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 dysphagia of ALS is characterized by an impaired oral stage of swallowing, which has a direct impact on the seemingly more robust pharyngeal stage. Increased duration of the volitional stage (PPRT) of swallowing, including DT and lingual motility deficits, appear to be the major contributors to the dysphagia. The abnormal temporospatial events during the oral stage of swallowing have a direct and significant effect on the more automatic, pharyngeal stage. Specifically, abnormal lingual movement may result in pharyngeal residue that is aspirated after the swallow is completed and respiration is resumed. ALS patients with bulbar involvement demonstrate more severe swallowing problems (such as aspiration); however, the current work indicates that predominantly nonbulbar ALS patients may also demonstrate dysphagia. The semisolid material was more effective than liquids in eliciting temporospatial abnormalities of swallowing in the nonbulbar group. Thus, more viscous materials may be most sensitive for eliciting behaviors indicative of the onset of bulbar dysfunction in patients who otherwise appear nonbulbar, or different neurophysiologic mechanisms are involved in swallowing the distinct types of substances.
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PMID:Swallowing in ALS and motor neuron disorders. 1168

We describe a patient with amyotrophic lateral sclerosis with dementia (ALS-D) displaying a long clinical course. A 68-year-old Japanese male with no family history of note was admitted complaining of severe dysarthria and dysphagia. At 63 years old, Pick's disease was diagnosed on the basis of abnormal behavior, such as "Denkfaulheit" and moria, and temporal lobe atrophy observed on magnetic resonance imaging (MRI). Five years after onset, dysarthria and dysphagia emerged, and gradually worsened. On admission, muscular weakness of the upper extremities, fasciculation, and exaggerated tendon stretch reflexes were noted. Needle electromyography performed on the left upper and lower extremities revealed neurogenic pattern changes. Based on these findings and clinical course, ALS-D was diagnosed. Due to severe bulbar palsy, verbal communication was impossible. However, neither specific symptoms of dementia nor abnormal behavior was demonstrated, although this latter had been observed 5 years ago, with only short-term memory impairment apparent. MRI disclosed severe knife-edge atrophy of bilateral temporal lobes, most prominently in the anterior regions. SPECT images revealed decreased uptake of tracer in bilateral inferior temporal lobes, predominantly on the left side. The patient died suddenly 4 months after admission, and post-mortem examination was not conducted. Total clinical course was about 8 years. Several cases of ALS-D have displayed similar clinical courses to the presented case. Some of these would also have initially been diagnosed as Pick's disease. We speculate that cases displaying psychiatric symptoms for several years and initially diagnosed as Pick's disease may finally be diagnosed as ALS-D upon the eventual emergence of motor symptoms(bulbar palsy).
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PMID:[A case of amyotrophic lateral sclerosis with dementia presenting long clinical course]. 1268 97

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

Swallowing and speech disorders are the dramatic consequences of bulbar and pseudo-bulbar syndrome in ALS. Evaluation is necessary to guide speech therapy and to measure the effects of treatment. This article revues the different examinations used to assess bulbar and pseudobulbar involvement in an ALS patient: oromotor assessment, evaluation of the functions with self assessment, perceptive and objective evaluation of speech disorders, fiberoptic endoscopic evaluation of dysphagia (FEES) and videofluoroscopy.
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PMID:[Assessment of dysarthria and dysphagia in ALS patients]. 1658 5

Recognizing an ALS-mimic can be challenging. Here, we describe a patient with a slowly progressive dysarthria and dysphagia, with fasciculations of the tongue and general hyperreflexia, fulfilling the diagnostic criteria of 'clinical probable ALS'. Because of a non-conclusive EMG, a muscle biopsy was performed that surprisingly showed widespread nemaline rods. The clinical features and the histological findings were compatible with a sporadic late onset nemaline myopathy. Three years after initial presentation the patient died and post-mortem examination not only showed nemaline bodies in every muscle examined, but also revealed an unsuspected final diagnosis: sarcoid brainstem encephalitis. Nemaline rods can be found in various disorders, and neurosarcoidosis should be added to this list.
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PMID:A case of neuromuscular mimicry. 1691 50

Artificial nutrition is necessary when oral feeding becomes insufficient to cover protein and energetic needs and becomes dangerous (risk of malnutrition, dehydration and aspiration). In ALS patients, enteral nutrition is the method of choice and gastrostomy is preferable to nasogastric tube which must be limited for a short term enteral nutrition or if gastrostomy is at risk (because of pulmonary function) or refused by the patient. The percutaneous gastrostomy can be placed endoscopically (PEG) or radiologically (RIG), surgical gastrostomy has to be avoided because of general anaesthesia. Advantages of RIG are a success rate of about 100 percent and a placement feasible without sedation but its superiority on PEG in ALS patients especially if pulmonary functions are altered is not demonstrated. No objective criterion permits to define the exact moment of enteral nutrition. However, enteral nutrition is recommended when dysphagia becomes symptomatic (insufficient caloric intake with weight loss, dehydration, frequent choking and aspiration). Swallowing disorders must be detected early to give to patients and their family information about enteral nutrition and gastrostomy as soon as possible and to help them to decide. It is desirable to propose gastrostomy when forced vital capacity is yet above 50 percent and nutritional state not altered (body mass index>18kg/m2 and/or weight loss<10 percent). Enteral nutrition is not desirable in ALS patients with dementia or in the preterminal phase. Suitable enteral nutrition with regular nutritional evaluation can improve nutritional status. Currently, improvement of quality of life and survival due to enteral nutrition has not been proved in ALS patients.
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PMID:[What are the means of alimentary function supply and their indications in amyotrophic lateral sclerosis?]. 1712 31

