History

In 1967, a disease outbreak occurred in a laboratory in Marburg, Germany, where the kidneys of cercopithecoid (Green African; vervet) monkeys were being taken out for preparation of cell cultures. Twenty-seven laboratory workers (including a worker in Yugoslavia) fell ill with a grave illness, and seven died. There were four secondary cases in total (including the wife of an infected laboratory worker in Yugoslavia secondarily infected by sexual intercourse), but none fatal. Early suspicions focused on yellow fever, but this was soon ruled out (Casals 1971). In due course, a virus was isolated and found to be quite distinct from any other known viruses. Electron micrographs revealed a virus with bizarre morphology of a type never seen before (Peters, Muller, and Slenckza 1971). Pictures taken resembled photographs of a bowl of spaghetti. The agent was named Marburg virus and the disease Marburg disease. Strict monkey quarantines were initiated. No further cases were seen in laboratory workers.

An intensive and extensive series of field studies were initiated in East Africa, which had been the monkeys’ homeland (Henderson et al. 1971; Hennessen 1971; Kalter 1971). No virus recoveries were made from any of the monkeys examined. In later years, serologic studies involving humans and primates, and also rodents, have been carried out in many regions, as can be seen in Table VIII.86.1.

The first Marburg cases seen in Africa occurred in February of 1975. A young Australian couple touring in Rhodesia (Zimbabwe) became ill by the time they got to South Africa. They were admitted to a major hospital in Johannesburg where the young man died and the young lady recovered. A nurse tending them also sickened and recovered.

Scrofula can be denned only historically. That is, scrofula is a term about which there was some measure of consensus in the past, but one that has now been largely superseded by terms that indicate some form of tuberculosis. It must be emphasized, however, that scrofula is not simply an old name for what we call tuberculosis. Our ontology of disease centers on the tubercle bacillus, and we would commit a grave historical error if we assume that with its aid we can know what was actually there in old discussions of scrofula. To understand these old discussions, we need to know how and why the old picture of scrofula was put together. The distribution of scrofula, as we shall see below, has much more to do with the religious and political convictions of those who saw it than with physical geography or economic conditions or other circumstances normally considered conducive to diseases. Likewise in regard to its clinical manifestations, we may note, first, that this term itself implies an underlying entity that becomes manifest. But, second, scrofula, historically, was its collection of symptoms and signs. What we need to understand is what went into that collection, and why.

History and Geography

“Scrophula,” like “scurvy” and “syphilis,” is not a term that was used by the ancients. Whereas there may be special reasons why the latter two were unknown (a distribution to the north of the ancient Mediterranean and a possible Columbian origin, respectively), there seems to be no reason to suspect that scrofula was a new disease.

Osteoarthritis (OA) is the most common rheumatic disorder afflicting humankind and vertebrates in general. The most common alternative terms, osteoarthrosis and degenerative joint disease, are used because of divergent concepts of the nature and cause of the disorder. One school maintains that OA is a family of systemic inflammatory disorders with similar clinical and pathological end results. Another supports the use of the term “osteoarthrosis” because inflammation is not present. Still another uses the term “degenerative joint disease” because it is held that aging and “wear and tear” are responsible for its occurrence.

William Heberden, an eighteenth-century English physician, gained immortality by describing what we now term Heberden’s nodes, a common heritable form of osteoarthritis, especially common in women. In his Commentaries, he writes:

“What are those little hard knots, about the size of a small pea, which are frequently seen upon the fingers, particularly a little below the top, near the joint? They have no connection with the gout, being found in persons who never had it: They continue for life; and being hardly ever attended with pain, or disposed to become sores, are rather unsightly, than inconvenient, though they must be some little hindrance to the free use of the fingers”. (Heberden 1802)

Modern research provides new data for a comprehensive definition encompassing clinical, biochemical, and anatomic features (Denko 1989). OA is a multifactorial systemic inflammatory disorder with clinical symptoms of pain and stiffness in movable joints, showing radiographic evidence of cartilage loss and bony overgrowth. overgrowth. The anatomic changes – cartilage loss and a kind of bony overgrowth and spurs – may occur physiologically without clinical symptoms.

Dengue is an acute febrile disease caused by infection with a group B arbovirus of four serotypes, transmitted by the bite of infected Aedes aegypti and Aedes albopictus mosquitoes. Endemic throughout the tropics and subtropics, uncomplicated dengue is rarely fatal, although return to normal health after an attack may take several weeks. It does not always have a benign course, however, and can be complicated by hemorrhagic manifestations (hemorrhagic dengue) and circulatory collapse (dengue shock syndrome) with a potentially fatal outcome unless facilities are available for the urgent medical treatment of those affected.

