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Amyotrophic Lateral Sclerosis Amyotrophic Lateral Sclerosis

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Amyotrophic Lateral Sclerosis Amyotrophic Lateral Sclerosis

  1. 1. Amyotrophic Lateral Sclerosis Jaffar Khan, MD Assistant Professor of Neurology Emory University
  2. 2. Motor Neuron Disease Terminology Lower motor neuron Upper motor neuron     Progressive Muscular Atrophy Amyotrophic Lateral Sclerosis Primary Lateral Sclerosis
  3. 3. Amyotrophic Lateral Sclerosis Pathology <ul><li>Degeneration and death of motor nerves </li></ul><ul><ul><li>Upper Motor Neuron </li></ul></ul><ul><ul><ul><li>within brain/spinal cord </li></ul></ul></ul><ul><ul><li>Lower Motor Neurons </li></ul></ul><ul><ul><ul><li>leaves brain (stem)/spinal cord </li></ul></ul></ul><ul><li>Relatively spared </li></ul><ul><ul><li>Eye movements and bowel/bladder function </li></ul></ul>
  4. 4. Amyotrophic Lateral Sclerosis Epidemiology <ul><li>Etiology – unknown </li></ul><ul><li>Average age of onset mid-50’s </li></ul><ul><li>Mode of transmission </li></ul><ul><ul><li>Sporadic – 90-95% </li></ul></ul><ul><ul><li>Familial – 5-10% (autosomal dominant) </li></ul></ul>
  5. 5. Amyotrophic Lateral Sclerosis Epidemiology <ul><li>Male : Female – 3:2 </li></ul><ul><li>U.S. Prevalence: 30,000 </li></ul><ul><li>Incidence 1-2.5 / 100,000 </li></ul><ul><li>Isolated areas of increased incidence </li></ul><ul><ul><li>Kii peninsula of Japan </li></ul></ul><ul><ul><li>Chamorro natives of Guam </li></ul></ul>
  6. 6. Amyotrophic Lateral Sclerosis Clinical Presentation <ul><li>Lower motor neuron signs </li></ul><ul><ul><li>Weakness, muscle wasting, hyporeflexia, muscle cramps, fasciculations </li></ul></ul><ul><li>Upper motor neuron signs </li></ul><ul><ul><li>Spasticity, hyperreflexia, weakness </li></ul></ul>
  7. 7. Amyotrophic Lateral Sclerosis Clinical Presentation <ul><li>Asymmetric Weakness – most common </li></ul><ul><li>Onset single limb or bulbar </li></ul><ul><li>Local spread then regional spread </li></ul><ul><ul><li>Bulbar, cervical, thoracic, lumbosacral </li></ul></ul><ul><li>Fasciculations </li></ul>
  8. 8. Amyotrophic Lateral Sclerosis Diagnosis <ul><li>Prominent upper and lower neuron signs with a progressive course without significant sensory or sphincter abnormalities </li></ul><ul><li>Laboratory investigation to search for a more treatable condition </li></ul>
  9. 9. Amyotrophic Lateral Sclerosis Clinical Signs and Symptoms <ul><li>Weakness </li></ul><ul><li>Hyporeflexia </li></ul><ul><li>Pain and cramps </li></ul><ul><li>Fasciculations </li></ul><ul><li>Wasting </li></ul><ul><li>Spasticity </li></ul><ul><li>Hyperreflexia </li></ul><ul><li>Babinski’s sign </li></ul><ul><li>Emotional Lability </li></ul>
  10. 10. Amyotrophic Lateral Sclerosis Atypical Features <ul><li>Dementia - < 5 % </li></ul><ul><li>Sensory loss – atypical </li></ul><ul><li>25% complain of paresthesias </li></ul><ul><li>Oculomotor dysfunction </li></ul><ul><li>Bowel or bladder dysfunction </li></ul>
  11. 11. Amyotrophic Lateral Sclerosis Diagnosis <ul><li>Two experienced Neurologists </li></ul>Laboratory Studies <ul><li>No study to prove or disprove </li></ul><ul><li>Look for an alternate diagnosis </li></ul>
