; Middle Aged ; Muscular Diseases/etiology ; Neuromuscular Junction/physiopathology ; Prednisolone/therapeutic use ; Thyroxine/therapeutic use

PURPOSE: To survey the use of invasive and noninvasive home mechanical ventilation (HMV) methods in South Korea from the perspective of physical medicine and rehabilitation (PM&R). MATERIALS AND METHODS: For 413 users of HMV, retrospective reviews of PM&R interventions and survey of HMV methods employed from Mar 2000 to Dec 2009. RESULTS: Of the 413 users, the majority of whom with progressive neuromuscular disorders (NMDs) (n=358), 284 patients initially used noninvasive mechanical ventilation (NIV), while 63 others who were using tracheostomy mechanical ventilation switched to NIV as part of their rehabilitation. The NMD patients began HMV at an earlier age (34.9+/-20.3 yrs), and used for longer (14.7+/-7.5) hours than patients with non-neuromuscular causes of respiratory impairment. CONCLUSION: Noninvasive management was preferred over invasive ones, and transition to the former was a result of PM&R interventions.
Adult ; Female ; Health Care Surveys ; Home Care Services/*statistics & numerical data ; Humans ; Male ; Middle Aged ; Muscular Dystrophies/*therapy ; Neuromuscular Diseases/therapy ; Republic of Korea ; Respiration, Artificial/*statistics & numerical data ; Retrospective Studies ; Ventilators, Mechanical/*statistics & numerical data

OBJECTIVE: To find the risk factors of critical illness polyneuropathy (CIP) on intensive care unit patients using early electrodiagnosis. METHOD: The adult patient who were admitted to the ICU and taken ventilator care with endotracheal intubation were included. The time after admission was 48 to 144 hours. In case of axonal neuropathy of peripheral nerve, if affected nerves were in different two limbs or different three nerves were affected, CIP was diagnosed. If some nerves got abnormal results but did not satisfied the above criteria, the patient was classified as peripheral neuropathy group. The days of using neuromuscular blockade, continuous insulin infusion, catecholamine, vasopressor, corticosteroid, benzodiazepine, parenteral nutrition and fact for continuous renal replacement therapy, SOFA (sequential organ failure assessment) score were evaluated to find the risk factors. RESULTS: Eighteen patients were included. Six patients were CIP and another six were peripheral neuropathy. Risk factors for CIP were age, duration of intensive care, days of neuromuscular blockade and parenteral nutrition (p<0.05). There was no difference on mortality rate among the three groups. CONCLUSION: The result of early electrodiagnosis on ICU patients for CIP diagnosis revealed that risk factors of CIP were age, duration of intensive care, days of neuromuscular blockade and parenteral nutrition.
Adult ; Axons ; Benzodiazepines ; Critical Illness ; Electrodiagnosis ; Extremities ; Humans ; Insulin ; Critical Care ; Intensive Care Units ; Intubation, Intratracheal ; Neuromuscular Blockade ; Parenteral Nutrition ; Peripheral Nerves ; Peripheral Nervous System Diseases ; Polyneuropathies ; Renal Replacement Therapy ; Risk Factors ; Ventilators, Mechanical