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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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surgical measures that have been advocated for this disorder Many patients with the most persistent complaints, beyond a year, have all the major symptoms of a depressive state and will be helped by appropriate antidepressive medications
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NEUROLOGIC DISEASES INDUCED BY RETROVIRUSES AND RESULTANT OPPORTUNISTIC INFECTIONS
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Retroviruses are a large group of RNA viruses, so called because they contain the enzyme reverse transcriptase, which permits the reverse ow of genetic information from RNA to DNA Two families of retroviruses are known to infect humans: (1) the lentiviruses, the most important of which is the human immunode ciency virus (HIV), the cause of AIDS, and (2) the oncornaviruses, which include the human T-cell lymphotropic viruses, ie, the agents that induce chronic T-cell leukemias and lymphomas (HTLV-II) and tropical spastic paraparesis (HTLV-I) In the following pages, we consider the major neurologic syndromes induced by the two main human retroviruses AIDS, a constellation of neurologic diseases caused by HIV, and tropical spastic paraparesis, caused by HTLV-I These diseases are now the subjects of great public interest and laboratory investigation A comprehensive account of the neurobiology, pathology, and clinical features of these infections can be found in the appropriate sections of Harrison s Principles of Internal Medicine
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At the time of this writing it was estimated by the World Health Organization (WHO) that approximately 34 million adults were infected worldwide and that about 850,000 adults in the United States were seropositive for the virus By all accounts, the incidence will continue to increase in the immediate future Particularly startling are the statistics from sub-Saharan Africa and Southeast Asia, where the WHO estimated that about 25,000,000 adults, or almost 9 percent of the adult population, were infected In some areas of East Africa, 30 percent of adults are infected with the virus In the United States, AIDS affects mainly homosexual and bisexual males (53 percent of all cases) and male and female drug users (30 percent) Somewhat less than 3 percent of patients at risk are hemophiliacs and others who receive infected blood or blood products, and the disease has occurred in infants born of mothers with AIDS Moreover, this virus may be transmitted by asymptomatic and still immunologically competent mothers to their offspring Spread of the disease by heterosexual contact accounts for about 5 percent of cases, but this number is gradually increasing, partly through the activities of intravenous drug users (By contrast, an estimated 80 percent of African AIDS patients acquire their disease through heterosexual contact) Clinical Features Infection with HIV produces a spectrum of disorders, ranging from clinically inevident seroconversion to widespread lymphadenopathy and other relatively benign systemic manifestations ranging from diarrhea, malaise, and weight loss (the so-called AIDS-related complex, or ARC) and to full-blown AIDS, which comprises the direct effects of the virus on all organ systems as well as the complicating effects of a multiplicity of parasitic, fungal, viral, and bacterial infections and a number of neoplasms (all of which require cell-mediated immunity for containment) Until the recent advent of multiple antiviral drug therapy, once the manifestations of AIDS had become established, one-half of the patients died by 1 year and most by 3 years Clinically, neurologic abnormalities were noted in only about one-third of patients with AIDS, but at autopsy the nervous system is affected in nearly all of them The infections and neoplastic lesions of the nervous system that complicate AIDS are listed in Table 33-2 Details of their pathology are to be found in the articles by Sharer and by Bell It has already been mentioned that HIV infection may present as an acute asymptomatic meningitis, with a mild lymphocytic pleocytosis and modest elevation of CSF protein The acute illness may also take the form of a meningoencephalitis or even a myelopathy or neuropathy (see below) Most patients recover from the initial acute neurologic illnesses; the relationship to AIDS may pass unrecognized, since these illnesses are quite nonspeci c clinically and may precede seroconversion Once seroconversion has occurred, the patient becomes vulnerable to all the late complications of HIV infection In adults, the interval between infection and the development of clinical AIDS ranges from several months to 15 years or even longer (the mean latency is 8 to 10 years and 1 year or less in infants) It is believed that practically all seropositive individuals will sooner or later develop AIDS, although new drugs are constantly lengthening the latent period AIDS Dementia Complex In the later stages of HIV infection, the commonest neurologic complication is a subacute or chronic HIV encephalitis presenting as a form of dementia; formerly it was called AIDS encephalopathy or encephalitis, but it is now generally referred to as the AIDS dementia complex, or ADC (Navia and Price) It has been estimated that only 3 percent of AIDS cases
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The Acquired Immunode ciency Syndrome (AIDS)
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In 1981, physicians became aware of the frequent occurrence of otherwise rare opportunistic infections and neoplasms notably Pneumocystis carinii pneumonia and Kaposi sarcoma in otherwise healthy young homosexual men The study of these patients led to the recognition of a new viral disease, AIDS In 1983, Montagnier and his colleagues isolated a retrovirus from a homosexual patient with lymphadenopathy and named it lymphadenopathy-associated virus (LAV) Shortly thereafter, Gallo and associates described a retrovirus in the blood of AIDS patients, which they called human T-cell lymphotropic virus (now HTLV-III) These two viruses, LAV and HTLV-III, were shown to be identical, and an international commission changed the name to human immunode ciency virus (HIV) sometimes referred to as HIV-1 to separate it from a similar virus (HIV-2) associated with AIDS predominantly in West Africa and elsewhere in persons of West African origin HIV infection is characterized by an acquired and usually profound depression of cell-mediated immunity, as manifest by cutaneous anergy, lymphopenia, reversal of the T-helper/T-suppressor cell ratio more accurately, CD4 /CD8 lymphocytes, as a result of reduction in CD4 cells and depressed in vitro lymphoproliferative response to various antigens and mitogens It is this failure of immune function that explains the development of a wide range of opportunistic infections and unusual neoplasms Virtually all organ systems are vulnerable, including all parts of the CNS, the peripheral nerves and roots, and muscle Moreover, the nervous system is susceptible not only to diseases that are due to immunosuppression but also to the AIDS virus infection per se Epidemiology In a span of 25 years, HIV infection and AIDS have spread worldwide, attaining immense pandemic proportions
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