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TeleEMG Interview What is Lou Gehrig's disease or ALS amyotrophic lateral sclerosis 20151012

Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment: report of the Quality Standards Subcommittee of the American Academy of Neurology.

Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment: report of the Quality Standards Subcommittee of the American Academy of Neurology. Author information:Department of Neurology, California Pacific Medical Center, San Francisco, California, USA. OBJECTIVE: To systematically review evidence bearing on the management of patients with amyotrophic lateral sclerosis. METHODS: The authors analyzed studies from 1998 to 2007 to update the 1999 practice parameter. Topics covered in this section include breaking the news, multidisciplinary clinics, symptom management, cognitive and behavioral impairment, communication, and palliative care for patients with ALS. 

RESULTS: The authors identified 2 Class I studies, 8 Class II studies, and 30 Class III studies in ALS, but many important areas have been little studied. More high-quality, controlled studies of symptomatic therapies and palliative care are needed to guide management and assess outcomes in patients with ALS. RECOMMENDATIONS: Multidisciplinary clinic referral should be considered for managing patients with ALS to optimize health care delivery and prolong survival and may be considered to enhance quality of life. For the treatment of refractory sialorrhea, botulinum toxin B should be considered and low-dose radiation therapy to the salivary glands may be considered. For treatment of pseudobulbar affect, dextromethorphan and quinidine should be considered if approved by the US Food and Drug Administration. 

For patients who develop fatigue while taking riluzole, withholding the drug may be considered. Because many patients with ALS demonstrate cognitive impairment, which in some cases meets criteria for dementia, screening for cognitive and behavioral impairment should be considered in patients with ALS. Other management strategies all lack strong evidence. 

Keywords: [“patient”,”considered”,”ALS”]

About ALS

While no one knows the cause of amyotrophic lateral sclerosis, people living with the disease and their families now have a role in finding a cause and cure, in part by registering for a new national ALS database. ALS, commonly known as Lou Gehrig’s disease, weakens and eventually destroys the body’s motor neurons, resulting in muscle weakness, paralysis and eventually death. While the cause of ALS is unknown, research shows that veterans – particularly those who served in the Gulf War – are about twice as likely to develop ALS. Identifying common threads in data could help find a cure. The hope of the National ALS Registry is to identify common threads of individuals with ALS in hopes of nailing down a root cause or that many factors together cause it. 

It is important to include as many people with the disease as possible in the registry in order to ensure the most accurate information. Individuals with ALS who choose to register for the database will be asked questions about their health, job, military history, physical activity and family histories. That data will be assessed along with collection data from selected states about the number of people in their areas diagnosed with ALS to evaluate the completeness and accuracy of the data in the ALS Registry. Information provided to the Registry will be kept private, accessible only to approved registry scientists. The data will not be accessible to employers or insurers, and registrants cannot be looked up in the registry by name. 

Registrants will be reminded via email every six months to update their information. Access special recources reserved for registry participants. The Registry also includes several resources for individuals living with ALS and their families, including clinical trials information, ALS clinic information, fact sheets, reports and journal articles and continuing education credits for health professionals. 

Keywords: [“ALS”,”Registry”,”information”]

Cerebral degeneration in amyotrophic lateral sclerosis

AbstractBackground We investigated cerebral degeneration and neurochemistry in patients with amyotrophic lateral sclerosis using magnetic resonance spectroscopy. Methods We prospectively studied 65 patients and 43 age-matched healthy controls. Participants were recruited from 4 centers as part of a study in the Canadian ALS Neuroimaging Consortium. Metabolites reflecting neuronal integrity and gliosis, as well as creatine and choline, were quantified in the midline motor cortex and midline prefrontal cortex. Comparisons were made between patients with ALS and healthy controls. 

Metabolites were correlated with clinical measures of upper motor neuron dysfunction, disease progression rate, and cognitive performance. Results In the motor cortex, tNAA/Cr, tNAA/Cho, and tNAA/Ino ratios were reduced in the ALS group compared with controls. Group differences in tNAA/Cr and tNAA/Cho in the prefrontal cortex displayed reduced ratios in ALS patients; however, these were not statistically significant. Reduced motor cortex ratios were associated with slower foot tapping rate, whereas only motor tNAA/Ino was associated with finger tapping rate. Disease progression rate was associated with motor tNAA/Cho. 

Verbal fluency, semantic fluency, and digit span forwards and backwards were associated with prefrontal tNAA/Cr. Conclusions This study demonstrates that cerebral degeneration in ALS is more pronounced in the motor than prefrontal cortex, that multicenter MRS studies are feasible, and that motor tNAA/Ino shows promise as a potential biomarker. 

Keywords: [“motor”,”cortex”,”ALS”]

ALS Symptoms, Treatment & Diagnosis

ALS is one of a group of diseases known as motor neuron diseases. This is called respiratory failure and is by far the leading cause of death for people with ALS.Most people with ALS die within five years of the onset of symptoms. We do not know exactly what causes ALS. Although some cases of ALS are hereditary, most are not. Using an evidence-based approach, smoking was found to be more likely than not a risk factor for ALS based on two excellent studies by Kamel et al. 

Finally, some patients with ALS frequently exhibit a so-called pseudobulbar affect, manifesting as involuntary, uncontrolled outbursts of crying or laughter, which is distinct from their underlying mood. As the disease progresses, the person with ALS loses the ability to carry out everyday activities such as dressing, eating, and working. Hereditary and nonhereditary cases of ALS may have similar symptoms and course, but in some forms of ALS these manifestations may be quite distinct. At onset, the signs and symptoms of ALS are often very subtle. No one test gives a definitive diagnosis of ALS. 

If you are having symptoms that suggest ALS, your health care provider will conduct a complete workup. It is important to distinguish ALS from other conditions because many of these conditions can be improved with appropriate treatment. This test can distinguish between ALS and other conditions with similar symptoms. ALS is a slowly progressive disease causing gradual disability. 

Keywords: [“al”,”muscle”,”disease”]