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Blood pressure control
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Physical therapy
Pyridostigmine
Riluzole
Steroids
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This patient who presents with weakness and is found to have mixed upper motor neuron (spasticity/hyperreflexia) and lower motor neuron (atrophy/areflexia) signs most likely has amyotrophic lateral sclerosis. This disease can be treated with riluzole. Amyotrophic lateral sclerosis presents with progressive weakness affecting any segment of the body. This most commonly manifests as asymmetric limb weakness with a combination of upper and lower motor neuron signs. These signs should be carefully evaluated on the physical exam because the combination of these signs is virtually diagnostic of this disease. Upper motor neuron signs include spasticity, hyperreflexia, and a positive Babinski sign. Lower motor neuron signs include atrophy, fasciculations, and hyporeflexia. Riluzole is the only medication that is indicated for this disease, and it functions by decreasing neuronal excitotoxicity. There is significant morbidity and mortality associated with amyotrophic lateral sclerosis despite treatment. Geevasinga et al. discuss the mechanisms by which riluzole prolongs survival in patients with amyotrophic lateral sclerosis. They find that its effects are mediated by inhibition of glutaminergic transmission and antagonization of sodium channel function. They recommend its use in this condition. Incorrect Answers: Answer 1: Blood pressure control is indicated in the treatment of hypertensive strokes; however, a stroke would present with a loss of motor function that occurs acutely rather than over the period of 3 months. Further management may include managing dyslipidemia and hyperglycemia. Answer 2: Physical therapy is indicated in patients who have peripheral musculoskeletal weakness caused by strains, sprains, or deconditioning; however, the presence of upper motor neuron signs in this patient is indicative of an underlying process. While physical therapy may be indicated in amyotrophic lateral sclerosis, it will not necessarily change the course of the disease. Answer 3: Pyridostigmine is indicated in patients with myasthenia gravis; however, the weakness from this disease would present with gradual progressive weakness during the course of the day and is usually symmetric. It is worsened with exertion and relieved with rest. The ice pack test may be revealing in this condition as well. Answer 5: Steroids are indicated in patients with multiple sclerosis, which can also present with asymmetric weakness that occurs sporadically; however, this disease usually presents in young women and has associated central nervous system manifestations such as optic neuritis, vertigo, or internuclear ophthalmoplegia. The deficits in multiple sclerosis may demonstrate a relapsing and remitting pattern. In contrast, amyotrophic lateral sclerosis only affects motor neurons so sensory defects are not seen in this disease. Bullet Summary: Amyotrophic lateral sclerosis presents with a combination of upper and lower motor neuron signs and can be treated with riluzole.
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