Clinical Diagnosis

The clinical diagnosis of ALS is suggested when there are signs of both upper and lower motor neuron dysfunction without other CNS dysfunction. ALS-like symptoms can be seen with other systemic illnesses such as diabetes, dysproteinemia, thyroid dysfunction, vitamin B 12 deficiency, lead toxicity, and vasculitis, as well as CNS and spinal cord tumors. The diagnosis of ALS also requires that other inflammatory neuropathies such as myasthenia gravis be excluded. Established criteria for the diagnosis of ALS, the El Escorial criteria, have been developed by the World Federation of Neurology. 7

Electromyography (EMG) is the most useful diagnostic tool, showing decreased muscle action potential amplitude and decremental responses to repetitive nerve stimulation

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