Typical uncomplicated dengue has an incubation period of 3 to 15 days and is characterized by abrupt onset of chills, headache, lumbar backache, and severe prostration. Body temperature rises rapidly, perhaps reaching as high as 40°C; bradycardia (slow heart rate) and hypotension (low blood pressure) accompany the high fever. Conjunctival injection, lymph node enlargement, and a pale, pink rash, especially noticeable on the face, are usually present during this first phase of the disease. In classical dengue, the fever lasts for 48 to 96 hours initially, subsides for 24 hours or so, and then returns (saddleback fever), although the peak of temperature is usually lower in the second phase than in the first. A characteristic red rash appears in the second phase, usually covering the trunk and extremities, but sparing the face. The fever, rash, and headache, together with the other pains, are known as the dengue triad. The acute illness ends in 8 to 10 days, and one attack confers immunity to the particular dengue subtype.

Pre-Columbian Peoples

The natural environment of the continent of South America is overwhelmingly diverse and thus has posed special problems of physiological adaptation to its indigenous populations, as well as later to European, African, and Asian intruders. Indeed, because of the harsh environments of much of the continent, there are but few places in which people can flourish without great effort and skillful labor. In much of the continent’s vast interior, even communication and transportation would be impossible without the river systems of the Amazon and the Paraná– Paraguay along with the smaller rivers of Colombia and Venezuela, the Magdalena and the Orinoco, and the Sāo Francisco of northeastern Brazil.

One of the most formidable environments is that of the Andes Mountains, which range from western Venezuela to the tip of the continent, with snow-capped peaks at more than 20,000 feet in altitude and with populations perched at 10,000 and 13,000 feet. At such altitudes, scarcity of oxygen has led to physiological adaptations in the bodies of the indigenous peoples of Peru and Bolivia that permit them to perform hard physical labor in the thin air.

The Andes break the westerly movement of rainfall from the Amazon basin, and rain falls in profusion on the eastern slopes, where lush tropical forests shelter the people of the Upper Amazon from outside invaders. On the opposite side of the Andes, a lack of rainfall creates the semiarid coastal lowlands intersected by small rivers flowing through the desert to the ocean.

Rocky Mountain spotted fever is a severe, acute, rickettsial disease transmitted by ticks and limited to the Western Hemisphere. Its major symptoms are similar to those of epidemic typhus, but its rash covers the entire body, including the face, the palms of the hands, and the soles of the feet. Between 20 and 25 percent of untreated victims die, making Rocky Mountain spotted fever the most severe rickettsial infection in the Americas. First identified in the Rocky Mountain region of the United States, this place name has never been dislodged, even though it is inaccurate and even misleading.

Etiology and Epidemiology

The severity with which Rocky Mountain spotted fever treats its victims underscores its natural existence as an infection of ticks and their mammalian hosts. The microbial cause of the disease, Rickettsia rickettsii, normally inhabits ixodid, or hard shell, ticks, apparently causing little harm to the host. Although small mammals are susceptible to a mild infection with R. rickettsii an may transmit it to uninfected ticks, the principal means by which the organism is maintained in nature is from one generation to the next in the eggs of the female tick.

The epidemiology of Rocky Mountain spotted fever is linked to areas favorable for the habitation of the vector ticks. The Rocky Mountain wood tick, Dermacentor andersoni, and the American dog tick, Dermacentor variabilis, are the most common vectors in the United States, although the Lone Star tick, Amblyomma americanum, also transmits the disease in the south central and southeastern parts of the United States.

The nature and role of public health are constantly changing, and its definition has been a major preoccupation of public health leaders in the twentieth century. Essentially, public health is and always has been community action undertaken to avoid disease and other threats to the health and welfare of individuals and the community at large. The precise form that this action takes depends on what the community perceives as dangers to health, the structure of government, the existing medical knowledge, and a variety of social and cultural factors. From the beginning, communities, consciously or not, have recognized a correlation between filth and sickness, and a measure of personal and community hygiene characterized even the earliest societies.

By the eighteenth century, personal and community hygiene were becoming institutionalized. A wide variety of local regulations governed the food markets, the baking of bread, the slaughtering of animals, and the sale of meat and fish. These regulations were motivated by a concern for the poor, a desire for food of a reasonable quality, and commercial considerations. Bread was always a staple of the poor, and regulations in the Western world invariably set the weight, price, and quality of loaves. For economic reasons, merchants shipping food abroad promoted regulations on meat and grains in order to protect their markets and save themselves from dishonest competition.

