Contemporary neurology series最新文献

The first case of cerebral paragonimiasis was reported by Otani in Japan in 1887. This was nine years after Kerbert's discovery of the fluke in the lungs of Bengal tigers and seven years after a human pulmonary infection by the fluke was demonstrated by Baelz and Manson. The first case was a 26-year-old man who had been suffering from cough and hemosputum for one year. The patient developed convulsive seizures with subsequent coma and died. The postmortem examination showed cystic lesions in the right frontal and occipital lobes. An adult fluke was found in the occipital lesion and another was seen in a gross specimen of normal brain tissue around the affected occipital lobe. Two years after Otani's discovery, at autopsy a 29-year-old man with a history of Jacksonian seizure was reported as having cerebral paragonimiasis. Some time later, however, it was confirmed that the case was actually cerebral schistosomiasis japonica. Subsequently, cases of cerebral paragonimiasis were reported. However, the majority of these cases were not confirmed histologically. It was pointed out that some of these early cases were probably not Paragonimus infection. After World War II, reviews as well as case reports were published. Recently, investigations have been reported from Korea, with a clinicla study on 62 cases of cerebral paragonimiasis seen at the Neurology Department of the National Medical Center, Seoul, between 1958 and 1964. In 1971 Higashi described a statistical study on 105 cases of cerebral paragonimiasis that had been treated surgically in Japan.

The striking concentration of amyotrophic lateral sclerosis (ALS) and parkinsonismdementia (PD) among the Chamorro people of Guam has attracted attention since the early 1950's. Extensive studies conducted over the past 20 years have not revealed the causes of these diseases and the reasons for their remarkable concentrations on Guam. Approximately 1 in 10 Guamanian deaths over age 25 is from ALS, and another 1 in 10 is from PD. ALS still occurs approximately 50 times more frequently on Guam than in the continental United States. Advances in virologic and biochemical techniques, and long years of study, appear to be leading us to a point where some of the problems will be resolved. We shall attempt to review the pertinent background information and summarize the data and observations up to the present time.

The most striking single feature of the clinical manifestations of leprosy is the very wide range of appearances shown by the skin lesions. These include the vague, hypopigmented macules of indeterminate leprosy; the large, sharply defined hypopigmented anaesthetic lesions of tuberculoid leprosy; the nodules and diffuse infiltration of lepromatous leprosy; and a wide range of plaques and annular lesions of the intermediate (borderline or dimorphous) types of disease. From superficial appearances it would be impossible to say that these were manifestations of the same infection. Moreover, histologically there is a similar wide range of appearances, including minimal lymphocyte infiltration around the neurovascular bundles in indeterminate leprosy; epithelioid granulomata with Langhans' giant cells and virtually no bacilli to be seen in tuberculoid cases; and in lepromatous leprosy, histiocytic infiltration occupying the whole thickness of the dermis, with massive parasitisation by Mycobacterium leprae, of which there may be up to 10-9 per gram of tissue. Strain differences have been recognised, but appear not to be responsible for this remarkable range of manifestations; they are caused by variations in the soil, not in the seed. Leprosy is almost unique among infectious diseases in the importance that host factors play in its development. The different clinical appearances shown by different types of leprosy can be graded into a continuous series, forming a spectrum from the single or scanty lesions of tuberculoid leprosy to the total body involvement o lepromatous; and the patient with leprosy can indeed truly be said to wear his skin lesions like medals displaying his capacity to resist Myco. leprate. The histopathologic features of the developed disease have a similar spectrum, from an epithelioid granuloma to infiltration with histiocytes that are full of bacilli and are ultimately converted to foam cells (Virchow cells)...

Cerebral malaria is an acute diffuse encephalopathy associated only with Plasmodium falciparum. It is probably a consequence of the rapid proliferation of the parasites in the body of man in relation to red cell invasion, and results in stagnation of blood flow in cerebralcapillaries with thromobotic occlusion of large numbers of cerebral capillaries. The subsequent cerebral pathology is cerebral infarction with haemorrhage and cerebral oedema. The wide prevalence of P. falciparum in highly endemic areas results in daily challenges to patients from several infected mosquitoes. It is thus important to understand the characteristics of P. falciparum, since this is one of the most important protozoan parasites of man and severe infection from it constitutes one of the few real clinical emergencies in tropical medicine. One of the more important aspects of the practice of medicine in the tropics is to establish a good understanding of the pattern of medical practice in that area. This applies to malaria as well as to other diseases. The neophyte might be somewhat surprised to learn, for example that an experienced colleague who lives in a holoendemic malarious area such as West Africa, sees no cerebral malaria. But the explanation is simple when the doctor concerned has a practice which involves treating adults only. Cerebral malaria is rare in adults, because in highly endemic areas, by the age of 1 year most of the infants in a group under study have already experienced their first falciparum infection. By the time they reach adult life, they have a solid immunity against severe falciparum infections. In fact, "clinical malaria" could occur in such a group under only two circumstances: 1) in pregnancy, a patent infection with P. falciparum might develop, probably due to an IgG drain across the placenta to the foetus;2) in an individual who has constantly taken antimalarials and who may have an immunity at such a low level that when antimalarial therapy is interrupted, clinical malaria might ensue. The above examples emphasise the paramount importance of the clinician dealing with malaria having some insight into the complex immunity processes operative in the human host; these have been reviewed by McGregor.

Myasthenia gravis is a subject of tremendous interest ot neurologists. Snake poisoning, however, which gives rise to a clinical picture resembling a myasthenic crisis, has evoked little interest among neurologists. This state of affairs exists partly because most snake bites occur in areas where physicians, let alone neurologists, are not commonly found. Hence, few neurologists have seen a case of snake bite with nervous system involvement. This is unfortunate, because many of the published cases of snake bite are the poorer for the lack of detailed examination and observations that a neurologist might have provided. Not only is the clinical picture of snake envenomation a fascinating one where the neurologist, haematologist, cardiologist, and renal physician can find a common clinical interest, but an understanding of the way in which snake venoms act on the nervous system is of importance to the neurologist since the neurotoxic snake venoms act principally at the neuromuscular junction. They produce a flaccid paralysis of the voluntary muscles and cause death from respiratory obstruction and/or respiratory insufficiency. Like the purified defibrinating fraction("Arvin") of the venom of the Malayan pit viper (Agkistrodon rehodostoma), which is currently being used and evaluated as an anticoagulant, the thereapeutic possibilities of a purified neurotoxin that could produce a flaccid paralysis lasting two days or more were anticipated well before 1891 by Lauterer, who, as a result of his experiments, "injected viper poison...under the skin of a boy suffering from tentanus treaumaticus (lockjaw) and slackened the muscles of the whole body by it." This chapter will initially describe the clinical picture of nervous system involvement in snake bite, with particular emphasis on Australian snake bite. The description will be based on observations made at the Port Moresby General Hospital over a period of seven years on 56 patients with paralysis following snake bite, and on some published cases of Australian snake bite. The discussion will then cover some of the recent published work on the action of snake venoms on the nervous system, dealing mainly with elapid venoms. There are several recent reviews describing the toxic properties and actions of all types of snake venoms.