Autonomic impairment in amyotrophic lateral sclerosis

Baltadzhieva, R.; Gurevich, Tanya; Korczyn, Amos D.

doi:10.1097/01.wco.0000183114.76056.0e

Cited by 137 publications

(106 citation statements)

References 54 publications

(95 reference statements)

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“…Further features of interest include creatine kinase, whose levels highly correlate with creatinine levels, suggested to be predictive of prognosis in ALS 42,43 . Pulse and blood pressure, which at first glance may be surprising predictive features for ALS, are supported by a body of literature suggesting sympathetic dysfunction in ALS [44][45][46][47] . Further research and studies are needed to assess the potential of these features and elucidate their involvement in ALS pathophysiology.…”

Section: Discussionmentioning

confidence: 87%

Crowdsourced analysis of clinical trial data to predict amyotrophic lateral sclerosis progression

et al. 2014

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Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with substantial heterogeneity in its clinical presentation. This makes diagnosis and effective treatment difficult, so better tools for estimating disease progression are needed. Here, we report results from the DREAM-Phil Bowen ALS Prediction Prize4Life challenge. In this crowdsourcing competition, competitors developed algorithms for the prediction of disease progression of 1,822 ALS patients from standardized, anonymized phase 2/3 clinical trials. The two best algorithms outperformed a method designed by the challenge organizers as well as predictions by ALS clinicians. We estimate that using both winning algorithms in future trial designs could reduce the required number of patients by at least 20%. The DREAM-Phil Bowen ALS Prediction Prize4Life challenge also identified several potential nonstandard predictors of disease progression including uric acid, creatinine and surprisingly, blood pressure, shedding light on ALS pathobiology. This analysis reveals the potential of a crowdsourcing competition that uses clinical trial data for accelerating ALS research and development

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Section: Discussionmentioning

confidence: 87%

Crowdsourced analysis of clinical trial data to predict amyotrophic lateral sclerosis progression

et al. 2014

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“…14,93,94 ALS with urinary and autonomic involvement Apart from patients with a C9orf72 (GGGGCC)exp mutation, 95 symptoms indicative of autonomic nervous system involvement are rare in ALS, although specific testing can indicate subclinical involvement of cardiovascular, gastrointestinal and sudomotor systems, and the salivary and lacrimal gland. 96,97 Bladder problems are commonly believed not to be a feature of ALS, because Onuf 's nucleus is typically spared or only minimally affected. 3 Of note, urinary incontinence and retention certainly do occur in patients with ALS, but these problems are usually attributed to the use of muscle relaxants and anticholinergics, or to the patient's motor problems.…”

Section: Als With Sensory Involvementmentioning

confidence: 99%

The phenotypic variability of amyotrophic lateral sclerosis

2014

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| Classic textbook neurology teaches that amyotrophic lateral sclerosis (ALS) is a degenerative disease that selectively affects upper and lower motor neurons and is fatal 3-5 years after onset-a description which suggests that the clinical presentation of ALS is very homogenous. However, clinical and postmortem observations, as well as genetic studies, demonstrate that there is considerable variability in the phenotypic expression of ALS. Here, we review the phenotypic variability of ALS and how it is reflected in familial and sporadic ALS, in the degree of upper and lower motor neuron involvement, in motor and extramotor involvement, and in the spectrum of ALS and frontotemporal dementia. Furthermore, we discuss some unusual clinical characteristics regarding presentation, age at onset and disease progression. Finally, we address the importance of this variability for understanding the pathogenesis of ALS and for the development of therapeutic strategies.

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“…Accumulating evidence shows that autonomic nervous abnormalities are found in both early and advanced stages of ALS. 1 The autonomic dysfunction may be clinically evident and devastating, especially in advanced stage when ventilators are required. Here, the autonomic dysfunctions in ALS are reviewed.…”

Section: Amyotrophic Lateral Sclerosis (Als) Is a Neurodegenerative Dmentioning

confidence: 99%

Sympathetic Hyperactivity and Sympathovagal Imbalance in Amyotrophic Lateral Sclerosis

Shimizu¹

2012

European Neurological Review

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with progressive loss of upper and lower motor neurons. Autonomic nervous abnormalities, including sympathetic hyperactivity and sympathovagal imbalance, have been found in both early and advanced stages of ALS. In early stage, the dysfunction may be subclinical. Occasionally, elevated blood pressure or heart rate and increased sweating may be observed. In advanced stage when ventilators are required, the sympathetic hyperactivity may lead to hypertensive crisis without counter-regulation of heart rate, followed by the consecutive circulatory collapse, known as the 'autonomic storm'. The symptoms of 'autonomic storm' are similar to that of 'baroreflex failure', and 'autonomic storm' indicates poor prognosis and may result in sudden death. Careful evaluation and individual treatment are strongly suggested, although appropriate therapeutic approaches have not been established. Causative central nervous lesions remain to be elucidated, although the limbic system may be involved. The autonomic dysfunction further supports the concept that ALS is a multisystem-degenerative disease. KeywordsAmyotrophic lateral sclerosis, autonomic nervous system, sympathetic hyperactivity, sympathovagal imbalance, baroreflex Disclosure: The author has no conflicts of interest to declare. Clinical and Haemodynamic StudiesThe autonomic nervous system is divided into sympathetic and

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Autonomic impairment in amyotrophic lateral sclerosis

Abstract: The wide range of autonomic involvement, together with results suggesting cognitive and extrapyramidal dysfunction, supports the view that ALS is a multisystem degenerative disease.

Cited by 137 publications

References 54 publications

Crowdsourced analysis of clinical trial data to predict amyotrophic lateral sclerosis progression

Crowdsourced analysis of clinical trial data to predict amyotrophic lateral sclerosis progression

The phenotypic variability of amyotrophic lateral sclerosis

Sympathetic Hyperactivity and Sympathovagal Imbalance in Amyotrophic Lateral Sclerosis

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