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

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

Abnormal neuronal accumulation and modification of TAR DNA binding protein 43 (TDP-43) have recently been discovered to be defining histopathological features of particular subtypes of frontotemporal dementia and amyotrophic lateral sclerosis, and are also common in aging, particularly coexisting with hippocampal sclerosis and Alzheimer's disease pathology. This case report describes a 72 year old Hispanic male with no family history of neurological disease, who presented at age 59 with obsessive behavior, anxiety, agitation, and dysphasia. Positron emission tomography imaging using the amyloid ligand 18F florbetapir (Amyvid) was positive. Postmortem examination revealed frequent diffuse and neuritic amyloid plaques throughout the cerebral cortex, thalamus, and striatum, Braak stage II neurofibrillary degeneration, and frequent frontal and temporal cortex TDP-43-positive neurites with rare nuclear inclusions. The case is unusual and instructive because of the co-existence of frequent cortical and diencephalic amyloid plaques with extensive TDP-43-positive histopathology in the setting of early-onset dementia and because it demonstrates that a positive cortical amyloid imaging signal in a subject with dementia does not necessarily establish that Alzheimer's disease is the sole cause.
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PMID:Positive florbetapir PET amyloid imaging in a subject with frequent cortical neuritic plaques and frontotemporal lobar degeneration with TDP43-positive inclusions. 2492 5

An 80 year old lady with a history of metastatic sigmoid carcinoma presented with expressive dysphasia and unsteady gait 4 days after commencement of adjuvant capecitabine chemotherapy. MRI demonstrated restricted diffusion and T2/FLAIR hyperintensity involving the course of the bilateral corticospinal tracts, the corpus callosum and the middle cerebellar peduncles. Discontinuation of chemotherapy lead to symptom resolution in 2 days; repeat MRI at 2 months demonstrated reversal of the diffusion changes and improvement of the previous T2W/FLAIR hyperintensity. This report describes the first case of capecitabine induced leukoencephalopathy causing restricted diffusion along the corticospinal tracts, which should be differentiated from other entities that involve the corticospinal tracts (i.e. amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS), hypoglycemic coma, etc.).
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PMID:Capecitabine-induced leukoencephalopathy involving the bilateral corticospinal tracts. 2720 Jan 61