Antibodies to human T-cell leukemia virus membrane antigens (HTLV-MA) in hemophiliacs.

Along with homosexual men, Haitians, and intravenous drug abusers, hemophiliacs are at high risk of contracting acquired immunodeficiency syndrome (AIDS). An earlier study revealed that 36 percent of a group of the AIDS patients had antibodies to cell membrane antigens associated with the human T-cell leukemia virus (HTLV-MA), whereas only 1.2 percent of matched asymptomatic homosexual controls had these antibodies. In the present experiments, serum samples from 172 asymptomatic hemophiliacs were examined for the presence of antibodies to HTLV-MA. Such antibodies were detected in 5 to 19 percent of the hemophiliacs examined from four geographical locations, but in only 1 percent or less of laboratory workers, normal blood donors, donors on hemodialysis, or donors with chronic active hepatitis.

Developing ICF Core Sets for persons with sleep disorders based on the International Classification of Functioning, Disability and Health.

BACKGROUND: With the International Classification of Functioning, Disability and Health (ICF), we can now rely on a globally agreed-upon framework and system for classifying the typical spectrum of problems in the functioning of persons given the environmental context in which they live. ICF Core Sets are subgroups of ICF items selected to capture those aspects of functioning that are most likely to be affected by sleep disorders. OBJECTIVE: The objective of this paper is to outline the developmental process for the ICF Core Sets for Sleep. METHODS: The ICF Core Sets for Sleep will be defined at an ICF Core Sets Consensus Conference, which will integrate evidence from preliminary studies, namely (a) a systematic literature review regarding the outcomes used in clinical trials and observational studies, (b) focus groups with people in different regions of the world who have sleep disorders, (c) an expert survey with the involvement of international clinical experts, and (d) a cross-sectional study of people with sleep disorders in different regions of the world. CONCLUSION: The ICF Core Sets for Sleep are being designed with the goal of providing useful standards for research, clinical practice and teaching. It is hypothesized that the ICF Core Sets for Sleep will stimulate research that leads to an improved understanding of functioning, disability, and health in sleep medicine. It is of further hope that such research will lead to interventions and accommodations that improve the restoration and maintenance of functioning and minimize disability among people with sleep disorders throughout the world.

Restless legs syndrome in hemodialysis patients: health-related quality of life and laboratory data analysis.

AIMS: To compare clinical data, sleep quality and health-related quality of life (HRQOL) with and without RLS in HD patients. MATERIALS AND METHODS: The international RLS study group diagnosis questionnaire was completed by 228 HD patients. The Pittsburg Sleep Quality Index (PSQI) for the evaluation of sleep quality and the Kidney Disease Quality of Life (KDQOL-SF) for the analysis of HRQOL were also used. RESULTS: 53 (23%) patients were diagnosed as RLS. Age and age at the initiation of HD were significantly younger in the RLS group. Serum calcium concentration (Ca) was significantly higher in the RLS group. Sleep quality evaluated by PSQI was significantly lower in the RLS group. In SF-36 domains of KDQOL-SF, bodily pain, general health perceptions, vitality, role functioning emotional, mental health and mental component score were significantly lower in the RLS group. In kidney targeted scales of KDQOL-SF, symptoms/problems, burden of kidney disease, cognitive function, quality of social interaction, sleep and patient satisfaction were significantly lower in the RLS group. CONCLUSION: High Ca was possibly connected to the pathophysiology of RLS which impaired sleep quality as well as HRQOL including mental health and many kidney disease related scales.

Case-control study of factors associated with human T-cell leukemia virus type I infection in southern Miyazaki, Japan.

BACKGROUND: An unusual age- and sex-specific distribution and a remarkably restricted geographic seroprevalence characterize human T-cell leukemia virus type I (HTLV-I) infection. Although the transmission routes of HTLV-I are known, these seroepidemiologic features cannot be fully explained. PURPOSE: This study was designed to identify potential characteristics associated with HTLV-I infection in a highly endemic Japanese community. METHODS: We evaluated occupational, residential, dietary, and medical histories in a case-control study conducted in two neighboring villages in southeastern Miyazaki Prefecture. One hundred forty-four case subjects and 276 control subjects, frequency matched by age, sex, and village, were interviewed. Village-specific profiles of demographic determinants of HTLV-I seroprevalence were generated using multiple logistic regression. RESULTS: Although a different pattern of factors was found for each village, occupations of the subjects and their fathers were associated with HTLV-I infection in both communities--farming in village A and fishing and farming in village B. For village A, there was more than a twofold association both with residence in the township for 55 years or more and with living in a particular area within the village. In addition, case subjects were more likely to have a mother who was deceased (odds ratio = 1.7; 95% confidence interval = 0.96-2.9). CONCLUSIONS: HTLV-I infection is characterized by a high degree of microepidemicity in this population, with seroprevalence related to both sociologic and geographic determinants. Moreover, as carriers' mothers themselves have a higher probability of being HTLV-I positive, an increased mortality among those infected with the virus is suggested.

