Presentation
Such syndromes may be present in as many as 60% of patients with ALS. Conversely, the occurrence of motor neuron dysfunction in patients with clinically pure frontotemporal dementia is increasingly recognized. [books.google.com]
Y, Tada M, Shiga A, Miura T, Aoki K, Ikeuchi T, Nishizawa M, Kakita A, Takahashi H Organizer 26th International Symposium on ALS/MND Place of Presentation Orlando, USA Year and Date 2015-12-11 Int'l Joint Research Data Source [Presentation] Distinct [nrid.nii.ac.jp]
[…] in ALS (which can present in a single extremity mimicking cubital/carpal tunnel syndrome), but absent in peripheral neuropathy ALS has normal sensory studies on EMG/NCS Treatment Nonoperative currently no cure or effective treatment goals of treatment [orthobullets.com]
Dementia and amyotrophic lateral sclerosis can present within other disorders. [medlink.com]
It is characterized by difficulty chewing and swallowing, and slurred speech (manifestations that may also represent the initial presentation of ALS). [mdedge.com]
Entire Body System
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Rigor
Each session is the result of a rigorous process which takes months to prepare. This attention to detail ensures that our audiences receive up-to-date, scientifically accurate, and usable information. [cdc.gov]
Malcolm Sperrin, Director of Medical Physics and Clinical Engineering at the Royal Berkshire NHS Foundation Trust, said: “This is a well-designed study that has been rigorously peer-reviewed and as such carries a lot of credibility. [sciencemediacentre.org]
Psychiatrical
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Behavior Disorder
disorders Amyotrophic lateral sclerosis syndrome (ALS) Bulbar dysfunction: Dysphagia Limb denervation Upper motor neuron signs in limbs Hyperreflexia Spasticity less prominent in some patients Fasciculations: May occur without signs of ALS Course & associations [neuromuscular.wustl.edu]
Neurologic
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Hyporeflexia
[…] asymmetric with single limb involvement [2] hands & arms involved more than legs hand muscles involved early muscle weakness muscle wasting / muscle atrophy ( lower motor neuron sign ) muscle stiffness muscle cramping muscle twitching hyperreflexia / hyporeflexia [anvita.info]
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Memory Impairment
The impact of semantic memory impairment on spelling: evidence from semantic dementia. Neuropsychologia. 2000;38(2):143–63. pmid:10660226 View Article PubMed/NCBI Google Scholar 53. Woollams AM, Patterson K. [journals.plos.org]
Workup
Therefore, it is prudent to complete screening for anxiety and depression as part of a comprehensive cognitive workup in patients with ALS. 9 ALS-Associated Clinical Scenarios and Decisional Capacity Since ALS is a progressive neuromuscular disease that [jaapl.org]
The findings also suggest that motor speech assessment should be performed as part of the diagnostic workup for patients with FTD. [journals.plos.org]
Treatment
Learning more about these responses may provide insights into new treatment modalities for PD. In the coming year, Dr. [books.google.com]
Riluzole: Riluzole (Rilutek) is the only FDA-approved medication for the treatment of ALS. [uspharmacist.com]
Although medical decisional capacity is required for every treatment decision made, its importance becomes paramount when making decisions on complex medical treatments that will invariably and significantly affect quality of life or life itself. [jaapl.org]
The primary goals of treatment are aimed at helping the patient cope with the effects of the disease and at preventing or relieving complications. A great deal of research is devoted to finding an effective treatment for the disease. [aanem.org]
Drug Treatment In 1995, the FDA approved riluzole (Rilutek), which was the first drug for the treatment of ALS. Riluzole is a medication that is taken by mouth (i.e. a pill). [caregiver.org]
Prognosis
LEARNING OBJECTIVES Describe the epidemiology, risk factors, etiology, pathogenesis, diagnosis, and general prognosis of amyotrophic lateral sclerosis (ALS). Compare and contrast the El Escorial diagnostic criteria for ALS. [fadavispt.mhmedical.com]
Progressive muscular atrophy is less common than ALS but has a better prognosis. It can be a painstaking process to make the diagnosis of progressive muscular atrophy since the symptoms are similar to other conditions. [verywell.com]
