Multiple Sclerosis: The History of a Disease

Abstract

'It would be nice if a physician from London, one of these days, were to gallop up hotspur, tether his horse to the gatepost and dash in waving a reprieve—the discovery of a cure!' Wilhelm Nero Pilate Barbellion, the pen name of Bruce Frederick Cummings, described his life with a chronic neurological illness in his Journal of a Disappointed Man. He died age 30 years, in 1919. Early features were recurrent numbness and weakness in the limbs, vertigo, depression, decreased sight in one eye, facial numbness and weakness in the right arm. Medications included arsenic and strychnine and homeopathic remedies. Sir Henry Head, the neurologist, asked suspiciously if he had ever been with women, and then ordered two months' complete rest in the country. He 'chased me around his consulting room with a drumstick tapping my tendons and cunningly working my reflexes'. The diagnosis was concealed from Cummings, who sought a military service examination to force a disqualification diagnosis of disseminated sclerosis. T Jock Murray is Professor of Medical Humanities and Director of the MS Program at Dalhousie University, Halifax, Canada. Multiple Sclerosis: The History of a Disease shows depth and breadth of understanding and a passion for the subject; moreover, the style of presentation makes it an easy and addictive read, enhanced by well annotated illustrations. In describing the history of multiple sclerosis Murray also provides a history of neurology and the associated philosophy of science. Possibly the earliest documentation of multiple sclerosis is the case of Lidwina the Virgin, who lived in Schiedam, Holland. In 1395, age 16 years, Lidwina developed an acute illness and subsequently fell while skating on a frozen canal. Later symptoms included blindness in one eye, weakness and pain. She died in 1433. After canonization she became the patron saint of both figure skating and sickness. While some commentators have considered there to be sufficient evidence for a diagnosis of multiple sclerosis, Murray in his gentle way points to elements of marked religiosity, mysticism, histrionic behaviour, and even self-mutilation. The features of multiple sclerosis were first well defined by Jean-Martin Charcot, neurologist at the Hopital de Salpetriere in 1868, as 'la sclerose en plaques'. In particular he made the distinction between the tremor of paralysis agitans (later called Parkinson's disease) and that of multiple sclerosis. The three most reliable indicators of multiple sclerosis—intention tremor, nystagmus, and scanning speech—became known as Charcot's triad. Dr Murray educates the reader painlessly on the aetiology and pathogenesis of multiple sclerosis while presenting the history of the disease. Thus we proceed through clinical description and classification, neuropathology, neurophysiology, immunology, and imaging, with a hint of genetics. The continuing critical theme is neuropathology. From the clinician's point of view the development of magnetic resonance imaging has been key to allowing more precise diagnosis as well as surrogate markers for clinical trials. Until very recently there has been no effective treatment, although a wide range of treatments have been used. It is surprising to see that the current use of steroids for acute relapses is quite recent (high-dose intravenous methylprednisolone replacing corticotropin in the early 1980s, following the pattern of usage by rheumatologists), with the first major controlled trial to demonstrate efficacy of the regimen published in 1987. Interferons, discovered in the 1950s, were initially promoted as a treatment for cancer. In 1977 Lawrence Jacobs of Buffalo, NY, was offered a returned supply of interferon (produced from the foreskin of recently circumcized infants). He was initially interested in using this for the rapidly progressive and fatal illness amyotrophic lateral sclerosis (motor neuron disease) but chose to study multiple sclerosis since there were more patients available. The Food and Drug Administration approved the first interferon for treatment of multiple sclerosis in 1993, with other interferons and copolymer following. The use of the interferons remains controversial, with the National Institute for Clinical Excellence in the UK being equivocal in its advice. Altogether this is a fascinating and monumental work, a pleasure to read. The contents should be accessible to a wide audience, and provide an excellent understanding of multiple sclerosis, the history of neurology, and the philosophy of science. In his final words Dr Murray offers a conclusion that may be a cliche but reflects the content of the work: 'For every breakthrough identified with an individual, there are her or his many colleagues, coworkers, staff and assistants— the person who developed the technological step that allowed the research to go forward, the statistician who showed that the work was relevant, the secretarial and administrative staff who kept the absent-minded professors free to pursue their scientific goals, and especially their colleagues, who provided a support system and added ideas and information that allowed them to go forward.' This is the nature and lesson of history.