What causes postpolio progressive muscular atrophy?

Updated: Feb 20, 2018
  • Author: Sridharan Ramaratnam, MD, MBBS; Chief Editor: Helmi L Lutsep, MD  more...
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Answer

Postpolio progressive muscular atrophy

  • New symptoms and signs can occur many decades after the acute illness in up to 40% of survivors of acute paralytic poliomyelitis. Symptoms and signs similar to PPMA have also been reported in individuals without preceding paralytic poliomyelitis (among those who have had nonparalytic polio). [1, 16, 17]

  • The muscle-related effects of postpolio syndrome are possibly associated with an ongoing process of denervation and reinnervation, reaching a point at which denervation is no longer compensated for by reinnervation. [18, 19]

  • Postulated pathogenetic mechanisms include persistent active poliovirus infection, superimposition of the normal aging process on a depleted motor neuron pool, inflammatory process, altered immunity, or increased vulnerability of poliovirus-damaged neural tissue to new infections. GM1 antibodies and serum insulinlike growth factor-1 are probably not involved in the pathogenesis of PPMA. [18, 20]

  • Proteomic analysis of the CSF of persons with PPMA displayed a disease-specific and highly predictive (P =0.0017) differential expression of 5 distinct proteins: gelsolin, hemopexin, peptidylglycine alpha-amidating mono-oxygenase, glutathione synthetase, and kallikrein 6, respectively, in comparison with the control groups. These 5 proteins require further evaluation as candidate biomarkers for the diagnosis and development of new therapies for PPMA patients. [21] Inflammatory markers such as serum TNF-alpha, IL-6, and leptin levels are abnormally increased in PPMA patients. [22]


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