Age at diagnosis continues to be the strongest predictor of prognosis; recall case reporting bias may play a role in estimates of survival time. [raredr.com]
The symptoms of ‘autonomic storm’ are similar to that of ‘baroreflex failure’, and ‘autonomic storm’ indicates poor prognosis and may result in sudden death. [touchneurology.com]
Because of the challenges in diagnostic evaluation, the rapidly evolving nature of the disease, and the dire prognosis of ALS, any patient suspected of having ALS should be referred immediately to a neurologist. [mdedge.com]
Etiology
LEARNING OBJECTIVES Describe the epidemiology, risk factors, etiology, pathogenesis, diagnosis, and general prognosis of amyotrophic lateral sclerosis (ALS). Compare and contrast the El Escorial diagnostic criteria for ALS. [fadavispt.mhmedical.com]
The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. {ECO:0000269 PubMed:11586298}. [genecards.org]
Guamanian amyotrophic lateral sclerosis associated with dementia seems to differ from dementia associated with sporadic or familial types of amyotrophic lateral sclerosis in etiology and pathology. [medlink.com]
Prognosis is poor; previous estimates of median survival from disease onset to death range from 20 to 48 months.[ 1 – 3 ] In most cases etiology is unknown, although a small proportion of cases appear to be of genetic origin.[ 4 ] A recent estimate of [raredr.com]
[…] disease & frontotemporal dementia, IBMPFD, muscular dystrophy limb-girdle with Paget disease of bone, pagetoid neuroskeletal syndrome, lower motor neuron degeneratio... primary juvenile lateral sclerosis sporadic amyotrophic lateral sclerosis (ALS) Etiology [anvita.info]
Epidemiology
However, current global epidemiological data are not available in the literature. [karger.com]
Research methods in occupational epidemiology. 2nd ed. New York, NY: Oxford University Press, Inc.; 2004. Breslow NE, Day NE. Statistical methods in cancer research. Volume II—the design and analysis of cohort studies. [imic.or.jp]
LEARNING OBJECTIVES Describe the epidemiology, risk factors, etiology, pathogenesis, diagnosis, and general prognosis of amyotrophic lateral sclerosis (ALS). Compare and contrast the El Escorial diagnostic criteria for ALS. [fadavispt.mhmedical.com]
Neil Pearce, Professor of Epidemiology and Biostatistics at the London School of Hygiene & Tropical Medicine, said: “Several previous studies have found that electrical workers are at increased risk of ALS. [sciencemediacentre.org]
Relevant External Links for ALS2 Genetic Association Database (GAD) ALS2 Human Genome Epidemiology (HuGE) Navigator ALS2 Atlas of Genetics and Cytogenetics in Oncology and Haematology: ALS2 No data available for Genatlas for ALS2 Gene Infantile-onset [genecards.org]
Pathophysiology
These observations may suggest a limbic origin for the autonomic storm, although the precise pathophysiology remains to be elucidated. [touchneurology.com]
Conversely, the extensive application of modern MR imaging based techniques to the study of ALS has undoubtedly improved our understanding of disease pathophysiology and is likely to have a role in the identification of potential biomarkers of disease [ajnr.org]
Immediate and additional causes of death were categorized using an author-developed algorithm based on pathophysiology and potential relevance to patients with advanced ALS ( Table 1 ). Table 1. [raredr.com]
Prevention
Because of the rapidly progressive nature of the disease, ALS requires close physician follow-up and appropriate interventions to help maintain quality of life and prevent complications. [aanem.org]
[…] in a single extremity mimicking cubital/carpal tunnel syndrome), but absent in peripheral neuropathy ALS has normal sensory studies on EMG/NCS Treatment Nonoperative currently no cure or effective treatment goals of treatment provide supportive care prevent [orthobullets.com]
However if this finding is real, it is important as it identifies a new, preventable cause of ALS. [sciencemediacentre.org]
Adequate hydration and stretching exercises are the best preventive measures for cramps. If cramps are severe, quinine sulfate, baclofen, dantrolene, gabapentin, benzodiazepines, or carbamazepine may be prescribed. [uspharmacist.com]