This disease is a pathological condition resulting from infection with the parasite Dracunculus medinensis. In most instances, the adult worms, which are about 1 meter long, are quite evident as they emerge slowly through the skin of their victims.

Distribution and Incidence

In the 1980s and 1990s, Dracunculiasis is found mainly in India, in Pakistan, and in a band of 19 African countries between the Sahara Desert and the equator, from Senegal in the west to Ethiopia in the east (see Map VIII.38.1). Formerly this disease was much more widespread in the Middle East and Africa, and it occurred for some years in the Americas after it was introduced there by infected Africans during the slave trade.

In general, the incidence of dracunculiasis is significantly higher in endemic rural Africa communities than in endemic Asian villages. In West Africa especially, for example, rates of infection in affected areas often reach 20 to 40 percent, and sometimes exceed 50 percent, whereas in Asia, the rates usually are below 20 percent. In rural areas, the disease occurs sporadically, with adjacent villages sometimes differing greatly in the percentage of those infected. Susan Watts (1987), a medical geographer, has estimated that the number of persons at risk of this infection in Africa is about 120 million, with Map VIII.38.1. Areas in which dracunculiasis is reported or probably exists. another 20 million at risk in India and Pakistan, based on the assumption that everyone is at risk ho is living in a rural district where a minimum of one case of dracunculiasis has occurred.

The semantic and logical quagmires that await anyone audacious enough to safari through the changing concepts of disease, illness, and health are portended by a cursory analysis of the definition formulated by the World Health Organization. “Health,” we are informed, “is a state of complete physical, mental and social well-being and not merely the absence of disease or infirmity” (Caplan, Engelhardt, and McCartney 1981). Aside from the fact that this seems more realistic for a bovine than a human state of existence, problems abound in what appears to be a fairly straightforward statement. The word “complete” immediately removes the definition from the realm of human reality. What is complete mental well-being, or physical for that matter? Worse still, the phrase “complete social wellbeing” is so freighted with individual interpretations that it alone renders the definition useless, if not pernicious.

This essay concentrates on ideas of physical health and disease, which is not to minimize the importance of psychiatric disease, but rather to admit that concepts of mental health and illness, although sharing most of the definitional difficulties of physical health and disease, are even more difficult to handle. In large part this is because with mental illness we lack the kinds of objective tools to measure brain function that have helped, though not resolved, questions of what constitutes health and disease in the physical realm. This is not, however, to deny the interconnectedness of the psychic and the physical, which is assumed in all of what follows.

Perhaps no one sentence captures the history of changing notions about disease better than a paraphrase of Humpty Dumpty’s haughty admonition: “When I use the word disease, it means just what I choose it to mean – neither more nor less.” mean - neither more nor less. A number of important considerations lead to this generalization. Among these are the following: (1) The definition of disease has varied with time and place in history; (2) the names assigned to diseases are ultimately abstractions, although it is useful at times to act as though they are real; (3) what we mean by diagnostic terms, as with words in general, can be discerned more accurately by what we do with them than what we say about them.

Ergotism is a disease condition acquired by eating cereal grains infected with ergot fungus. Known since the time of Galen, it was prevalent in medieval Europe, particularly among the poor who, during famine, consumed bread made from spoiled rye. Ergot (secale cornutum, spur of the corn, horned rye, womb grain), the dried sclerotium of Claviceps purpurea, develops on the ovary of common rye, or on corn, where it was previously known as corn smut. The actual cause of ergot in grasses was hotly debated by early naturalists, some of whom thought it occurred in rainy weather and was attributable to fog or impure atmosphere. Others believed it to be the work of worms or butterflies, whereas still others regarded it as the product of improper fecundation or perhaps the cooking of the sexual parts of the plants.

Classification

Ergotism has two forms: (1) convulsive, or spasmodic, also known as creeping, which affects the central nervous system; and (2) gangrenous, which affects the blood vessels and blood supply to the extremities. Common names for the gangrenous form are St. Anthony’s fire (after the patron saint of the disease), hidden fire, saint’s fire, evil fire, devil’s fire, and holy fire. As a result of early imprecision in disease specificity and diagnosis, physicians confused ergotism with the plague and a variety of other diseases including leprosy, anthrax, typhus, smallpox, and scurvy.

Malaria is the disease resulting from infection by one or more of four species of protozoan parasites of the genus Plasmodium. These parasites are normally transmitted from one human host to the next by the bite of an infected female mosquito of the genus Anopheles. Although malaria has receded from many temperate regions in this century, the disease continues to be a major cause of morbidity and mortality in many tropical and subtropical countries. Three of the species – Plasmodium viuax, Plasmodium falciparum, and Plasmodium malariae – are widely distributed; the fourth, Plasmodium ovale, is principally a parasite of tropical Africa. P. vivax (the agent of benign tertian malaria) and P. falciparum (causing malignant tertian malaria) are responsible for the great majority of cases and deaths attributed to malaria throughout the world.

