Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The patient with ALS can be managed almost entirely as an outpatient by a team consisting of a nurse, physiotherapist, occupational therapist, speech pathologist, nutritionist, respirologist, social worker, and certain other consultants from time to time. The team's goal is to maintain physical function and extend the useful life of the patient through the skills that the team members are trained to provide. Pulmonary function tests, especially spirometry, should be done at regular intervals and a modified barium swallow should also be done at least once in cases with dysphagia. It is possible with these tests to anticipate and even correct a number of hazards, such as upper airway obstruction and aspiration. Some patients are candidates for gastrostomy and tympanic and chorda tympani neurectomy, but full knowledge of their pulmonary function is essential before undertaking any operative procedure. Death in ALS is due to pulmonary failure and the choice of respirator care requires careful deliberation with the family. The neurologist and ALS team should work in close cooperation with the home care personnel in the patient's own community. Does the care of the ALS patient in any way affect survival? In the attempt to answer this question we have estimated the survival of ALS patients in southwestern Ontario, most of whom have visited our clinic over the period of 1978 to 1985, inclusive. As shown in Figure 4, there was an apparent decline in the annual mortality rate over this period, although there was no significant change in the incidence of ALS in this region.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Outpatient management of amyotrophic lateral sclerosis. 314 Mar 37

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

The bulbar symptoms of ALS include difficulty with the management of swallowing, saliva, aspiration, and communication. These symptoms originate from the disability of the oropharyngeal muscles and actually represent varying degrees of severity of a single problem. The management of these symptoms requires the concerted effort of a rehabilitation team, which should include a surgeon, speech pathologist, neurologist, psychosocial worker, and dietitian. Early education of the patient and patient's family greatly facilitates successful management of bulbar symptoms. Early recognition of the signs of dysphagia, aspiration, and communicative disability are important in order to provide solutions before severe and possibly life-threatening debilitation occurs. The treatments and techniques presented in this chapter must be applied to each patient on an individual basis. The moral and social decisions regarding the providing or withholding of treatment to ALS patients are difficult ones. It is important, however, to realize that management decisions of ALS patients must deal not only with the quantity of life but also the quality of life. This distinction often falls into a 'gray zone' with management of bulbar symptoms since swallowing difficulties are life-threatening as well as uncomfortable. One of the difficulties that we present ourselves as health care providers in ALS is that, more often than not, our patient is in a noncommunicative position and cannot contribute to treatment decisions. If careful and aggressive care is taken to preserve the communicative abilities of our patients, the difficult situation of making decisions based on the family's inclinations and our own inclinations can be avoided. When we are confronted with a decision to maintain or cease support for a patient, the moral and emotional issues that present can be overwhelming. However, the patient's input, if it were direct and included the ability to express complex thoughts, could often provide the information necessary to make the decisions more clear.
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PMID:Management of bulbar symptoms in amyotrophic lateral sclerosis. 357 11

We report a 65-year-old woman with progressive dysarthria, dysphagia, weakness, and gait disturbance. The patient was well until 59 years of age (January of 1986) when she noted bilateral ptosis. One year later, she noted a gradual onset of difficulty in speech (articulation). Her speech slowly deteriorated and she noted weakness in chewing power and difficulty in swallowing in addition. In October 1987, she developed emotional incontinence. In January of 1988, she started to drag her left foot. She was admitted to our hospital on June 13 of 1988. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed no dementia; her higher cerebral functions appeared intact. Ptosis was present bilaterally more on the right. She showed difficulty in opening her eyes on command; no contraction of the frontal muscles was seen upon attempted eye opening. There was a moderate limitation in the vertical gaze. Forced laughing and crying were seen. Facial muscles were moderately weak without apparent atrophy. The movement of the soft palate was very weak, and swallowing disturbance was more prominent for liquid staff. The tongue appeared somewhat small, however, no fasciculation was noted. Her step was small and the posture was stooped. Retropulsion was present, however, Romberg's sign was absent. No muscle atrophy was apparent, however, diffuse mile to moderate muscle weakness was noted in all four limbs. Cerebellar sign was absent. Deep tendon reflexes were exaggerated bilaterally, and Babinski sign was present on the left side. Sensation was intact. Routine blood tests were unremarkable as was a cranial CT scan. Her ptosis did not improve after 10 mg of edrophonium injection. CSF was also normal. She was transferred to another hospital but her neurological disabilities further progressed. In 1989, she was totally unable to move her limbs; she could only move her eyes; still consciousness was clear without dementia. She developed respiratory difficulty and expired on July 25, 1992. She was discussed in a neurological CPC, and the opinions were divided into ALS and primary lateral sclerosis (PLS). The chief discussant arrived at the conclusion that the patient might have had the pyramidal form of ALS. Postmorten examination revealed marked myelin pallor in the anterior as well as lateral corticospinal tracts. Pyramidal tract degeneration was prominent starting at the level of the cerebral peduncle and was continued to be seen until the level of lumbar cord. The number of anterior horn cells showed only slight decrease in the cervical level, however, it was normal in the lumbar cord.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[A 65-year-old woman with dysarthria, dysphagia, weakness, and gait disturbance]. 777 10