  12. 12. Amyotrophic Lateral Sclerosis Laboratory Studies <ul><li>Nerve conduction studies </li></ul><ul><ul><li>assess for demyelinating vs. axonal involvement </li></ul></ul><ul><li>Electromyography </li></ul><ul><ul><li>confirm ALS </li></ul></ul><ul><ul><li>myopathy </li></ul></ul>
  13. 13. Amyotrophic Lateral Sclerosis Laboratory Studies <ul><li>MRI cervical spine </li></ul><ul><ul><li>Cervical Spondylosis with cord compression </li></ul></ul><ul><ul><li>Herniated disc </li></ul></ul><ul><ul><li>Syrinx </li></ul></ul>
  14. 14. Amyotrophic Lateral Sclerosis Laboratory Studies <ul><li>ESR – inflammatory/malignancy </li></ul><ul><li>SPEP – monoclonal gammopathy </li></ul><ul><li>TSH – hyperthyroidism </li></ul><ul><li>B 12 – combined systems degeneration </li></ul><ul><li>Calcium/PTH - hyperparathyroidism </li></ul>
  15. 15. Amyotrophic Lateral Sclerosis Prognosis <ul><li>Variable – difficult to predict in an individual patient </li></ul><ul><li>50% live 3-4 or more years </li></ul><ul><li>20% live 5 or more years </li></ul><ul><li>10% live 10 or more years </li></ul><ul><li>Occasional patients live 20 years </li></ul>
  16. 16. Amyotrophic Lateral Sclerosis Treatment <ul><li>Rilutek </li></ul><ul><li>2 large clinical trials </li></ul><ul><ul><li>Bulbar onset </li></ul></ul><ul><ul><li>Entire population </li></ul></ul><ul><li>Endpoint </li></ul><ul><ul><li>Death </li></ul></ul><ul><ul><li>Ventilator dependence </li></ul></ul>
  17. 17. Amyotrophic Lateral Sclerosis Treatment <ul><li>Bulbar onset </li></ul><ul><ul><li>Prolonged survival </li></ul></ul><ul><ul><li>Improved muscle strength </li></ul></ul><ul><li>Entire population </li></ul><ul><ul><li>Prolonged survival </li></ul></ul><ul><ul><li>No effect on decline in muscle strength </li></ul></ul><ul><li>Prolonged survival an average of 2-3 months </li></ul>
  18. 18. Amyotrophic Lateral Sclerosis Rilutek 50 mg po bid <ul><li>Hepatotoxicity </li></ul><ul><ul><li>Serum transaminase levels </li></ul></ul><ul><ul><li>Check every month x 3 </li></ul></ul><ul><ul><li>Then every 3 months x 3 for the first year </li></ul></ul><ul><li>Adverse effects </li></ul><ul><ul><li>Neutropenia </li></ul></ul><ul><ul><li>Nausea/vomiting </li></ul></ul>
  19. 19. Amyotrophic Lateral Sclerosis Rilutek 50 mg po bid <ul><li>Reasons for not taking the drug </li></ul><ul><ul><li>Expense </li></ul></ul><ul><ul><li>Minimal benefit </li></ul></ul><ul><ul><li>Unwillingness to take a medication that would prolong life </li></ul></ul>
  20. 20. Amyotrophic Lateral Sclerosis Management <ul><li>weakness </li></ul><ul><li>fatigue </li></ul><ul><li>nutrition </li></ul><ul><li>dysphagia </li></ul><ul><li>feeding tube </li></ul><ul><li>dysarthria </li></ul><ul><li>communication </li></ul><ul><li>spasticity </li></ul><ul><li>cramps </li></ul><ul><li>pain </li></ul><ul><li>depression </li></ul><ul><li>anxiety </li></ul><ul><li>breathing </li></ul><ul><li>end-of -life </li></ul>
  21. 21. Amyotrophic Lateral Sclerosis Multidisciplinary Approach to Care <ul><li>Neurologist </li></ul><ul><li>Clinical/research nurse </li></ul><ul><li>Dietician </li></ul><ul><li>Speech/swallowing therapist </li></ul><ul><li>Family/caregivers </li></ul><ul><li>Psychologists </li></ul><ul><li>Physical therapist </li></ul><ul><li>Occupational therapist </li></ul><ul><li>Social worker </li></ul><ul><li>GI physician </li></ul><ul><li>Support organizations </li></ul><ul><li>Homehealth/hospice </li></ul><ul><li>Pulmonologist </li></ul>

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