The Post Polio Syndrome clinical research program performs integrated multidisciplinary studies that will explore the cause and mechanisms resulting in degeneration of motor neurons and dysfunction of the neuromuscular units. This has raised fundamental issues regarding the behavior of polio viruses and all the enteroviruses, the mechanisms of viral persistence and immune and physiological factors involved. PPS may be a multifactorial disease and the rational approach is with multidisciplinary studies, with the following interrelated projects and cores. Clinical core recruits, diagnoses, and follows patients serially with neuromuscular examinations that generate quantitative scores of disease activity and progression which can then be interrelated with the research projects and database. The PPS Clinic coordinates and supervises patient management. The immunology project will study cerebrospinal fluid and cells, peripheral blood and cells, and the CNS for immune or autoimmune mechanisms (humoral or cell mediated). The findings will complement the immunocytopathic studies of the neuropathology project. The neuropathology project explores in-depth inflammatory and vasculitis phenomena that have been reported in spinal cord and muscle specimens of PPS patient. Morphometric and ultramicroscopic studies of spinal cord and myoneural junction will establish diagnostic features of PPS. Neurovirology project seeks enteroviral sequences in spinal cord, muscles, CSF and peripheral blood lymphocytes using techniques of dot blot, northern blot, in situ DNA hybridization and polymerase chain reaction for gene amplification. The animal studies project will study motor unit physiology following partial denervation that mimics motor unit changes in PPS. Possible risk factors will be studied in relation to PPS and also possible immune mechanisms following neural tissue damage.

The investigators have long experience in the clinical and research aspects of motor neuron disease (MND) and intend to gain meaningful knowledge that will lead to prevention, management, and specific therapy of PPS. The information developed will be of wide interest since there are similarities of PPS to other catastrophic MND, such as amyotrophic lateral sclerosis. Representative