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Query: UMLS:C0032290 (aspiration pneumonia)
 2,291 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 26-year-old housewife, born of consanguineous parentage, began to have gait and speech disturbance. Her brother had died from suffocation because of dysphagia. At thirty-two, she developed difficulty in swallowing, clumsiness and incontinence. When she was thirty-six she had pseudobulbar palsy, vertical gaze paresis, hyperreflexia and muscular atrophy of the upper half of the body. CT scan showed cerebral atrophy. Her mental function progressively deteriorated and amyotrophic lateral sclerosis associated with dementia was suspected. She died at the age of thirty-seven. Diagnosis was made only by autopsy. There was no particular general pathologic finding excepting aspiration pneumonia. Microscopical examination revealed numerous distended neurons with accumulation of light brown pigments by Luxol fast blue/H & E stains, especially in hypothalamus, substantia nigra and nuclei of oculomotor nerves. To a lesser extent such neurons were noted ubiquitously. The stored material was mainly composed of lipofuscin and ceroid. Ultrastructurally they presented the various structures which have previously been reported, except for finger print profiles. The pigmentary deposits were shown to be immunoreactive with polyclonal antibody directed against amyloid beta-protein.
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PMID:[Dementia and amyotrophy in Kufs disease. The adult type of neuronal ceroid lipofuscinosis]. 774 8

Amyotrophic lateral sclerosis (ALS) raises progressive dysphagia that causes aspiration. This diseases affects oral phase first and then pharyngeal phase of swallowing. Aspiration pneumonia by dysphagia becomes major problem from clinical aspect to cause aggravation of vital prognosis. We explain the mechanism of dysphagia from deglutition dynamics and discuss the clinical managements. Several functional operations such as cricopharyngeal myotomy, laryngeal suspension and so forth, have been applied to prevent aspiration, with satisfactory results in cases with mild dysphagia. However, in cases of ALS patients with severe aspiration, the results are poor. Then we have to divide airway from digestive tract completely to prevent aspiration by laryngectomy. However, the larynx is a multi-functional organ, loss of the larynx does improve the swallowing but loose phonatory function. We need to evaluate a deglutition function before starting of treatments of dysphagia. Besides, we also need to put hopes and life style of patients in consideration. We have to be very careful to choose the way of surgical treatment and the time of operation. We can conclude that laryngectomy is useful for vital prognosis and amelioration of QOL in ALS patients.
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PMID:[Dysphagia in amyotrophic lateral sclerosis--the mechanism and managements]. 875 62

The authors' hospital is a 585-bed hospital under the direct management of the National Health Insurance System. The hospital has been providing visiting nurses for the past 8 years, who work from local medical centers and the Visiting Nurses Department. Thirty-seven patients have received such home care, among whom 8 had intractable disease. Patient S was a 46-year-old woman who suffered from amyotrophic lateral sclerosis (ALS). The onset of the disease was in April, 1993, when the patient experienced muscular atrophy in both legs and deteriorating muscular strength. The diagnosis was definitive in 1995. On March 1, 1998, the patient received emergency hospitalization for breathing difficulties and aspiration pneumonia, and on March 5 underwent tracheotomy. A cannula had to be inserted for tubal feeding, and the physician in charge explained to her family that her prognosis was 3 months. Both the patient and her family desired home care, and the patient returned home on April 11. Respecting the wishes of the patient, the visiting nurse provided support so that home treatment could be continued. In the end, the patient lived at home while receiving home treatment for 7 months. Through the support provided by the visiting nurse, efforts were made to keep the patient's condition stable, and she was able to continue home treatment and living at home for a higher quality of life.
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PMID:[Visiting nurse for a terminal ALS patient]. 1063 Feb 15

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by death of upper and lower motor neurones. Nutritional and respiratory failure occurs in most patients with ALS. Nutritional failure occurs primarily as a result of dysphagia, although malnutrition may also develop in the absence of clinically apparent dysphagia. The optimal management of nutrition in early ALS has not been established. In later stages of the disease, parenteral nutritional support using percutaneous endoscopic gastrostomy confers a significant survival benefit in selected patients. Respiratory failure occurs as a result of bulbar, cervical and thoracic loss of motor neurones. Inspiratory muscles are preferentially affected. Management of respiratory failure includes the use of strategies that limit aspiration pneumonia, the reduction in secretions, and positioning of the patient to a maximal mechanical advantage. Use of non-invasive positive pressure ventilation in appropriate patients significantly enhances survival. The decision to undertake invasive mechanical ventilation should be made prior to the development of symptoms that might warrant this intervention. The progressive nature of the condition should be taken into account when such a decision is discussed with the patient and carer. Further studies are required to determine the optimal nutritional requirements of patients with ALS, and to elucidate the physiological changes involved in the decline in respiratory function.
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PMID:Symptomatic treatment of respiratory and nutritional failure in amyotrophic lateral sclerosis. 1083 14

