Myoclonus epilepsy with ragged-red fibers
Jun. 10, 2021
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Poliovirus is an acute enteroviral infection that is spread from person to person, primarily via the fecal-hand-oral routes. Although most patients who acquire the infection are asymptomatic, those afflicted may develop a variety of neurologic manifestations, including aseptic meningitis, polioencephalitis, bulbar poliomyelitis, and paralytic poliomyelitis. In paralytic poliomyelitis, muscle weakness is preceded by intense myalgias of the involved limbs and axial skeleton. Following recovery, as many as 20% to 30% of individuals who develop paralytic poliomyelitis may suffer from post-polio syndrome, which produces muscle weakness, pain, atrophy, and fatigue many years after acute illness.
There has been a large worldwide effort for poliomyelitis eradication. Polio cases have decreased by over 99% since 1988, from an estimated 350,000 cases in more than 125 endemic countries to 42 reported cases in 2016 (37 wild-type and 5 vaccine-derived) (32; 38; 82; 83; 88; 40; 53). Poliomyelitis due to wild type virus has now been eliminated from the Americas, Europe, and Western Pacific, and cases in Africa and Asia have markedly decreased. Although great strides have been made, poliomyelitis remains and is rising in regions, including Nigeria, Afghanistan, and Pakistan, and there is a risk for new outbreaks to occur.
• Poliovirus is an acute enteroviral infection that is spread from person to person, primarily via the fecal-hand-oral route.
• Neurologic manifestations of poliomyelitis include aseptic meningitis, polioencephalitis, bulbar poliomyelitis, and paralytic poliomyelitis.
• As many as 20% to 30% of individuals who develop paralytic poliomyelitis may suffer from post-polio syndrome, which includes new muscle weakness, pain, atrophy, and fatigue many years after the acute illness.
• There has been a large worldwide effort for poliomyelitis eradication, with a decrease of polio cases by over 99% since 1988. Though great strides have been made, there remains a high risk for new outbreaks.
• On September 20, 2015, the Global Commission for the Certification of Poliomyelitis Eradication declared that poliovirus type 2 has been eradicated worldwide.
Poliomyelitis has afflicted humans for centuries. The first recognized clinical description was by English physician and surgeon Michael Underwood (1736-1820) in the second edition of A Treatise on Diseases of Children, which was published in 1789 (112; 75; 97). Major contributions to the understanding of the disease were made by the German orthopedist Jacob Heine (1800-1879), who described the clinical features of acute poliomyelitis (47).
In 1870, French neurologist Jean-Martin Charcot (1825-1893) and his junior colleague Alix Joffroy (1844-1908) recognized that the flaccid paralysis of poliomyelitis was caused by spinal anterior horn cell damage (23).
In the mid- to late 19th century, the general belief was that polio was not infectious; in the late 19th century, when bacteriological explanations were in vogue for almost all conditions, polio was suspected to be another bacterial disease. Numerous attempts were made to identify the responsible microorganism, with many claims to have successfully done so, but none were reproducible.
Finally, in 1908, Austrian pathologist Karl Landsteiner (1868-1943) and his colleague Erwin Popper (1879-1955) showed that the etiological agent of poliomyelitis was a filterable virus (64). The existence of more than one type of poliovirus was first inferred by Australian virologist Frank Macfarlane Burnet (1899-1984) and his collaborator Dame Annie Jean Macnamara (1899-1968) in 1931, when they demonstrated that monkeys who had recovered from infection with a strain recovered in Melbourne subsequently developed disease when given a virulent mixed virus strain (18; 30).
In 1949, the 3 antigenic poliovirus types (poliovirus 1, poliovirus 2, poliovirus 3) were identified (13; 62).
The first recorded outbreaks of poliomyelitis occurred in the mid- and late nineteenth century in northern Europe and North America, followed by much larger epidemics in the early 20th century.
