Amyotrophic Lateral Sclerosis


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1 : Amyotrophic Lateral Sclerosis Jaffar Khan, MD Assistant Professor of Neurology Emory University
2 : Motor Neuron DiseaseTerminology Lower motor neuron Upper motor neuron    
3 : 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
4 : Amyotrophic Lateral SclerosisEpidemiology Etiology – unknown Average age of onset mid-50’s Mode of transmission Sporadic – 90-95% Familial – 5-10% (autosomal dominant)
5 : 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
6 : Amyotrophic Lateral SclerosisClinical Presentation Lower motor neuron signs Weakness, muscle wasting, hyporeflexia, muscle cramps, fasciculations Upper motor neuron signs Spasticity, hyperreflexia, weakness
7 : Amyotrophic Lateral SclerosisClinical Presentation Asymmetric Weakness – most common Onset single limb or bulbar Local spread then regional spread Bulbar, cervical, thoracic, lumbosacral Fasciculations
8 : 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
9 : Amyotrophic Lateral SclerosisClinical Signs and Symptoms Weakness Hyporeflexia Pain and cramps Fasciculations Wasting Spasticity Hyperreflexia Babinski’s sign Emotional Lability
10 : Amyotrophic Lateral SclerosisAtypical Features Dementia - < 5 % Sensory loss – atypical 25% complain of paresthesias Oculomotor dysfunction Bowel or bladder dysfunction
11 : Amyotrophic Lateral Sclerosis Diagnosis Two experienced Neurologists Laboratory Studies No study to prove or disprove Look for an alternate diagnosis
12 : Amyotrophic Lateral SclerosisLaboratory Studies Nerve conduction studies assess for demyelinating vs. axonal involvement Electromyography confirm ALS myopathy
13 : Amyotrophic Lateral SclerosisLaboratory Studies MRI cervical spine Cervical Spondylosis with cord compression Herniated disc Syrinx
14 : Amyotrophic Lateral SclerosisLaboratory Studies ESR – inflammatory/malignancy SPEP – monoclonal gammopathy TSH – hyperthyroidism B12 – combined systems degeneration Calcium/PTH - hyperparathyroidism
15 : 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
16 : Amyotrophic Lateral SclerosisTreatment Rilutek 2 large clinical trials Bulbar onset Entire population Endpoint Death Ventilator dependence
17 : 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
18 : 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
19 : Amyotrop

 

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Added On : 6 Years ago.
Assistant Professor of Neurology. Emory University. Motor Neuron DiseaseNeurologist; Clinical/resea    more
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