Polysomnographic characteristics of healthy elderly subjects with somnambulism-like behaviors.

We compared the sleep characteristics of seven healthy elderly people complaining of nocturnal somnambulism-like behaviors with those of 14 age-matched healthy elderly people who had never shown such behavior. Polysomnographic data revealed the appearance of "Stage 1-REM with tonic electromyographic (EMG) activity" sometimes accompanied by abnormal behavior in the first group, but the sleep architecture and parameters showed no significant difference between the two groups except for higher REM density in the first group. "Stage 1-REM with tonic EMG," observed in the first group was considered equivalent to REM sleep without muscle atonia. It is suggested that both reduced activity of tonic phenomena (muscle atonia) and increased activity of phasic phenomena (higher REM density) of REM sleep are essential for the induction of somnambulism-like behaviors during sleep in healthy elderly subjects.

Inhibitory effect of ethanolamine plasmalogen on iron- and copper-dependent lipid peroxidation.

The effect of ethanolamine plasmalogen (EtnPm) on lipid peroxidation was investigated in liposomal suspension of egg yolk phosphatidylcholine. EtnPm inhibited iron- and copper-dependent peroxidation in the presence of preformed hydroperoxides, although it was not effective for radical initiator mediated lipid peroxidation. EtnPm resulted in complete binding of iron to liposomal lipids, suggesting that EtnPm exerts its inhibitory effect on lipid peroxidation through inhibiting preformed peroxide decomposition by trapping transition metal ions.

Suppression of delayed-type hypersensitivity to PPD and PHA in elderly HTLV-I carriers.

In a previous study on immune responsiveness among asymptomatic human T-cell leukemia virus type I (HTLV-I) carriers, we found that carriers had significantly reduced delayed-type hypersensitivity (DTH) response to purified protein derivative (PPD) skin testing. The association was strongest among persons at least 60 years of age. In order to evaluate this finding further, we evaluated the response to both PPD and phytohemagglutinin (PHA) in an elderly population. Fifty-six consecutive hospitalized patients with nonimmunosuppressive diseases were examined. None had a history of tuberculosis nor evidence of the known HTLV-I-associated diseases. The subjects' ages ranged from 62-93 years (median = 75 years); 43 were women and 13 were men. Twenty-two of the subjects were HTLV-I antibody positive. Among the carriers, there was an increased level of nonreactivity to PPD, the relative risk adjusted for age (RR) being 1.9 (95% confidence interval, 0.62-5.8), as well as to PHA of RR = 2.3 (0.60-9.0). When subjects were cross-classified for response to both skin tests, 15 of 17 carriers were nonreactive to either or both antigens compared to 15 of 25 noncarriers [RR = 5.1 (0.99-25.9) (p value, one-sided = 0.026)]. The decline in reactivity to both antigens increased with age, but was consistently lower among the carriers. Among subjects with positive reactions to PPD, the degree of reaction as measured by the size of erythema was reduced among the carriers; however, for PHA responders, the response in carriers appeared to be normal. Among the HTLV-I antibody negative subjects, the size of erythema for both antigens was strongly correlated (p = 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Comparative diagnostic assay results for detecting antibody to HTLV-I in Japanese blood donor samples: higher positivity rates by particle agglutination assay [corrected].

Higher positivity rates for prevalence of anti-HTLV-I antibody have been reported for the gelatin particle agglutination (PA) assay when compared to that of the indirect immunofluorescence assay using acetone-fixed HTLV-I producing cells (IF-FA). To evaluate the discrepancy between these two screening methods, PA-positive/IF-FA-negative sera were tested by four additional assays for anti-HTLV-I: indirect immunofluorescence assay using live HUT102 cell membranes (IF-MA), enzyme immunoassay (EIA), radioimmunoprecipitation (RIP), and Western blotting (WB). Sera obtained from 6915 Japanese blood donors were assayed for anti-HTLV-I antibody by PA, and 389 (5.6%) were positive. These 389 sera were re-examined by IF-FA, and 29 (7.5%) were negative. Sufficient material was present for 20 of the 29 PA-positive/IF-FA-negative sera for further evaluation by the IF-MA, EIA, RIP, and WB. Fifteen (75%) of the 20 were positive by IF-MA, but only 6 (30%) were positive by EIA. Both RIP and WB confirmed 17 (85%) of the samples, with each detecting a serum that was negative by the other. Thus, 18 (90%) of the 20 were confirmed by either RIP or WB. The nonconfirmed sera were all positive on PA at low titer. These findings suggest that the PA assay is more sensitive than either IF-FA or EIA.