We report a case displaying upper motor sign, parkinsonism, and behavioral abnormality, with marked degeneration of the precentral cortex, neostriatum and frontotemporal lobes, as well as ubiquitinated neuronal inclusions. The patient was a 66-year-old male at the time of death. At age 57, he noticed progressive difficulties in speaking and swallowing. At age 60, he was severely anarthric and displayed emotional lability and incontinence. Neurologically, very poor movement of tongue was observed, but without atrophy or fasciculation. Deep tendon reflexes were hyperactive. Grasp reflex and snout reflex were also positive. Needle electromyography revealed no abnormalities. A diagnosis of primary lateral sclerosis and character change was made. At age 62, he developed bradykinesia and rigidity of the neck and all extremities. Treatment with carbidopa-levodopa was initiated, but resulted in minimal improvement. At age 65, he was bed-ridden, and had repeated occurrences of aspiration pneumonia; he died of pneumonia. Neuropathological examination revealed marked atrophy of the frontal and temporal lobes with Betz cells completely absent and moderate atrophy of the neostriatum. The spinal cord and nerve roots appeared normal. Immunohistochemically, ubiquitin-positive but tau-negative intraneuronal inclusions were found in the frontal and temporal cortices, including the precentral cortex and the hippocampal dentate gyrus, and the neostriatum. This case could be included with inclusion-associated disorders such as frontotemporal dementia or amyotrophic lateral sclerosis with dementia, and furthermore, predominant upper motor sign and parkinsonism could represent phenotypes of clinical manifestations with such inclusions.
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PMID:Frontotemporal dementia with ubiquitinated neuronal inclusions presenting with primary lateral sclerosis and parkinsonism: clinicopathological report of an autopsy case. 1476 75

Amyotrophic lateral sclerosis (ALS) is a progressive and lethal motor neurodegenerative disease. The cause of the disease is unknown, and there is no cure current clinical treatment methods include Riluzole and supportive therapy. However, Riluzole only decelerates the patient's muscle strength loss and prolongs survival by about 3-5 months (Aventis Pharmaceutical, 2004). The patient will die eventually because of aspiration pneumonia or respiratory failure. This paper documents the use of Orem's self-care theory and holistic nursing assessment in a patient with amyotrophic lateral sclerosis. There are four areas of nursing concern: impaired self-care ability (dressing, feeding, toileting, and bathing), risk of falls, impaired verbal communication, and powerlessness. In terms of these problems suitable nursing activities are provided to develop the patient's ability to care for himself, prevent accidents, promote skills of non-verbal communication, and alleviate powerlessness in order to strengthen control.
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PMID:[An experience nursing a patient with amyotrophic lateral sclerosis using the theory of self-care]. 1598 7

We report a 59-year-old woman with generalized amyotrophy and dementia. She showed personality change at 53 years of age. When she was 56 years old, she began to show abnormal and violent behaviors. At age 58, she developed dysphagia and amyotrophy of upper limbs. She was admitted to a hospital for the treatment of aspiration pneumonia. She was severely demented and showed pseudobulbar palsy, amyotrophy of tongues, weakness of upper limbs, and pyramidal signs. She was still able to walk by herself. Dementia, pseudobulbar palsy, and amyotrophy progressed rapidly. At age 59, she became bed ridden and required tube feeding. She died by aspiration pneumonia at age 59. The patient was discussed at a neurological CPC and the chief discussant arrived at the conclusion that the patient had ALS dementia. Other possibility discussed was Pick's disease with amyotrophy. Post-mortem examination revealed severe lower motor neuron degeneration. The upper motor neurons were unaffected. Neuronal loss was not observed in the cerebral cortex, but moderate gliosis was seen in the cerebral white matter. In addition, the substantia nigra was moderately degenerated. There were ubiquitin positive neuronal inclusions in the granular cells of the dentate gyrus. Also, Bunina bodies were seen in the neurons of spinal anterior horns. These findings were characteristic pathology for ALS with dementia.
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PMID:[A 59-year-old woman with personality change and abnormal behavior followed by amyotrophy and dementia]. 1624 71

