Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 66 year old man whose activity was almost normal underwent a lombar sympathectomy under general anesthesia (thiopental, gallamine, N2O). Immediately after recovery an acute respiratory distress became evident. It was due to a respiratory paralysis on which neostigmine had no effect. Neurologic examination showed the symptoms of an amyotrophic lateral sclerosis which had remained unknown so far. The respiratory paralysis persisted and one year later artificial ventilation is still necessary almost continuously. Two aspects of this very rare observation are discussed: first the cause and mecanisms of the aggravation of the neurologic disease following anesthesia, secondly the rather unusually important part played by the respiratory paralysis in this case of lateral amyotrophic sclerosis.
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PMID:[Aggravation following anaesthesia in a case of unknown lateral amyotrophic sclerosis (author's transl)]. 49 45

Reported herein is the experience of the authors with 38 amyotrophic lateral sclerosis (ALS) patients for whom cricopharyngeal myotomy was performed. The major surgical objectives were facilitation of swallowing and amelioration of the tendency of these patients to aspirate ingestants and secretions. In the group of these patients in which the follow-up information has been adequate, 64% were improved and 36% experienced no significant benefits. The authors' surgical indications, methods of anesthesia, surgical technique, and postoperative care are described.
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PMID:Cricopharyngeal myotomy in amyotrophic lateral sclerosis. 93 77

Percutaneous endoscopic gastrostomy (PEG) is an enteral nutritional assistance technique using a simple device compatible with conventional feeding and thus enabling the patient to be integrated into his or her social and familial surroundings. This inexpensive device is quickly and easily inserted under local anaesthesia. It causes little morbidity and virtually no mortality and has many advantages for patients with amyotrophic lateral sclerosis (ALS). We report the results of PEG in 28 ALS patients with bulbar involvement. Three of these patients developed minor complications during 6 consecutive months of PEG-assisted nutrition (2 had periostomial infection, 1 had mild haematemesis). There were no major complications, and mortality directly ascribable to PEG was nil. All patients put on weight or had their weight stabilized, and GEP was well accepted in all cases.
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PMID:[Role of percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis]. 190 69

We report two cases of patients diagnosed of motoneuron disease. First patient had amyotrophic lateral sclerosis with bulbar involvement and developed prolonged apnea after general anesthesia for elective hemorrhoidectomy. Second patient had Kugelberg-Welander disease or type III hereditary mononeuron disease, underwent laparotomy and had no anesthetic complications. Patients with motoneuron disease should be considered as high anesthetic risk patients because they may develop respiratory complications accordingly to their previous muscle involvement so neurologic and respiratory assessment is basic. The use of succinylcholine should be limited to cases in whom external denervation and muscle atrophy may be ruled out. Short half-life muscle relaxants such as atracurium and vecuronium are advised with monitoring of neuromuscular function. Currently, local-regional techniques are not contraindicated.
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PMID:[Anesthesia in two patients with motor neuron disease]. 209 63

The increase in the content of taurine in the central nervous system (CNS) of amyotrophic lateral sclerosis (ALS) may probably be related to the impairment of folate cycle. In addition, the appearance of pyramidal sign in patients with hyperthyroidism as well as impairment of folate metabolism in thyropathies have been reported. This study was undertaken to investigate whether excess or deficiency of thyroid hormones could induce changes in free amino acids and folate cycle in CNS. Wistar male rats weighing approximately 370 g were used. Forty rats were thyroidectomized, and twenty were sham-operated as control under pentobarbital anesthesia. After 22 to 24 weeks, these animals were sacrificed by decapitation and the blood was collected in a heparinized and a non-heparinized container. Brain, spinal cord, liver, skeletal muscle, erythrocytes, plasma, and serum were stored at -80 degrees C until analyzed. To make hyperthyroidism in rats, triiodothyronine (T3) solved in alkaline 0.9% saline (10 micrograms of T3/100 g of body weight) were injected intraperitoneally to 16 rats six times a week, totally thirty times. Only alkaline 0.9% saline were injected to sixteen controls. These rats were sacrificed by the same way described above and each material was also stored at -80 degrees C. The content of taurine was changed in various tissues including CNS in thyroidectomized and T3-injected rats. The significant increase of taurine was seen in spinal cord and skeletal muscle of thyroidectomized rats. On the other hand, the significant decrease of taurine was observed in brain, liver, and skeletal muscle of T3-injected rats. The folate level was increased only in liver of thyroidectomized rats.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Changes in free amino acids in the central nervous system of hypo- and hyperthyroid rats]. 258 84

Otolaryngological manifestations were examined in a series of 250 patients diagnosed as having ALS between 1976 and 1986. Surgical intervention was only required in 10 cases due to excessive drooling and aspiration. Five patients had submandibular gland excisions with only limited improvement in respect to drooling. One case having a unilateral tympanic neurectomy had significantly better drooling control. Cricopharyngeal myotomy is helpful when dysphagia is predominantly due to cricopharyngeal spasm. In our series, tympanic neurectomy and chorda tympanectomy provide the better control of drooling for these patients and has the added advantage of being performed under local anesthesia.
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PMID:Otolaryngologic manifestations of amyotrophic lateral sclerosis. 334 21

