We report a new case of acquired immune-deficiency syndrome (AIDS) in a 43 year-old white homosexual man, characterized by the association of disseminated cutaneo-mucous Kaposi's sarcoma and cerebral toxoplasmosis. This man had Kaposi's sarcoma for about 10 years but evolution became quickly extensive in July 1981. Chlorambucil was prescribed at that time and was the cause of a pancytopenia. Death occurred in July 1982 due to a cerebral mass identified as toxoplasmosis on a left temporal biopsy. This observation is typical of AIDS, a new syndrome which suddenly developed in the last 2 years in the United States in homosexual men, Haitians and hemophiliacs, and is characterized by disseminated Kaposi's sarcoma and/or opportunistic infections, with a very high mortality rate. Severe toxoplasmosis of CNS has been reported in AIDS and appears to result from defects in cellular immunity which permit recrudescence of latent infection. Cerebral biopsy is necessary for the diagnosis of cerebral toxoplasmosis as seroconversion occurs infrequently in immuno-suppressed hosts. AIDS appeared in Western Europe in 1982. Most of the cases were reported in France, Denmark, Belgium and Great Britain. These cases differ from reported cases in the USA: fewer drug or poppers users, fewer homosexual men, an important number of people having lived or travelled in the Kaposi's endemic area (Mediterranean basin and Central Africa). The immunological profile of patients presenting AIDS in Europe doesn't seem to differ from the american profile: serious cellular immunodeficiency and marked increase in the suppressor/cytotoxic cell population. As in the United States, one may suspect, among several hypotheses, that it is caused by one or several transmissible agents now present in France. The nature of these agents, transmissible by sexual contacts and blood, is not yet known: the role of the CMV is now less probable and most of the studies look for the role of other factors such as the HTLV.
The series comprised 479 consecutive patients from all parts of Norway operated on in the period 1955-1976. Only 8% were less than 7 years at surgery. Dominating preoperative symptoms were dyspnea and increased fatigue. The follow-up period ranged from 2 to 21 years, mean 10,28 years. There were 4 early deaths (within 30 days after surgery), and at follow-up further 5 patients had died. Excluding a man who died in a traffic accident the total mortality is 1,7%. Of the 470 surviving patients 81,7% were improved, 17% were unchanged and 1,3% had deteriorated. In patients with symptoms dyspnea and palpitations were common complaints. Different types of arrhythmia were found in 31 patients. One third were paroxysmal tachy-arrhythmias, and 9 patients had atrial fibrillation. Only 3 of the 31 patients had some type of preoperative arrhythmia, and 26 were more than 20 years of age of surgery. Seven patients were reoperated because of a residual left to right shunt, 5 of them were initially operated in hypothermia dn inflow occlusion. During the follow-up period 10 patients had late cardiovascular disorders.
In a retrospective study of 12 patients with Behçet's disease, more than half were found to originate from the Near East, where the prevalence of the disease is known to be high. The immigrant patients were all males, whereas 3 of the 5 patients with Swedish ancestry were females. Certain differences emerged between the two groups, including different sex ratio and absence of HLA B5 association and pathergy skin reaction among the Swedish patients. Moreover, serious neurological and ocular symptoms showing no tendency to recede with age afflicted all the Swedish female patients. Urogenital symptoms were, besides ulcers, common in both groups, including prostatitis, urethritis, orchitis, chronic sterile cystitis and relapsing salpingitis. Although the maternal does not allow statistical inferences, the estimated prevalence was higher than expected among both Swedish and immigrant patients. Recent studies, including the diagnostic criteria proposed by the "International Study Group for Behçet's disease", are discussed in relation to previously used criteria as well as present findings. The sensitivity and specificity of the first mentioned criteria and the ones proposed by Mason & Barnes seemed equal.