Malaria is characteristically paroxysmal, and often periodic. The classical clinical episode begins with chills, extends through a bout of fever, and ends with sweating, subsiding fever, a sense of relief, and, often, sleep. Between the early paroxysms the infected person may feel quite well; as the disease progresses, however, the patient may be increasingly burdened by symptoms, even in the periods between febrile paroxysms. Although infection by any species may have serious, even fatal, consequences, P. falciparum infection is particularly dangerous because of complications associated with this parasite.

The term malaria, from the Italian mala and aria (“bad air”), was certainly in use in Italy by the seventeenth century to refer to the cause of intermittent fevers thought to result from exposure to marsh air or miasma. Horace Walpole wrote home from Italy in 1740 about “[a] horrid thing called mal’aria, that comes to Rome every summer and kills one.”

There are good reasons for believing that diseases and complaints of various kinds and degrees of severity were as much a part of everyday life in classical antiquity as were the assorted battle wounds and injuries so dramatically portrayed from Homer onward. This is indicated not only by the surviving Greek and Latin medical texts and the fragments preserved in Greco-Egyptian papyri, but also by the large corpus of nonmedical Greek and Latin texts, some of which are still being read today. In poetry, tragedy, and comedy, in history and annals, in philosophy and theology, as well as in botanical, agricultural, and pharmacological texts, illness and health and life and death constitute distinctive motifs.

To be certain, the evidence, both written and nonwritten, has survived in different states of preservation. It permits us, nonetheless, to reconstruct in part the intellectual and technological achievements of our past. Large gaps, however, exist in our knowledge of that past, and the absence of crucial details has led to hypotheses and inferences that cannot be tested directly.

Our knowledge of the diseases of classical antiquity stands somewhere between demonstrative certainty and complete ignorance. There is, after all, a sizable body of Greek and Latin medical texts, and it, in turn, has generated an even larger body of secondary literature. But for all that, our knowledge of the diseases of classical antiquity is far from complete. There are several reasons for its incompleteness, but it is important to keep in mind the enormous differences between the conceptual bases of the modern medical sciences and those of antiquity.

Only because of a great movement in China that has been going on for about 70 years have we been able to review the records of diseases in ancient China and publish them in a Western language. This movement has been closely allied with a revaluation of the practice of traditional Chinese medicine by those who have taken a special training in it. Many valuable works have been written in Chinese on the history of Chinese medical art and science. So far, however, all this material has remained practically unassimilated by sinologists and other Western students of Chinese culture. Thus, for example, most of the dictionary definitions in common use are quite out of date. Among the works that we have used in preparing the present contribution is the brilliant monograph of Yü Yün-hsiu on ancient nosology, or what might be called pathognostics – the recognition and classification of individual disease entities. Western historians of medicine should be aware that the treatise of Wu Lien-te and Wang Chi-min (K. C. Wong and Wu Lien-teh 1932) on Chinese medicine (nearly always the only one they know) may be described as the very small exposed piece of an iceberg, 90 percent of which is “below the surface” (i.e., in the Chinese language and therefore inaccessible to most historians of medicine). Since about the mid-1950s, the study of Chinese medicine has been revitalized; a great number of rare medical books from ancient and medieval times have been republished in photographic form, and some ancient texts have been reproduced in the modern colloquial (pai-hua) style, “translated” as it were from the ancient (ku-wen) style, either abridged or complete.

Parkinson’s disease, or parkinsonism, is a syndrome (i.e., a constellation of clinical signs and symptoms) consisting of four cardinal features: resting tremor, bradykinesia (physical and mental sluggishness), rigidity, and impaired postural reflexes. The diagnosis is made on the basis of finding any three of the four cardinal features.

Distribution and Incidence

This disease occurs throughout the world, with no population protected against the condition. Most surveys have investigated Caucasian populations of northern European or of Anglo-Saxon descent, and few studies have been done on the occurrence of Parkinson’s disease in other populations. In Caucasians the prevalence is 84 to 187 per 100,000 of population, with no geographic patterns and no clusters of increased incidence. Two studies seem to indicate a lower prevalence in blacks; this has been the clinical experience as well, probably indicating a decreased risk of Parkinson’s disease for blacks. The annual incidence varies from 5 to 24 per 100,000 of the white population. These figures, of course, depend on the methods of ascertainment, the population studied, the length of time that data have been collected, and many other factors. If the prevalence is divided by the annual incidence, the average duration of the illness is approximately 10 years.