Determinants of HTLV-1 seroprevalence in Miyazaki Prefecture, Japan: a cross-sectional study.

To evaluate determinants of the prevalence of human T-cell leukemia virus type I (HTLV-I) antibody positivity in an endemic region, Miyazaki Prefecture, Japan, demographic and serologic data were collected on 7,055 individuals consecutively seen at Miyazaki City Health Promotion Center between September 1983 and December 1984. The overall HTLV-I seroprevalence was 8.5%; age and gender distributions of HTLV-1 antibody positivity were consistent with previous findings for endemic populations. The prefecture could be divided into two geographic areas based on seroprevalence: high prevalence (12.1%) in the southwest and medium prevalence (6.6%) for the rest of the prefecture. Current occupation in fishing, forestry, or livestock raising significantly correlated with HTLV-1 seropositivity [relative risk (RR) = 3.0, 2.5, 2.0, respectively]; farming also was associated but only in the medium prevalence region (RR = 1.3; p = 0.06). For a subset of 157 HTLV-I-positives and 175 negative controls screened for antibodies to the toxoplasmosis organisms, toxoplasma exposure was not significantly associated with HTLV-I infection. In this endemic Japanese population, both geographic and sociologic factors characterized the distribution of HTLV-I seroprevalence, reflecting transmission patterns and historical duration of the infection.

Changes in hematologic parameters among Japanese HTLV-I carriers.

Hematologic data from 1,039 persons who participated in the Miyazaki Cohort study on human T-cell lymphotropic virus type-I (HTLV-I) infection were analyzed. Individuals were classified by HTLV-I antibody status and the presence of abnormal lymphocytes (Ably). We identified several differences in selected leukocyte populations: lymphocyte percent was higher among the HTLV-I carriers with Ably (36.5 +/- 2.0%, n = 29) compared with the carriers without Ably (33.1 +/- 0.6%, n = 299) and the seronegatives 36.4 +/- 0.4%, n = 711) (p = 0.04). Conversely, there was a trend of decreasing eosinophil percent among both carrier groups with the lowest percent among carriers with Ably (1.8 +/- 0.5%) compared with the seronegatives (2.8 +/- 0.1%) (p = 0.05). Mean basophil percent was decreased among both carriers groups (p = 0.09). Additionally, red cell count was elevated among the carriers with Ably (461 +/- 7 x 10(4)/mm3) compared with the seronegatives (446 +/- 2 x 10(4)/mm3) (p = 0.03). The HTLV-I carriers with Ably had lower serum albumin (4.39 +/- 0.05 g%) compared with the seronegatives (4.47 +/- 0.01 g%) (p = 0.10). These alterations may be a consequence of HTLV-I infection, with the greatest changes among carriers with Ably, a subset thought to be at risk for developing adult T-cell leukemia.

The distribution of T-cell subsets among HTLV-I carriers in Japan.

Data on T-cell subsets from 89 human T-cell lymphotropic virus-I (HTLV-I) carriers and 25 seronegative people were analyzed to identify differences in T-cell subset values among three subgroups: HTLV-I carriers with abnormal lymphocytes (Ably; n = 24), carriers without Ably (n = 65), and HTLV-I seronegatives (n = 25). Estimates of mean values were adjusted for age, sex, smoking, and alcohol drinking, as appropriate. The percentage of CD25+ T cells was elevated in carriers with Ably (mean, 16.7 +/- 1.0) compared with the seronegatives (11.4 +/- 1.4; p = 0.0002); individuals with CD25 T-cell percentages above the median for the seronegatives had a corresponding 5.4-fold risk for being a carrier with Ably. Similarly, the percentage of CD4 T cells was elevated in carriers with Ably. Conversely, the percentage of CD8 T cells was lower among both groups of HTLV-I carriers than in the seronegatives. There was a corresponding significant increase (p = 0.0004) of the CD4/CD8 ratio among carriers with Ably (1.57 +/- 0.12) compared with the seronegatives (1.22 +/- 0.12). Among subjects with CD4/CD8 ratios above the median for the seronegatives, there were 6.8- and 4.5-fold risks for being carriers with or without Ably, respectively. The percentage of CD7 was lower among carriers with Ably (75.6 +/- 1.6) than among seronegatives (78.9 +/- 1.5; p = 0.13). The percentage of beta-interleukin-2-receptor-positive T cells did not vary among the three subgroups.(ABSTRACT TRUNCATED AT 250 WORDS)

Association of HTLV-I antibody profile of asymptomatic carriers with proviral DNA levels of peripheral blood mononuclear cells.

