amyotrophic lateral sclerosis. motor neuron disease terminology lower motor neuron upper motor...
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Amyotrophic Lateral Sclerosis
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Motor Neuron DiseaseTerminology
Lower motor neuron Upper motor neuron
ProgressiveMuscular Atrophy
Amyotrophic Lateral Sclerosis
Primary LateralSclerosis
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Amyotrophic Lateral SclerosisPathology
Degeneration and death of motor nerves• Upper Motor Neuron
– within brain/spinal cord
• Lower Motor Neurons – leaves brain (stem)/spinal cord
Relatively spared• Eye movements and bowel/bladder function
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Amyotrophic Lateral SclerosisEpidemiology
Etiology – unknown Average age of onset mid-50’s Mode of transmission
• Sporadic – 90-95%• Familial – 5-10% (autosomal
dominant)
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Amyotrophic Lateral SclerosisEpidemiology
Male : Female – 3:2 U.S. Prevalence: 30,000 Incidence 1-2.5 / 100,000 Isolated areas of increased incidence
• Kii peninsula of Japan• Chamorro natives of Guam
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Amyotrophic Lateral SclerosisClinical Presentation
Lower motor neuron signs• Weakness, muscle wasting, hyporeflexia,
muscle cramps, fasciculations
Upper motor neuron signs• Spasticity, hyperreflexia, weakness
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Amyotrophic Lateral SclerosisClinical Presentation
Asymmetric Weakness – most common Onset single limb or bulbar Local spread then regional spread
• Bulbar, cervical, thoracic, lumbosacral Fasciculations
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Amyotrophic Lateral SclerosisDiagnosis
Prominent upper and lower neuron signs with a progressive course without significant sensory or sphincter abnormalities
Laboratory investigation to search for a more treatable condition
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Amyotrophic Lateral SclerosisClinical Signs and Symptoms
Weakness Hyporeflexia Pain and cramps Fasciculations Wasting
Spasticity Hyperreflexia Babinski’s sign Emotional Lability
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Amyotrophic Lateral SclerosisAtypical Features
Dementia - < 5 % Sensory loss – atypical 25% complain of paresthesias Oculomotor dysfunction Bowel or bladder dysfunction
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Amyotrophic Lateral Sclerosis
Diagnosis Two experienced Neurologists
Laboratory Studies
No study to prove or disproveLook for an alternate diagnosis
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Amyotrophic Lateral SclerosisLaboratory Studies
Nerve conduction studies • assess for demyelinating vs. axonal
involvement Electromyography
• confirm ALS• myopathy
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Amyotrophic Lateral SclerosisLaboratory Studies
MRI cervical spine • Cervical Spondylosis with cord compression• Herniated disc• Syrinx
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Amyotrophic Lateral SclerosisLaboratory Studies
ESR – inflammatory/malignancy SPEP – monoclonal gammopathy TSH – hyperthyroidism B12 – combined systems degeneration
Calcium/PTH - hyperparathyroidism
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Amyotrophic Lateral SclerosisPrognosis
Variable – difficult to predict in an individual patient
50% live 3-4 or more years 20% live 5 or more years 10% live 10 or more years Occasional patients live 20 years
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Amyotrophic Lateral SclerosisTreatment
Rilutek 2 large clinical trials
• Bulbar onset• Entire population
Endpoint • Death• Ventilator dependence
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Amyotrophic Lateral SclerosisTreatment
Bulbar onset • Prolonged survival • Improved muscle strength
Entire population• Prolonged survival• No effect on decline in muscle strength
Prolonged survival an average of 2-3 months
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Amyotrophic Lateral SclerosisRilutek 50 mg po bid
Hepatotoxicity• Serum transaminase levels• Check every month x 3• Then every 3 months x 3 for the first year
Adverse effects• Neutropenia• Nausea/vomiting
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Amyotrophic Lateral SclerosisRilutek 50 mg po bid
Reasons for not taking the drug• Expense• Minimal benefit• Unwillingness to take a medication that
would prolong life
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Amyotrophic Lateral SclerosisManagement
weakness fatigue nutrition dysphagia feeding tube dysarthria communication
spasticity cramps pain depression anxiety breathing end-of -life
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Amyotrophic Lateral SclerosisMultidisciplinary Approach to Care Neurologist Chiropractor Dietician Speech/swallowing
therapist Family/caregivers Psychologists
Physical therapist Occupational therapist Social worker GI physician Support organizations Homehealth/hospice Pulmonologist
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