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)

Respiratory insufficiency is a problem that develops in nearly all people diagnosed with amyotrophic lateral sclerosis (ALS). A 46-year-old man with ALS, who had been in a bedridden state with tracheal ventilation support, complained of faintness and dyspnea. The airway pressure of the ventilator had increased, and bleeding from the trachea had occurred several times. A fiberoptic bronchoscopy showed granulation located on the anterior wall of the trachea and severe airway obstruction of the tracheostomy tube. Although a long tracheostomy tube had been intubated for the initial management of the tracheal granulation, a tumor on the posterior tracheal wall had relapsed and occluded the tracheal lumen. A self-expandable metallic airway stent was placed into the tracheal stenosis. After stenting, his symptoms of dyspnea and syncope imploved, and the increased airway pressure of the ventilator was normalized. We speculated that the tracheal granuloma had occurred due to a tracheal mucosal injury related to endotracheal suctioning. We should pay attention to complaints of dyspnea in ALS patients with tracheostomy and make a careful consideration to airway care including suction management.
 ...
PMID:[Unexpected treatable dyspnea caused by intratracheal granuloma in an amyotrophic lateral sclerosis patient with mechanical ventilation]. 1801 17

Sympathetic nerve functions in patients with amyotrophic lateral sclerosis (ALS), spinocerebellar degeneration (SCD), and Parkinson disease (PD) were evaluated using microneurography. In patients with ALS, the muscle sympathetic nerve activity (MSNA) at rest was greater than that in healthy subjects and patients with other neuromuscular disorders. This finding is not observed in the advanced stages of ALS. The resting frequency of skin sympathetic nerve activity (SSNA) significantly greater in ALS patients than in healthy controls. In patients with SCD, the MSNA was slightly reduced, although these patients did not experience orthostatic dizziness or syncope, or significant fall in blood pressure during the head-up tilt test. The reflex latency of SSNA induced by electric stimulation was slightly but significantly prolonged in patients with cortical cerebellar atrophy and markedly prolonged in patients with multiple system atrophy-C (MSA-C). In patients with PD, a significantly negative correlation was observed between MSNA and age, and between MSNA and disease duration. In these patients, the resting frequency of SSNA was significantly lower than in healthy controls, while SSNA reflex latencies were similar to those in the controls. Further investigations to determine whether the autonomic nervous system was impaired are imperative for elucidating the pathogenesis of neurodegenerative disorders.
 ...
PMID:[Sympathetic neurograms in patients with neurodegenerative disorders--an overview]. 1930 96

Microneurography is a unique neurophysiological technique allowing direct recording of unmyelinated postganglionic sympathetic or afferent nociceptive fibers by tungsten needles inserted into a peripheral nerve fascicle. In recent years, microneurography has been used to ascertain autonomic impairments in central neurological disorders such as sleep disorders, Parkinson's disease, amyotrophic lateral sclerosis, or vasovagal syncope. Abnormal resting muscle sympathetic nerve activity (MSNA) and skin sympathetic nerve activity (SSNA) or the abnormal sympathetic response to arousal have been described in these disorders, thereby clarifying important pathophysiological aspects of the underlying impairment. In addition, microneurography was also recently used to demonstrate absent or decreased sympathetic outflow in diseases affecting the peripheral nervous system such as Ross syndrome, pure autonomic failure, and small-fiber neuropathy. Microneurography has also been used to study nociceptor outflow in pain disorders affecting the peripheral nervous system such as small-fiber neuropathy, diabetic neuropathy, erythromelalgia, complex regional pain syndrome, and fibromyalgia. In these disorders, microneurography mainly documented mechano-insensitive C-nociceptor hyperexcitability that might account for the ongoing pain.
...
PMID:Microneurographic recording from unmyelinated nerve fibers in neurological disorders: an update. 2545 52