The human T-lymphotropic virus type I (HTLV-I) antibody profile of 216 asymptomatic carriers in Miyazaki, Japan, was analyzed in conjunction with the HTLV-I proviral DNA levels in their peripheral blood mononuclear cells (PBMC) determined by the semiquantitative polymerase chain reaction (PCR) method. The geometric mean HTLV-I titer by particle agglutination assay (PA) of 58 subjects (27%) with a high DNA level was 1:1, 240; 94 (44%) with a medium DNA level, 1:740; 38 (18%) with a low level, 1:476; and, 26 (12%) with an undetectable level, 1:263. Moreover, when the subjects were divided into four groups according to titer from high to low, the correlation between DNA level and antibody titer level was highly significant (p < 0.0001). HTLV-I antibody subclass by Western blot (WB) was determined for 78 randomly selected samples from these carriers. Immunoglobulin (Ig) M antibody was detected in 35 (45%). The mean PA antibody titer was higher in carriers with IgM antibody than in those without, at each detectable proviral DNA level. These findings suggest that HTLV-I antibody titer is related to proviral DNA level and also to the presence of IgM antibodies among those with proviral DNA of the same level. Seven carriers (9%) were negative for IgG antibody by WB, among whom the proviral DNA level was low or undetectable and the PA titer was also low. Three of these were positive only for IgM antibody.

Increased prevalence of vascular dementia in Japan: a community-based epidemiological study.

BACKGROUND AND OBJECTIVE: It has been suggested that there is a major difference in the ratio of AD to vascular dementia (VaD) between Japan and Western countries. To determine the type-specific prevalence of dementia in community-dwelling elderly from the Japanese community of Nakayama, all patients with dementing illness underwent a CT scan. METHODS: A door-to-door three-phase population survey was carried out on all persons aged 65 years and older residing at home on the prevalence day (January 1, 1997). The ascertainment of cases was made between January 1997 and March 1998. The study included a psychiatric interview; physical, neurologic, and neuropsychologic examinations; comprehensive laboratory tests; and cranial CT. A public health nurse also interviewed a person close to each subject. Dementia was defined according to the Diagnostic and Statistical Manual of Mental Disorders, third edition-revised, criteria, AD according to the National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association, and VaD according to Diagnostic and Statistical Manual of Mental Disorders, fourth edition, combined with information from the patient's neurologic history and CT scanning. RESULTS: Of 1438 inhabitants, 1162 (81.0%) completed the protocol. The prevalence of dementia was 4.8%. Of the 60 subjects with dementia, 35% had AD, 47% had VaD, and 17% had dementia resulting from other causes. CONCLUSIONS: The prevalence of dementia was similar to previous reports, but, contrary to results of virtually all studies conducted in developed countries and those recently conducted in Japan, almost half of the cases in the present study appeared to have VaD with neuroradiologic confirmation.

Flow cytometric determination of intracytoplasmic Wiskott-Aldrich syndrome protein in peripheral blood lymphocyte subpopulations.

We have produced a novel monoclonal antibody (mAb) directed against Wiskott-Aldrich syndrome protein (WASP) by immunizing mice with the recombinant protein. The mAb designated 5A5 is highly specific to WASP and suitable for Western blot analysis and immunoprecipitation. A flow cytometric assay using the 5A5 mAb identifies expression of intracytoplasmic WASP in lymphocytes from normal individuals. Double staining analysis with cell surface CD3, CD19, and CD56, and intracytoplasmic molecules revealed WASP expression in each subpopulation. With regard to WASP expression in patients with Wiskott-Aldrich syndrome (WAS) and X-linked thrombocytopenia (XLT), peripheral blood mononuclear cells (PBMCs) from nine patients and Epstein-Barr virus-transformed B-lymphoblastoid cell lines from seven patients examined did not show WASP expression by flow cytometric analysis. These results were confirmed by Western blot analysis. We conclude that WASP expression in lymphocyte subpopulations from patients with WAS and XLT can be more precisely evaluated by flow cytometry as compared with Western blot analysis. This flow cytometry method is important as a supplement to Western blots, but even more important as an alternative and powerful assay that can contribute to research on WASP as well as diagnosis in a clinical setting.

Antibodies to HTLV-1 in saliva of seropositive individuals from Japan.