Epidemiology

Parkinson’s disease usually occurs in late middle life or beyond. The mean age of onset is 58 to 62. Onset before age 30 is rare but is not unknown, and there is a juvenile form of Parkinson’s disease. The greatest incidence is in the decade age 70 to 79 years, with an incidence of 1 to 2 per 1,000 population per year.

Pinta (meaning “spotted”) is also called mal de pinto and carate. It is the least destructive of the treponematoses that are pathogenic for humans. Although the taxonomy of these treponemes is by no means resolved, pinta is sufficiently distinctive to argue for a separate causal species, Treponema carateum. As a specific treponemal variety, it was not described until 1938. The disease is chronic, predominantly affects the skin, and is now found only among isolated rural groups in Central and South America and Mexico, where it is endemic. Local names for the illness are tiña, empeines, and vitiligo.

Distribution and Incidence

According to one historian of treponemal diseases, pinta may have had a considerable world distribution at the end of the Paleolithic period, some 10,000 years ago. However, its past geographic distribution is in some doubt, and an alternative view suggests that it may have evolved purely in Amerindian communities of the New World, as a final level of micro-evolutionary change in the treponematoses there. Because it is not a very destructive condition, and may remain untreated in many individuals of the Third World countries of Latin America, it has been estimated that as many as a million individuals may have the disease.

Epidemiology and Etiology

Pinta is caused by T. carateum, which cannot be distinguished from Treponema pallidum (the causative agent of endemic and venereal syphilis). These treponemes are found mainly in the lower Malpighian layers of the epidermis, and may be present for years before the skin lesions eventually become inactive and depigmented. Large areas may be infected, and the disease may remain infectious for a long period.

Although today, some 200 fungi are established as pathogenic for humans, causing a wide range of diverse mycoses (with an incidence measured in millions and a worldwide distribution), through the mid-nineteenth century, only two human diseases (or rather disease complexes) caused by fungi were generally recognized. These were ringworm and thrush, known since Roman times. Two important additions came at the end of the century: mycetoma of the foot (Carter 1874) and aspergillosis (Lucet 1897; Rénon 1897).

Fungi were the first pathogenic microorganisms to be recognized. Toward the end of the eighteenth century and the opening years of the nineteenth, they had been shown experimentally to cause disease in plants and insects, and during the 1840s both ringworm and thrush were shown to be mycotic in origin. For a short period, fungi were blamed for causing many diseases. Cholera, for example, was attributed to fungi. But with the recognition of the major role played by bacteria (and later, viruses) in the etiology of human disease, fungi were neglected and medical mycology became very confused. It has been only since the 1930s, with the deployment of trained mycologists to work in conjunction with clinicians, that the identity of the pathogenic fungi has been clarified, and studies on their ecology have done much to elucidate epidemiological problems. In general, the geographic distribution of mycoses (which at first tended to coincide with that of medical mycologists) has been established, and the relation of mycoses to other human diseases has been brought into perspective.

Cytomegalic inclusion disease (CID) usually occurs as a subclinical infection followed by periodic reactivation revealed by shedding of the virus. It may be serious in the neonate when infection is transmitted to the fetus in utero.

Clinical Manifestations and Pathology

Cytomegalic infection is characterized histologically by the presence of large cells containing inclusion bodies in the midst of an infiltration of mononuclear cells that may be present in any of the body organs.

In prenatal infections most infants are born without clinical evidence of disease, although some 10 to 15 percent may show microcephaly, retardation of growth or mental development, hepatosplenomegaly, jaundice, and calcifications in the brain. There may be abnormalities in liver function tests and in hematopoiesis. Some 10 to 30 percent of infants with symptomatic disease die in early life. Evidence of involvement of the central nervous system can develop in the early years of life, even though the child may appear normal. The evidence is manifested as impaired intellect, neuromuscular abnormalities, chorioretinitis, optic atrophy, or hearing loss.

Neonatal infection acquired at birth from an infected cervix or later from the mother’s milk usually goes unnoticed but can be identified by the development of antibodies. In addition, respiratory symptoms including pneumonia, as well as petechial rash and enlargement of the liver and the spleen, may occur. In these cases, however, acute involvement of the central nervous system is rare. Infection in children is generally asymptomatic and is evidenced only by the development of antibodies and the shedding of virus. Occasionally hepatosplenomegaly and abnormal liver function are found. There is no proof that pharyngitis occurs at the presumed portal of entry.