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INFECTIONS OF THE NERVOUS SYSTEM AND SARCOIDOSIS
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INFECTIONS CAUSED BY RICKETTSIAS, PROTOZOA, AND WORMS Rickettsial Diseases
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Rickettsias are obligate intracellular parasites that appear microscopically as pleomorphic coccobacilli The major ones are maintained in nature by a cycle involving an animal reservoir, an insect vector (lice, eas, mites, and ticks), and humans Epidemic typhus is an exception, involving only lice and human beings, and Q fever is probably contracted by inhalation At the time of the First World War, the rickettsial diseases, typhus in particular, were remarkably prevalent and of the utmost gravity In eastern Europe, between 1915 and 1922, there were an estimated 30 million cases of typhus with 3 million deaths Now, the rickettsial diseases are of minor importance, the result of insect control by DDT and other chemicals and the therapeutic effectiveness of broad-spectrum antibiotics In the United States these diseases are quite rare, but they assume signi cance because, in some types, up to one-third of patients have neurologic manifestations About 200 cases of Rocky Mountain spotted fever (the most common rickettsial disease) occur each year in the United States, with a mortality of 5 percent or less Neurologic manifestations occur in a small portion, and neurologists may not encounter a single instance in a lifetime of practice For this reason, the rickettsial diseases are simply tabulated here (A comprehensive account may be found in Harrison s Principles of Internal Medicine) The following are the major rickettsial diseases: Epidemic typhus, small pockets of which are present in many undeveloped parts of the world It is transmitted from lice to humans and from person to person 2 Murine (endemic) typhus, which is present in the same areas as Rocky Mountain spotted fever (see below) It is transmitted by rat eas from rats to humans 3 Scrub typhus or tsutsugamushi fever, which is con ned to eastern and southeastern Asia It is transmitted by mites from infected rodents or humans 4 Rocky Mountain spotted fever, rst described in Montana, is most common in Long Island, Tennessee, Virginia, North Carolina, and Maryland It is transmitted by special varieties of ticks 5 Q fever, which has a worldwide distribution (except for the Scandinavian countries and the tropics) It is transmitted in nature by ticks but also by inhalation of dust and handling of materials infected by the causative organism, Coxiella burnetii With the exception of Q fever, the clinical manifestations and pathologic effects of the rickettsial diseases are much the same, varying only in severity Typhus may be taken as the prototype The incubation period varies from 3 to 18 days The onset is usually abrupt, with fever rising to extreme levels over several days; headache, often severe; and prostration A macular rash, which resembles that of measles and involves the trunk and limbs, appears on the fourth or fth febrile day An important diagnostic sign in scrub typhus is the necrotic ulcer and eschar at the site of attachment of the infected mite Delirium followed by progressive stupor and coma, sustained fever, and occasionally focal neurologic signs and 1
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optic neuritis characterizes the untreated cases Stiffness of the neck is noted only rarely, and the CSF may be entirely normal or show only a modest lymphocytic pleocytosis In fatal cases, the rickettsial lesions are scattered diffusely throughout the brain, affecting gray and white matter alike The changes consist of swelling and proliferation of endothelial cells of small vessels and a microglial reaction, with the formation of so-called typhus nodules Q fever, unlike the other rickettsioses, is not associated with an exanthem or agglutinins for the Proteus bacteria (Felix-Weil reaction) In the few cases with which we are familiar, the main symptoms were those of a low-grade meningitis Rare instances of encephalitis, cerebellitis, and myelitis are also reported, possibly as postinfectious complications There is usually a tracheobronchitis or atypical pneumonia (one in which no organism can be cultured from the sputum) and a severe prodromal headache In these respects, the pulmonary and the neurologic illness resembles that of the other main cause of atypical pneumonia, M pneumoniae The Q fever agent (Coxiella) should be suspected if there are concomitant respiratory and meningoencephalitic illnesses and there has been exposure to parturient animals, to livestock (including abattoir workers), or to wild deer or rabbits The diagnosis can be made by the nding of a severalfold increase in speci c immuno xation antibodies Patients who survive the illness usually recover completely; a few are left with residual neurologic signs Treatment This consists of the administration of chloramphenicol or tetracycline, which are highly effective in all rickettsial diseases If these drugs are given early, coincident with the appearance of the rash, symptoms abate dramatically and little further therapy is required Cases recognized late in the course of the disease require considerable supportive care, including the administration of corticosteroids, maintenance of blood volume to overcome the effects of the septic-toxic reaction, and hypoproteinemia
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