Acetylcholine receptor (AChR) binding and AChR modulating antibodies were found with approximately the same frequency (86%) in 349 patients with myasthenia gravis (MG). However, the total yield of positive serological results was significantly improved (90%) by assaying AChR modulating antibodies when AChR binding antibodies were not detected, because in 27 patients (8%) only one of the two tests was positive. The immunoprecipitation test for AChR blocking antibodies yielded fewer positive results (52%), but there was a significant correlation between the degree of AChR blockade and generalization of muscle weakness. In no patient was this the only positive test, because the test for AChR modulating antibodies in this study detected both AChR blocking and modulating antibodies. Human muscle AChR was used as antigen in all tests. False positive results were rare and were attributed to unexplained antibodies to 125I-alpha-Bgt (AChR binding antibody assay) and recent general anesthesia using muscle relaxants (AChR blocking and AChR modulating antibody assays). Unexplained positive results, documented previously in 5% of patients with the Lambert-Eaton myasthenic syndrome and amyotrophic lateral sclerosis (ALS), were found in this study in two of 22 patients with ALS, but in none of 427 patients with miscellaneous neurological diseases. Patients with severe generalized MG and/or thymoma tended to have higher titers of AChR binding antibodies and greater AChR modulating antibody activity. However, some patients with severe muscle weakness had low titers of antibodies, and some patients in remission or with only ocular manifestations had high titers. These seemingly paradoxical results reflect heterogeneity in the specificities, affinities, and isotypes of anti-AChR antibodies. To effect pathogenicity, antibodies must have access in vivo to extracellular antigenic sites on the AChR. One would anticipate that antibodies with greatest pathophysiological potential would be of an IgG with greatest pathophysiological potential would be of an IgG subclass that activates complement, or of a specificity that competes for acetylcholine's binding site on the receptor or readily cross-links two AChR molecules to trigger receptor modulation (e.g., by binding to sites on the duplicated alpha-subunit). In patients with suspected MG who lack serological evidence of anti-AChR antibodies, motor endplate biopsy is required for microelectrophysiological, immunochemical, and ultrastructural studies to establish with certainty whether or not the condition is acquired MG.
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PMID:Clinical correlations of antibodies that bind, block, or modulate human acetylcholine receptors in myasthenia gravis. 347 35

Amyotrophic lateral sclerosis (ALS) is a disease characterized by progressive degeneration of the lower motor neurons, motor nuclei of the brain stem, and the descending pathway of the upper motor neurons. As the disease progresses, atrophy and weakness involve most of the skeletal muscles, including those of the tongue, pharynx larynx and chest. Impairment of respiration, altered response to muscle relaxants, and predisposition of aspiration affect a safe anesthetic management. General anesthesia with tracheal intubation might ensure intraoperative ventilatory management, but it could also increase the risk of aspiration with its entailing airway obstruction and demand the need of postoperative artificial ventilatory support. In this report, we described the use of epidural analgesia with 2% lidocaine combined with continuous infusion of low dose propofol for sedation for a ALS patient undergoing abdominal hysterectomy. The anesthetic management of this patient was smooth and successful. Besides, epidural morphine could provide excellent postoperative pain relief without respiratory complications.
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PMID:Epidural anesthesia combined with propofol sedation for abdominal hysterectomy in a patient with amyotrophic lateral sclerosis--a case report. 981 21

A 49-year-old male with amyotrophic lateral sclerosis (ALS) was scheduled for gastrectomy. Anesthetic management was performed under general anesthesia with sevoflurane and epidural anesthesia with lidocaine. He showed increased response to vecuronium under monitoring of neuromuscular block. But he responded favorably to anticholineesterase. He had little pain and showed no progress in neurological symptoms in the postoperative period. Neuromuscular monitoring is essential in administrating non-depolarizing neuromuscular blocking agents to patients with ALS, and epidural anesthesia may be useful for perioperative management of patients with ALS.
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PMID:[Anesthetic management of a patient with amyotrophic lateral sclerosis]. 1070 27

To determine how the education of basic life support (BLS)/advanced cardiac life support (ACLS) and emergency medicine for anesthesiologists is conducted, we performed a survey of chairpersons at university departments and newly board certified anesthesiologists in 1999. Basic and advanced life support courses for residents were provided in more than half of the anesthesiology departments that responded to this survey. However, approximately only 10% of the respondents had regular BLS/ACLS courses for residents. On the other hand, more than 80% of respondents considered a rotation in emergency medicine desirable as a part of anesthesia training. To improve the resuscitation skills of anesthesiologists, an urgent need to establish regular BLS/ALS courses and educational programs in emergency medicine in anesthesia training does exist.
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PMID:[Education of basic/advanced life support and emergency medicine for anesthesiologists: a survey of chairpersons at university department and newly board certified anesthesiologists]. 1145 81


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