The efficacy and tolerability of amitriptyline on the pathologic crying and other pseudobulbar signs were investigated in 22 consecutive patients diagnosed mostly as ALS. The occurrence and intensity of pathologic crying, dysarthria, dysphagia, jaw reflex and primitive reflexes (snout, palmo-mental and oral), were assessed before and after 3 and 6 weeks of amitriptyline treatment. The drug administered in low dose (30-100 mg, mean 64 +/- 17.6 mg) significantly decreased the frequency of pathologic crying in 17 patients after 3 weeks and in 20 patients after 6 weeks of treatment. There were no changes in the intensity of the other pseudobulbar signs. Only few mild and transient side effects were observed. The authors conclude, that amitriptyline is an effective treatment of pathologic crying in ALS patients.
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PMID:[The effect of amitriptyline on the pathological crying and other pseudobulbar signs]. 858 93

For the bulbar ALS/MND patient with dysphagia, the accumulation of oral secretions is a distressing problem. The control of these secretions, particularly the thick mucus form, is very difficult. In approaching this problem it is important to realise that the source of these secretions is not just from the oral salivary glands. The nose and lungs also contribute a constant flow of serous and particularly mucoid fluids. The neural control of these different forms of secretion is of importance in controlling them. Stimulation of cholinergic receptors produces thin serous secretions whereas beta adrenergic receptors produce thick protein and mucus-rich secretions. Therefore 16 bulbar ALS/MND patients were treated with beta antagonists after maximising other therapy. 75% of this group had fast and significant relief from their thick secretions. It is therefore proposed to perform a large controlled trial of beta blockers in the control of thick oral secretions in ALS/MND based on these promising pilot data.
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PMID:The control of oral secretions in bulbar ALS/MND. 889 57

We report a 49-year-old man with progressive bulbar palsy and respiratory failure. He was well until his 48 years of the age (December 1994) when he noted a difficulty in speaking in loud voice. In February, 1995, he noted regurgitation of foods to his nose and difficulty in his speech. He was admitted to our service in May 29, 1995. On admission, he was alert and oriented to all spheres and he was not demented. His higher cerebral functions were normal. In cranial nerves, he showed dysarthria and dysphagia; muscle atrophies were seen in the tongue, the bilateral sternocleidomastoid, supraspinatus, and infraspinatus muscles. Fasciculations were seen in these muscles. He showed no muscle weakness in his limbs except for the upper limb girdle muscles, no ataxia, no reflex abnormalities, nor sensory changes. EMG showed neurogenic changes in the affected muscles. MRI of the brain and the spinal cord was entirely normal. He was discharged for out patient follow-up, however, in October of 1995, he noted difficulty in swallowing solid foods. Gastrostomy was placed and he was discharged to his home. In February 11th of 1996, he was found unresponsive and brought into the ER of our hospital. On admission, he was comatose without spontaneous respiration. BP could not be obtained. He was immediately intubated and artificial ventilation was started. On the following morning, he became alert and he was not demented. He continued to show marked dysarthria and dysphagia; again no weakness was noted in the distal parts of the upper and lower extremities. Laboratory examination showed increase in serum CK to 2,173 IU/L and amylase to 2,032 IU/L. He was extubated on February 15th, however, his spontaneous respiration was not suffice to maintain his blood gas. According to his will, he was not placed on respirator and he died on February 24th, 1996. The patient was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had ALS. Although no upper neuron signs were observed clinically, it is not uncommon to see degeneration in the corticospinal tract in post-mortem examination. The question was what might have been the cause of increase in CK and amylase. Many participants thought that they were secondary to multiple organ failure due to prolonged hypoxic state at his last admission; other possibilities raised included acute myocardial infarction and acute bowel necrosis. Post-mortem examination revealed muscle atrophy in the facial, lingual, cervical, intercostal, and the upper limb girdle areas. The lungs were unremarkable except for old organized pneumonic foci in the right middle and lower lobes. Marked to moderate congestion was seen in many internal organs, however, no other gross abnormality was found. It was thought that respiratory palsy itself was the direct cause of his agonal event. In the spinal cord, the anterior horns showed various degree of neuronal loss and gliosis. No clear evidence of pyramidal tract degeneration was seen at the light microscope level. Lower brain stem motor neurons were markedly reduced. But no Bunina body was found. The substantia nigra showed moderate degree of neuronal loss and extraneuronal neuromelanins. The locus coeruleus showed similar but milder changes. The degree of nigral degeneration appeared to be well beyond those which could be seen in usual ALS patients. The question was whether or not this patient might have been in an early stage of the extended form of ALS.
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PMID:[A 49-year-old man with progressive bulbar palsy and respiratory failure]. 949 5