OBJECTIVE: To investigate the relation between HIV-induced brain lesions, zidovudine (ZDV) treatment and survival length in a well-defined population of HIV-positive patients. METHODS AND PATIENTS: Ulleval Hospital has the responsibility for treating all AIDS patients from the city of Oslo except haemophiliac patients. The patient population in this autopsy study comprised all adult AIDS patients in Oslo who were treated at our hospital and died during 1983-1994 (n = 171). This represents 86% of all adult AIDS patients from Oslo who died during the same period. Full autopsy, including neuropathological examination of the brain and spinal cord, was performed on 128 (75%) of those who died. RESULTS: No significant differences were found between autopsy and non-autopsy cases with regard to sex, age, risk groups, survival length or ZDV treatment. In the autopsy material, multinucleated giant cells (MGC) in brain tissue were found in 29 cases and diffuse damage of white matter in 52 cases. Analysis shows that ZDV (600 mg per day) reduced the incidence of these brain lesions, but only if continued until death. A second finding was an increased incidence of HIV-induced brain lesions for those with long-term survival. Together these observations may explain a substantial part of the time-trend in the incidence of MGC in Oslo. MGC were frequent (40%) during the first years of the epidemic, although survival length was short in this period. The incidence fell markedly around the time ZDV was introduced and later remained low in those using ZDV until death. The incidence of MGC has, however, increased during the later years, the new cases mainly occurring in patients who had discontinued ZDV use. CONCLUSION: If continued until death, ZDV can reduce the incidence of HIV-induced brain lesions in AIDS patients. When ZDV treatment is terminated a rapid increase occurs in the incidence of HIV encephalitis.
Overdrainage of the cerebrospinal fluid (CSF) and collapse of the ventricles, slit ventricles (SLV), can cause clinical symptoms and result in the slit ventricle syndrome (SLVS). The EEG changes and the frequency and type of epilepsy in patients with SLV was analysed from a material of 113 shunt-treated hydrocephalic children. During the follow-up time (mean 8.9 years), 63 patients (56%) had developed SLV. The age at initial shunting was significantly lower (1.2 years) in patients who developed SLV than for those who did not (2.7 years). After initial shunting generalized spike and sharp wave activity (SWA) developed more frequently in patients who developed SLV (81%) than in those who did not (54%). Severe generalized SWA developed almost entirely in patients in the SLV group. This severe generalized SWA disappeared from the EEG in patients after treatment of the SLVS. Epileptic seizures appeared after initial shunting in 44% of patients in the SLV group but in only 6% of the non-SLV group. Treatment of the SLVS decreased the frequency of epilepsy to a level corresponding with the non-SLV group. Repeated EEG evaluation of shunt-treated hydrocephalic children is a valuable aid in follow-up. If EEG abnormality appears after initial shunting, especially SWA, shunt malfunction and overdrainage of the CSF should be suspected.
We report a neurological disease among Cree Indian children in a northern Quebec village. The disease manifests as severe mental retardation, cerebral atrophy with white matter changes and calcifications, and systemic immunological abnormalities. Eleven cases are known in five families. The familial incidence of cases and the high degree of parental consanguinity suggest a genetic contribution. We propose that this entity may be caused by an unusual viral infection in a genetically vulnerable host.
In the post human-genome area, the challenge is to derive details of heritable variation in relation to how human variation reflects adaptation to the different environments. Heterozygote advantage represents a superior genetic adaptation presumably explaining the presence of the allele at frequencies above those to be expected from a simple replacement of a homozygous lethal allele by mutation alone (Saugstad 1977a, 1975b, 1972). Mean birthweight of unaffected offspring of parents heterozygous for the phenylketonuria (PKU) allele averaged significantly above mean weight of all Norwegian births, rendering unaffected offspring more viable at birth and thus improving the chance for survival of the allele. A successful adaptation requires natural selection acting on that part of the body that makes a difference in survival. Skin colour variation is such a successful adaptation, for the North as opposed to the dark skins of the equator. Human Evolution in Africa and subsequent adaptations have enabled human survival all over the world with highly different light intensity (Jablonski & Chaplin 2000). That continuous variables, height, pubertal age and brain development, are multifactorially inherited and affected by epigenetic factors, was nicely demonstrated in the increase in height in Norway 1860-1960 with at the same time a reduction in pubertal age by 4yrs which may have affected the final stage in brain development. This created an increased need for brain food, N-3, to secure optimal brain function. Body growth is not brain growth. Given that the consumption of brain food (N-3) has declined to 20% only of the level 100yrs ago, what disorders are to be expected with an N-3 dietary deficit: in pregnancy, infancy and later in life? In this paper I discuss the significance of prepubertal selective pruning of excitatory synapses compared to delayed pruning and suggest relationships with brain disorders.