Aspiration pneumonia is believed to be an uncommon complication of amyotrophic lateral sclerosis (ALS). However, the incidence of aspiration pneumonia and its contribution to morbidity and mortality are unknown in ALS. All cases of ALS from Olmsted County, Minnesota from 1990 to 2005 were reviewed. Baseline demographics were noted for all cases. Dates of diagnosis, PEG tube placement, death and survival status were also noted. All cases of aspiration pneumonia were identified. Forty cases of ALS were identified over the 15-year period. The incidence rate was 1.7 cases per 100,000 person years. There was an overall mean survival of 26 months from diagnosis. Aspiration pneumonia occurred in five (13%) cases. There was a mean survival of two months following aspiration pneumonia. The strongest risk factor for aspiration pneumonia was nursing home residence with a relative risk of 7.1 (p = 0.02) We conclude that the incidence rates and demographics of our ALS population have remained stable over time. Aspiration pneumonia occurs in a minority but is associated with a high mortality. ALS subjects residing in nursing homes are at the greatest risk.
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PMID:Incidence of aspiration pneumonia in ALS in Olmsted County, MN. 1745 35

Amyotrophic lateral sclerosis (ALS) is a degenerative disease involving both upper and lower motor neurons and the pathogenesis of this disorder is still unknown. To date, few reports have suggested that motor neuron diseases may have a paraneoplastic origin. However, it is still under discussion whether ALS occurring in cancer patients is paraneoplastic. A 60-year-old man with rectal cancer (Stage IV) having multiple lung, liver and para-aortic lymph node metastases underwent anterior resection of the rectum as palliative surgery. He was referred to our hospital for adjuvant chemotherapy. Lung and lymph node metastases decreased after 2 courses of chemotherapy using CPT-11 and 5-FU/LV but liver metastases were enlarged, following up increase in CEA. Thereafter, he suffered from muscle weakness in hands, arms, and legs and results of neurophysiologic studies were compatible with primary lateral sclerosis (ALS). For second line chemotherapy, he was treated with low-dose CDDP/5-FU over 6 courses. As a result, the size the of metastatic lesions markedly reduced and CEA was decreased to the normal level. Although significant tumor reduction was observed, his neurological symptoms rapidly progressed. He died of aspiration pneumonia 8 months after onset of the disease. Autopsy revealed that his neuropathological findings were compatible with ALS, and it was thought to be the primary cause of death in the because of absence of cancer progression. In this case the neurological syndrome was not affected by cancer therapy. Thus our case does not support the hypothesis that ALS in associated with cancer and the relationship between both disorders remains uncertain.
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PMID:Report of an autopsy case of colon cancer with amyotrophic lateral sclerosis. 1782 8

To evaluate the usefulness of laryngotracheal separation or tracheoesophageal diversion (LTS/TED), we investigated changes in medical management after LTS/TED. We performed LTS/TED for intractable aspiration in patients with amyotrophic lateral sclerosis (ALS) and patients with other neurological diseases. Most of the subjects had already received a tracheostomy, and all the patients with ALS had tracheostomy positive pressure ventilation. However, they remained at risk of intractable aspiration, had experienced frequent suctioning of aspiration material, and could not eat. In all cases, LTS/TED was performed safely within 3 hours and without any complications. After LTS/TED, there was no aspiration of saliva in any of the patients. In most subjects, the frequency of suctioning by medical staff and caregivers was much reduced. The frequency of aspiration pneumonia was also extremely lowered. Follow-up study demonstrated that complete control over aspiration was achieved in all of the patients. Some of them were completely self-sufficient in their ability to eat. Some of the other patients were at least able to enjoy taste. Nutritional status was significantly improved. All the patients, family caregivers, and medical staff involved in this study were satisfied with the outcome. These results indicate that LTS/TED is a very useful procedure in several aspects: it benefits patients who have a fear of aspiration; it reduces the burden on patients and family caregivers; it promotes their quality of life; and it limits the aspiration-associated demands on medical staff. Based on the results, we have proposed an indication of LTS/TED for dysphasia in ALS.
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PMID:[Laryngotracheal separation and tracheoesophageal diversion for intractable aspiration in ALS--usefulness and indication]. 1796 55


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