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RECENT ADVANCES IN TREATING POST-POLIO SYNDROME

RECENT ADVANCES IN TREATING POST-POLIO SYNDROME. Susan L. Perlman, M.D. Associate Professor of Neurology UCLA Medical Center. CURRENT LITERATURE 32 CITATIONS SINCE 5/00. CAUSES OF POST-POLIO SYNDROME FATIGUE BRAINSTEM NEURONAL INJURY ANIMAL MODELS DRUG TRIALS STEM CELL RESEARCH

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RECENT ADVANCES IN TREATING POST-POLIO SYNDROME

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  1. RECENT ADVANCES IN TREATING POST-POLIO SYNDROME Susan L. Perlman, M.D. Associate Professor of Neurology UCLA Medical Center

  2. CURRENT LITERATURE32 CITATIONS SINCE 5/00 • CAUSES OF POST-POLIO SYNDROME • FATIGUE • BRAINSTEM NEURONAL INJURY • ANIMAL MODELS • DRUG TRIALS • STEM CELL RESEARCH • HOW ARE PEOPLE DOING

  3. WHAT PPMA IS AND IS NOT (I) • It has not yet been shown to have genetic risk factors (Bartholdi et al., SMA gene nl. Neuromuscular Disorders 10:99, 2000) • Similar delayed progressive decline seen in survivors of other conditions (myelopathy) that injured anterior horn cells (metabolic overload of enlarged or fragile motor units) (Narayanaswami et al., J Neurol Sci184:11,2001)

  4. WHAT PPMA IS AND IS NOT (II) • It is not just normal aging of the motor units. Taiwanese polio survivors are 10-20 years younger than Western patients at onset of post-polio symptoms (Chang et al., Spinal Cord39:526, 2001) • There were no publications addressing the role of the immune system or the effects of other infections or medical conditions.

  5. WHAT PPMA IS AND IS NOT (III) • What about persistant polio virus? Dalakas feels that leftover pieces of the old virus may still be present in the nervous system, causing an immune reaction, but not ongoing infection. Girard, et al., have shown in mice that viral RNA does not replicate and infect, but may persist in an altered form (J Gen Virol83:1087, 2002)

  6. FATIGUE (I) • While not the hallmark of PPMA, is by far the most common symptom (>80%). • Klein, et al., studied 120 patients and reported decreasing strength, at a rate higher than normal aging and in upper extremities and flexors of the leg (repetitive stepping muscles, not weight-bearing) (Arch Phys Med Rehabil81:1059, 2000)

  7. FATIGUE (II) • Sunnerhagen, et al., discuss several types of fatigue in PPMA (Acta Physiol Scand171:335, 2001) central fatigue emotional fatigue fatigue from deconditioning augmented peripheral fatigue (enlarged muscle fibers that activate more slowly, contract less well, or recover abnormally)

  8. BRAINSTEM NEURONAL INJURY (the basis of much of Bruno’s work) • Bruno (Am J Phys Med Rehabil79:4, 2000) non-paralytic polio did cause neuronal lesions and these survivors are at risk for PPMA (14-42%) • Bruno, et al., also discussed word-finding difficulties in 79% of survivors with fatigue, documented by psychological testing and associated with decreased brain dopamine (ibid., 79:343, 2000)

  9. NEW TECHNOLOGIES • Trojan, et al., at the national neurology meetings in April 2002, presented the use of magnetic resonance spectroscopy to assess neuronal number and metabolism in fatigued polio survivors. Neuronal loss and dysfunction was found in the brainstem (reticular activating system), but not the temporal lobes, discounting a generalized brain problem as a cause of PP fatigue.

  10. ANIMAL MODELS • Efforts have been ongoing to develop PPS animal models that can be used to study disease progression and treatment response. • Rats with partially injured/enlarged motor units were seen to lose axon terminal connections with time and with repetitive exercise (Tam et al., Muscle & Nerve25:359, 2002)

  11. DEVELOPING DRUG TRIALS (I) • Nollet, et al., at the national neurology meetings in April 2002, presented another randomized, double-blind, placebo-controlled study of pyridostigmine (Mestinon 60mg four times per day) in 67 patients with abnormal single-fiber EMG studies. Results were assessed at 5 and 14 weeks.

  12. Subjective fatigue, isometric strength, and EMG did not improve. • Timed walking performance improved about 5-6% on Mestinon, mainly in patients with normal sized motor units (“wiring was there, but connections were faulty”). • Confirms the prior report by Trojan (Neur53:1225, 1999)--helpful, but not compelling

  13. DEVELOPING DRUG TRIALS (II) • Trojan, et al., in a study of 112 survivors found insulin-like growth factor levels did not correlate with strength and may not be a cause of fatigue in PPMA (J Neurol Sci 182:107, 2001) • MRS can detect levels of lipids, creatine, and carnitine in muscle fibers and may be useful in trials of new drugs or rehab and in studies of causes of PPMA (Jagannathan et al., Magn Reson Imaging20:113, 2002)

  14. HOW ARE PEOPLE DOING? • More publications indicate that polio survivors are best served in multidisciplinary clinics staffed by knowledgeable professionals. • Polio survivors report poorer functional status and health-related quality of life. • The life-altering effects of PPMA have not been adequately addressed by health care providers.

  15. EDUCATIONAL IMPERATIVE • The March of Dimes has issued a report for physicians that outlines the best practices in diagnosis and care of post-polio syndrome. • This report has been publicized by the American Medical News and has been reprinted in the Medical Board of California ACTION REPORT, which is mailed to all physicians licensed in California.

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