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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive loss of voluntary motor function. The disease is almost always fatal, and approximately 50% of patients die within 3 to 4 years after onset of symptoms. [1,2] ALS is a distinct syndrome characterized by a combination of upper motor neuron (UMN) and lower motor neuron (LMN) involvement and steady progression. There has recently been a substantial amount of research into the pathophysiology and treatment of ALS. A wide variety of therapeutic approaches, including glutamate antagonists such as riluzole, neurotrophic factors such as IGF-1 and BDNF, and protease inhibitors, among others, are being investigated in an attempt to halt progression of the disease and to improve life expectancy.
This article is based on a consensus conference involving a group of experts who treat patients with ALS held on August 3, 1996 in San Francisco, California. The objectives were to establish a Standard of Care Consensus on Diagnosis and Management of ALS for neurologists and others who treat patients with the disease. The conference covered the aspects of diagnostic criteria, symptom management, and pharmacologic treatment.
Diagnostic criteria.
Specific guidelines for the diagnosis of ALS are listed in Table 1, Table 2, and Table 3. More detailed considerations in diagnosis are elaborated by Dr. Mitsumoto elsewhere in this issue.
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Table 1. World Federation of Neurology El Escorial diagnostic criteria for ALS3
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Table 2. Reasons for performing an EMG examination in ALS3
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Table 3. Other diagnostic tests performed in ALS3
Symptom management.
Initially, patients with ALS seek medical help to obtain a diagnosis and some form of treatment. As physicians become focused on the etiologic and diagnostic aspects of the disorder, patients become preoccupied with the clinical significance of specific clinical and laboratory findings. This preoccupation leads to increased anxiety and …
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