tailieunhanh - Manual of neurologic therapeutics - part 4

3. No evidence of conduction block or other features of primary demyelination. 4. EMG demonstrates evidence of active denervation in the form of fibrillation potentials and positive sharp waves as noted above. The earliest abnormality is fasciculation potentials due to motor unit hyperexcitability/instability that occur prior to motor unit degeneration. TREATMENT 1. Riluzole: a. Two controlled trials have demonstrated that riluzole 50 mg . . extends tracheostomy-free survival by 2 to 3 months. Unfortunately, the studies did not find that riluzole improves strength or the quality of life. b. Riluzole is thought to act by inhibiting the release of glutamate at presynaptic. | 3. No evidence of conduction block or other features of primary demyelination. 4. EMG demonstrates evidence of active denervation in the form of fibrillation potentials and positive sharp waves as noted above. The earliest abnormality is fasciculation potentials due to motor unit hyperexcitability instability that occur prior to motor unit degeneration. TREATMENT 1. Riluzole a. Two controlled trials have demonstrated that riluzole 50 mg . . extends tracheostomy-free survival by 2 to 3 months. Unfortunately the studies did not find that riluzole improves strength or the quality of life. b. Riluzole is thought to act by inhibiting the release of glutamate at presynaptic terminals. c. Side effects include nausea abdominal discomfort and hepatotoxicity. d. Check hepatic function tests every month for 3 months and then every 3 months while on riluzole. Hepatotoxicity is reversible once riluzole is discontinued. 2. Supportive care a. Despite that the lack of effective therapy to halt or reverse the progression of the disease there are many therapeutic measures that improve the quality of life in patients with ALS. b. A multimodality approach in treating patients with ALS is essential. c. Patients are seen in clinic at least every 3 months in conjunction with physical occupational speech and respiratory therapy. d. They are also evaluated by psychiatry gastroenterology pulmonary medicine and social workers as necessary. 3. Physical therapy a. Stretching exercises passive and active to prevent contractures. b. Assess gait and needs . cane walker wheelchair . 4. Occupational therapy a. Patients should be evaluated for adaptive devices . ball-bearing feeders that may improve function. b. The patient s home should be evaluated for equipment needs. 5. Dysarthria a. Patients should be evaluated by a speech therapist. b. Techniques may be given to help patient with articulation. c. Patients may benefit from various speech augmentation devices and switch- or .