Adult motor neuron disease (amyotrophic lateral sclerosis [ALS]) is a neurodegenerative disorder characterized by loss of motor neurons in the cortex, brain stem, and spinal cord, manifested by upper and lower motor neuron signs and symptoms affecting bulbar, limb, and respiratory musculature. Clinically, the disease course is characterized by progressive weakness, atrophy, spasticity, dysarthria, dysphagia, and respiratory compromise, ultimately resulting in death or mechanical ventilation in the vast majority of patients. Patterns of presentation and pathological features of the disease, along with clinical and electrophysiologic criteria for diagnosis, are discussed in this review. Since 8% to 22% of patients survive more than 10 years without ventilator use, meticulous medical and rehabilitation management is extremely important to ensure optimal health and quality of life in these patients. Major issues in the care of individuals with ALS include weakness and spasticity, impairments in activities of daily living and mobility, communication deficits and dysphagia in those with bulbar involvement, respiratory compromise, fatigue and sleep disorders, pain, and psychosocial distress. Research in ALS changes rapidly, but is currently focused on potential etiologic factors such as glutamate excitotoxicity, role of oxidative stress, autoimmunity to calcium channels, and cytoskeletal abnormalities, as well as related treatment initiatives including glutamate modulators, neurotrophic factors, antioxidants, antiapoptotic factors, and gene therapy. Recently, mutations in the gene encoding Cu/Zn superoxide dismutase were identified in a subset of familial ALS patients. Riluzole, a glutamate antagonist and Na-channel blocker, became the only drug currently approved for treatment of ALS after studies showed a small positive effect on survival. Until a definitive treatment or cure for ALS is found, the multifaceted rehabilitation team approach remains the best hope for improving health and survival in this devastating illness.
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PMID:Evaluation and rehabilitation of patients with adult motor neuron disease. 1045 74

Percutaneous endoscopic gastrostomy (PEG) has been proposed as symptomatic treatment of dysphagia in patients with ALS. Safety and factors related to survival after PEG were analyzed in 50 consecutive ALS patients. No major acute or long-term complications were observed. Stabilization or increase in weight were observed after PEG. Median survival after PEG was 185 days, with a worse outcome in patients with weight loss > or =10% healthy body weight and forced vital capacity <65%. PEG may be a useful option in the symptomatic treatment of dysphagia in ALS.
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PMID:Safety and factors related to survival after percutaneous endoscopic gastrostomy in ALS. ALS Percutaneous Endoscopic Gastrostomy Study Group. 1049 78

Percutaneous endoscopic gastrostomy (PEG) provides a reliable route for nutrition and hydration in ALS patients with dysphagia. We performed a retrospective analysis of the CNTF and BDNF databases to determine the clinical status of ALS patients within 30 days preceding PEG insertion. This analysis revealed an approximately 50% reduction of function across multiple measures of ALS disease status. A trend to earlier intervention with PEG was apparent upon review of published studies and the CNTF and BDNF studies. By comparing the rate of decline pre- and post-PEG, nutritional supplementation via PEG stabilized the weight loss experienced by patients. Death within 30 days post-PEG was associated with a marked reduction in forced vital capacity (FVC) and identified a group of ALS patients in whom PEG should be cautiously performed. These data emphasize the importance of sequential measurement of FVC in managing ALS patients to guide the timing of nutritional intervention with PEG.
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PMID:A retrospective study of percutaneous endoscopic gastrostomy in ALS patients during the BDNF and CNTF trials. 1054 19


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