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Amyotrophic Lateral Sclerosis

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- See: - a onclick="window.open(this.href,'','resizable=no,location=no,menubar=no,scrollbars=yes,status=no,toolbar=no,fullscreen=no,dependent=no,status'); return false" href="http://www.ncbi.nlm.nih.gov/pubmed/11464847">El Escorial Revisited: Revised Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis br/>
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      - a href="/nerves/neuropathic-disorders/">Disorders of Nerves/a>: br/>
      - a href="/muscles-tendons/spinal-disorders-involving-the-anterior-horn-cells/">Anterior Horn/a> Cell Disorders: br/>
      - a onclick="window.open(this.href,'','resizable=no,location=no,menubar=no,scrollbars=yes,status=no,toolbar=no,fullscreen=no,dependent=no,status'); return false" href="http://www.als.ca/">ALS Society Homepage/a> br/>
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strong>- Discussion:/strong> br/>
    - amyotrophic lateral sclerosis is a syndrome characterized by both upper and lower motor neuron disease; br/>
            - destruction of a href="/muscles-tendons/spinal-disorders-involving-the-anterior-horn-cells/">anterior horn/a> cells & descending corticospinal tracts; br/>
            - some patients will demonstrate more upper motor neuron disease where as others show more lower motor dz; br/>
    - disease is generally acquired and etiology is unknown; br/>
    - most common in middle & later life & rarely occurs in childhood; br/>
    - strong>clinical course:/strong> br/>
            - symptoms include progressive muscle wasting, weakness, & a href="/muscles-tendons/fibrillations/">fasciculations/a>; br/>
            - symptoms may begin in arms or legs or may begin with slurred speech; br/>
            - muscles are affected randomly, but eventually respirator failure develops; br/>
            - in 25% of pts the dz is slowly progressive, however, most pts follow a more fulminant course to early death; br/>
    - strong>diff dx:/strong> it is important to consider other conditions since these can be successfully treated; br/>
            - multifocal motor neuropathy br/>
                  - can be treated with cyclophosphamide or IV immune globulin; br/>
            - a href="/bones/spine/myelopathy/">cervical spondylotic myelopathy/a> br/>
            - X-linked spinobulbar muscular atrophy br/>
                  - (Kennedy's disease): slowly progressive lower motor neuron disease of the proximal limbs, usually affecting the upper extremities and bulbar muscles; br/>
            - a href="/muscles-tendons/myasthenia-gravis/">myasthenia gravis/a> br/>
            - lead intoxication br/>
            - a href="/arthritis/lyme-disease-erythema-chronicum-migrans/">lyme disease/a> br/>
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strong>- Exam:/strong> br/>
    - clinical presentation is often painless weakness of the arms or legs; br/>
    - manifests with lower motor neuron symptoms of muscle wasting, weakness, and fasciculation; br/>
    - contralateral limb may have hyperreflexia; br/>
    - the ocular muscles and voluntary muscles to the bladder and bowel are often spared; br/>
    - sensation is always normal; br/>
    - clonus: br/>
          - a href="http://www.nejm.org/doi/full/10.1056/NEJMicm1203111#figure=f1">Clonus after a Stroke/a>br/>
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strong>- Biopsy:/strong> br/>
    - muscle bx is particularly useful in dx of a href="/muscles-tendons/spinal-muscular-atrophy/">spinal muscular atrophy/a> or amyotrophic lateral sclerosis. br/>
    - degeneration occurs in small clusters of fibers (small group atrophy). br/>
    - later on, larger numbers of axons degenerate; br/>
    - those fibers supplied by axons not yet involved remain normal; br/>
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strong>- a href="/muscles-tendons/electromyography/">EMG Findings/a>:/strong> br/>
    - normal or low compound motor action potentials br/>
    - normal sensory-nerve action potentials br/>
    - evidence of both active (fibrillation potentials, positive sharp waves, and fasciculation potentials) br/>
    - chronic (large-amplitude, large-duration motor-unit action potentials) denervation in multiple sites without conduction block br/>
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strong>References/strong>br/>
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a onclick="window.open(this.href,'','resizable=no,location=no,menubar=no,scrollbars=yes,status=no,toolbar=no,fullscreen=no,dependent=no,status'); return false" href="http://www.ncbi.nlm.nih.gov/pubmed/11386269">Amyotrophic Lateral Sclerosis/a>.  br/>
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a ref="ordinalpos=1&ncbi_uid=16855271&link_uid=16855271&linksrc=docsum_title" onclick="window.open(this.href,'','resizable=no,location=no,menubar=no,scrollbars=yes,status=no,toolbar=no,fullscreen=no,dependent=no,status'); return false" href="http://www.ncbi.nlm.nih.gov/pubmed/16855271">Case records of the Massachusetts General Hospital. Case 22-2006--a 77-year-old man with a rapidly progressive gait disorder./a>br/>
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a onclick="window.open(this.href,'','resizable=no,location=no,menubar=no,scrollbars=yes,status=no,toolbar=no,fullscreen=no,dependent=no,status'); return false" href="http://www.nytimes.com/2010/08/18/sports/18gehrig.html?hpw">Study Says Brain Trauma Can Mimic A.L.S./a>/div>br/>hr/>br/>br/>br/>Last updated by Clifford R. Wheeless, III, MD on Monday, February 25, 2013 1:33 pmbr/>

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