In August 1921, while vacationing with his family at their summer home on Campobello Island off the coast of Maine, politician Franklin Delano Roosevelt (FDR; 1882-1945) became ill, lost motor power in his legs, and was diagnosed with polio. Roosevelt later sought treatment at a resort in Warm Springs, Georgia. Because of his self-perceived improvement at the resort, in 1927, Roosevelt and his friend, American lawyer Basil O’Connor, created the Georgia Warm Springs Foundation, in which O'Connor served first as treasurer and later as president.
The foundation was subsequently reconstituted as the National Foundation for Infantile Paralysis in 1938. A solicitation prior to Roosevelt’s birthday in 1938 resulted in a huge influx of small donations that swamped the White House mail room.
As a result, radio star Eddie Cantor dubbed it the “March of Dimes” -- a play on the contemporary radio and newsreel series, The March of Time. The March of Dimes ultimately became the official name of the organization, which served as the largest source of funding for research and clinical care for poliomyelitis at the time. Indeed, the organization supported the work of Jonas Salk and others that led to the development and testing of polio vaccines.
Prototype “iron lung” negative-pressure ventilators were developed in the late 1920s and early 1930s by industrial hygienist Philip A Drinker (1894-1972), physiology instructor Louis Agassiz Shaw Jr. (1886-1940), and inventor/medical-equipment manufacturer John Haven (“Jack”) Emerson (1908-1997). From around 1926 to 1928, Drinker and Shaw, both at Harvard Medical School in Boston, designed an electrically powered tank respirator. On October 13, 1928, Drinker and pediatrician Charles F McKhann demonstrated the potential of this device in an 8-year-old girl with poliomyelitis, respiratory failure, and coma who was treated at Boston Children’s Hospital and briefly survived before succumbing to pneumonia. A second trial on Friday, September 13, 1929, at Peter Bent Brigham Hospital in Boston, on a 21-year-old Harvard student, was unquestionably successful: Hoyt was weaned from the respirator in 4 weeks and was discharged from the hospital before Christmas. Emerson built a mechanically superior device in the summer of 1931; Emerson’s device was first used clinically to save the life of a priest with polio at the Providence City Hospital in Providence, Rhode Island.
After this, the manufacture of "iron lungs" expanded markedly over the next 2 decades. At their peak use in the early 1950s, wards at some referral centers were crowded with dozens of these devices, all in use for affected patients, with a large compliment of attendant nursing and respiratory therapy staff.
Indeed, the iron lung required intensive nursing care and respiratory therapy and a supporting hospital infrastructure.
From the 1920s through at least the early 1940s, the orthodox treatment for poliomyelitis consisted largely of absolute and prolonged immobilization of affected limbs with splints or plaster casts, followed by often permanent orthopedic braces; in retrospect, this approach was less than ideal, and in many ways counterproductive as it caused or contributed to disuse atrophy, joint contractures, and lifelong disability. Beginning around 1911, in a sparsely populated area of Australia, “Sister” Elizabeth Kenny (1880-1952) developed an empiric approach to rehabilitation following poliomyelitis that combined physical and psychological techniques.
The physical methods she employed included the labor-intensive application of moist warm wraps for muscle spasms, passive range of motion, and massage. Kenny’s physical methods were combined with early mobilization, strong encouragement to achieve both functional independence and a prompt return to normal activities, and confident optimism for improvement. The Kenny methods were widely adopted in the United States and elsewhere in the 1940s (although not in Australia where she was strongly opposed by physicians and, particularly, orthopedic surgeons). Kenny’s approach represented a significant advance in the care of paralyzed patients and helped foster the growth of physical therapy and the medical discipline of physical medicine and rehabilitation.
It was not until the development of the first successful inactivated poliovirus vaccine in the 1950s by American virologist Jonas Salk that the severity of polio epidemics started to decrease.
In 1954 and 1955, Salk’s inactivated poliovirus vaccine was successfully tested in a monumental controlled trial involving more than 1.8 million United States schoolchildren, all funded by the National Foundation for Infantile Paralysis (68).
Polish-American virologist Albert Sabin (1906-1993) developed the first effective trivalent live-attenuated (oral) poliovirus vaccine.
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