Progressive loss of motor neurons causes Amyotrophic Lateral Sclerosis. Patients complain, most often, of progressive weakness in the distal limbs. However, weakness may manifest in any body segment (bulbar, cervical, thoracic, or lumbosacral). The diagnosis of ALS is suggested by clinical examination that reveals both upper and lower motor neuron failure. Formal diagnostic criteria have been developed and validated. Nerve conduction and electromyography studies improve diagnostic sensitivity and exclude some alternate, treatable diagnoses. Likewise, conventional imaging studies and laboratory evaluation refute other diseases that may masquerade as ALS. Experimental imaging and laboratory evaluations may improve ALS diagnosis in the future. The cause of motor neuron death is not known but inherited forms of motor neuron disease may suggest mechanisms. The goal of ALS treatment is control of the symptoms of progressive weakness, especially respiratory insufficiency and dysphagia and is best managed in an integrated clinic.
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PMID:Clinical presentation and diagnosis of amyotrophic lateral sclerosis. 1819 25

Current practice guidelines state that enteral feeding should be proposed and provided whenever ALS patients show significant dysphagia and/or weight loss. Percutaneous endoscopic gastrostomy (PEG) is usually available at ALS centres, but this method is not indicated in the case of patients with severe respiratory impairment. We undertook a study to assess the feasibility of applying home parenteral nutrition (PN) to ALS patients with respiratory insufficiency, to assess the complications and survival times after the procedure. PN was provided, via an implanted port, to 30 ALS patients with respiratory insufficiency requiring nutritional support. Nutritional and neurological follow-up were carried out. The results were compared with those obtained on a group of 35 patients who underwent PEG, who were subdivided into patients with respiratory insufficiency and those without respiratory insufficiency at the time of the PEG. Post-procedure survival of PEG patients with respiratory insufficiency was similar to those of the PN group. The results of the study suggest that PN is a safe and effective method, which improves the patients' nutritional status and stabilizes their weight in most cases. Home PN could be applied to patients with advanced ALS and poor respiratory function as an alternative to enteral feeding.
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PMID:Enteral and parenteral nutrition in the later stages of ALS: an observational study. 1861 38

Twenty ALS patients with sialorrhea refractory to medical therapy were enrolled in this double-blind, randomized study to receive either 2,500 U of botulinum toxin type B (BTxb) or placebo into the bilateral parotid and submandibular glands using electromyographic guidance. Patients who received BTxb reported a global impression of improvement of 82% at 2 weeks compared to 38% of those who received placebo (P < 0.05). This significant effect was sustained at 4 weeks. At 12 weeks, 50% of patients who received BTxb continued to report improvement compared to 14% of those who received placebo. There were no significant adverse events, including dysphagia, in the BTxb group, and there was no significant increase in the rate of decline of vital capacity.
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PMID:Randomized double-blind study of botulinum toxin type B for sialorrhea in ALS patients. 1914 53

We reported the two cases with bulbar-onset ALS showing isolated agraphia without overt dementia and aphasia. Patient 1 was a 69-year-old man and patient 2 was an 81-year-old woman, and both were right-handed. Each patient developed dysarthria as an initial symptom followed by dysphagia, and neurological examinations showed atrophy and fasciculation of the tongue with upper and lower motor-neuron involvement of the extremities. These characteristic features with electromyographic evidence including widespread acute and chronic denervation led to a diagnosis of bulbar-onset ALS. Around 1 year after the onset of ALS, dysarthria was mild enough to allow oral communication enabling the determination that aphasia was absent with well preserved confrontation naming, repetition, reading and comprehension. The patients were polite without abnormal behavior or character change, and their general intelligence was also well preserved with excellent scores on the Mini Mental State Examination (30 and 27 points for patients 1 and 2, respectively) and Frontal Assessment Battery (16 points for each patient). However, spontaneous writing and dictation revealed abundant writing errors characterized by omission of kana letters and paragraphia of kana and kanji letters in both patients. Some syntactic errors were also observed in writing but in spoken language. A letter-number effect on writing errors was observed in patient 1. Copying of letters or words was intact and structure and orientation of written letters was well preserved, indicating the absence of constructional, apraxic or spatial feature of agraphia. Single photon emission computed tomography demonstrated reduced uptake in the bilateral frontotemporal lobes, predominantly in the left hemisphere, with less evident alternation in magnetic resonance imaging. Our results suggest that patients with bulbar-onset ALS may develop isolated agraphia as a single-domain cognitive impairment, preceding the clinical manifestation of aphasia or dementia. We speculate that the main responsible region might be the posterior part of the middle and inferior frontal gyri including Exner's writing center and Broca's area beyond the primary motor cortex.
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PMID:[Bulbar-onset amyotrophic lateral sclerosis (ALS) with isolated agraphia]. 2019 88


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