Journal of the neurological sciences
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Clinical Trial
Patients' health-related quality of life and utilities associated with different stages of amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a devastating disease that has serious consequences in terms of impairments and disabilities, which are expected to impact on health-related quality of life (HRQL). The aim of the present study was to assess self-reported health status and HRQL, as well as patients' own valuation of their present health state in a sample of patients with different levels of severity of ALS. ⋯ The results from the ALSAQ-40 and EQ-5D descriptive system indicate that patients' HRQL decreases systematically with increasing severity of disease. Patients' mean VAS rating of their own health ranged from 0.74 for stage 1 (early) disease severity, to 0.37 for stage 4 (late stage) disease severity. Utilities elicited via SG were systematically higher than VAS scores and ranged from a mean of 0.79 for stage 1 disease severity to a mean of 0.45 for stage 4 disease severity.
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Multicenter Study
Amyotrophic lateral sclerosis care in Italy: a nationwide study in neurological centers.
Recently, formal standards for the management of amyotrophic lateral sclerosis (ALS) have been proposed by the American Academy of Neurology (AAN). However, there are few information about the actual care of ALS. We have assessed the management of ALS in Italy in various clinical settings, on basis of a self-reported questionnaire. ⋯ Respiratory management seemed to be lacking both in large and in small centers since non-invasive positive pressure ventilation (NIPPV) was proposed by only 70% of large and 50% of small centers; however, the percentage of patients who underwent NIPPV was significantly higher in large centers (p=0.03). Moreover, the discussion of respiratory issues was performed quite late in the course of the disease, usually when the patients have first respiratory symptoms. Therefore, there are considerable opportunities to improve the care of ALS patients in Italy, primarily through the education of neurologists on AAN standards of care for ALS.
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Case Reports
Recurrent jaw dislocation after botulinum toxin treatment for sialorrhoea in amyotrophic lateral sclerosis.
Botulinum toxin (BTX) has been used successfully to treat various movement disorders, and is increasingly used for many other medical conditions. Sialorrhoea is a disabling symptom in many neurological patients including those with Parkinson's disease, stroke and amyotrophic lateral sclerosis (ALS). ⋯ We report an ALS patient who developed recurrent jaw dislocation following BTX treatment for sialorrhoea to highlight the observation that intraparotid BTX may be complicated by jaw dislocations in some at-risk ALS patients. Clinicians using BTX to treat sialorrhoea in ALS need to be aware of this potentially serious complication.
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Elevated anti-ganglioside antibody levels mainly of anti-GM1 and anti-GD1a specificities have been reported in THE serum of patients with Guillain-Barré syndrome (GBS). The relevance of anti-ganglioside antibodies other than anti-GM1 and anti-GD1a IgG antibodies and the temporal profile of anti-ganglioside antibodies in GBS is less clear. We studied serum antibodies to GM1, GD1a, GD1b, GQ1b, sulfatide and cardiolipin of the IgM, IgG and IgA classes over the course of GBS in patients who were untreated or treated with high dose intravenous immunoglobulin (IvIg). ⋯ Anti-GM1 IgG and anti-GD1a IgM antibodies are thus strongly associated with more severe- and predominantly axonal cases of GBS. The appearance of anti-GM1 IgG and anti-GD1a antibody peaks in the serum after the termination of the acute phase of GBS suggests that these antibodies are produced secondary to nerve damage in GBS. The data does not exclude the possibility that secondarily secreted anti-GM1 IgG and anti-GD1a IgM antibodies may themselves be biologically active and play a role in disease propagation and/or recovery from disease in some patients with GBS.
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The majority of studies on neuropsychological complications after cardiac surgery used the raw variation of selective tests scores to define the occurrence of cognitive decline. We prospectively estimated the frequency of cognitive impairment after cardiac surgery, with a particular emphasis on persistent and clinically relevant cognitive decline. Possible baseline and operative predictors were also evaluated. ⋯ A considerable proportion of cardiac surgery patients may undergo clinically relevant cognitive impairment. The knowledge of variables influencing cognitive outcome is essential for the adoption of preventive measures.