Secretory antibodies protect mucosal surfaces against transmission of many viruses. Human T-lymphotropic Virus, Type I (HTLV-I) is transmitted via blood products and via sexual contact across mucosal surfaces. We investigated the presence of HTLV-I-specific antibodies in whole saliva samples from 10 seronegative and 28 seropositive volunteers from a hospital in southern Japan. Antibodies directed to HTLV-I antigens were found in the salivas from 22 of 28 (79%) of the seropositive subjects. None of the seronegative individuals showed evidence of salivary antibodies. Antibodies directed to the envelope antigens of the virus were found in 21 of 22 positive saliva samples. Secretory antibodies may be important in preventing mucosal transmission.

Subclinical REM sleep behavior disorder in a patient with corticobasal degeneration.

Various neurodegenerative diseases have been reported to be associated with rapid eye movement (REM) sleep behavior disorder (RBD). This is the first report of a patient with corticobasal degeneration (CBD) associated with subclinical RBD. A 72-year-old woman was admitted complaining of fine tremor of the right hand and weakness of the right lower extremity. She was diagnosed as having CBD on the basis of clinical features and neuroimaging studies. Her family noticed snoring and increase in sleep talk, but they did not regard them as pathological. All-night polysomnography (PSG) revealed REM sleep without atonia (RWA) during which 14 episodes of talking and singing were observed. They ranged from the utterance of one word to that of comprehensible words of a song for about 3 minutes accompanied by various nonpurposeful movements of the mouth, hands, and limbs. These episodes were not associated with any sleep-disturbed breathing. Future PSG studies on CBD patients together with postmortem analysis of brain stem structures that are crucial for generating REM sleep-related atonia are warranted for further understanding of the pathophysiological mechanism of RBD.

Sleep bruxism as a manifestation of subclinical rapid eye movement sleep behavior disorder.

A 59-year-old man with temporomandibular joint pain/dysfunction syndrome presented with a long history of nocturnal bruxism. All-night polysomnographic recordings with video monitoring showed episodes of teeth grinding or clenching occurring exclusively during rapid eye movement (REM) sleep, which were often associated with movements of the hands and fingers and/or brief vocalization. Throughout REM sleep, there was excessive phasic chin electromyographic twitching, without increased tone, and also excessive phasic electromyographic twitching in multiple muscle sites. The patient maintained a normal nonrapid eye movement-REM cycle, but showed increased REM density. Polysomnographic characteristics suggested that there may be a common pathophysiology in a certain type of sleep bruxism and the REM sleep behavior disorder. Sleep bruxism seen in this case is concluded to be a manifestation of subclinical REM sleep behavior disorder.

Supplementary motor area seizure resembling sleep disorder.

Two patients presented with a complaint of frequent sudden arousals during sleep followed by tachypnea and palpitation associated with stiffness in the upper extremities in one case and by elevation of the left lower limb in the other. All night video-electroencephalogram (EEG) polysomnography (VPSG) confirmed the diagnosis of seizure arising from the supplementary motor area (SMA seizure) in both cases. Carbamazepine (CBZ) produced remarkable improvement both in clinical seizures and in their subjective sleep quality. Repeated polysomnography after treatment showed a clear improvement in sleep architecture with higher percentages of slow wave sleep. SMA seizure could disturb nocturnal sleep and is one of the important differential diagnoses for a patient complaining of frequent arousals associated with motor disturbance during sleep.

Clinical features of OKT4+/OKT8+ adult T-cell leukemia.

Adult T-cell leukemia (ATL) has a range of clinical characteristics. Phenotypically the leukemic cells usually express the helper/inducer associated antigen OKT4 with lack of OKT8. We have observed three patients with acute ATL cytologically indistinguishable from OKT4+/OKT8- ATL but whose neoplastic cells had the unusual phenotype, OKT3+, OKT4+, OKT6-, OKT8+ OKT9+/-, OKT11+, Tac+/-, TdT-. All patients had abnormal karyotypes and antibodies against anti-ATL associated antigens as well as proviral DNA of human T-cell leukemia virus in the leukemic cells. The clinical course was complicated by skin eruptions, hypercalcemia, pulmonary infection and disseminated intravascular coagulopathy. All died of complications shortly after diagnosis. The clinical features of these patients were similar to those of OKT4+/OKT8- ATL. However, their acute course suggests that co-expression surface antigens OKT4 and OKT8 may be a sign of aggressive nature of the disease with poor prognosis.

Nodular lung disease with five year survival and unilateral pleural effusion in AL amyloidosis.

A 67-year-old female patient with biopsy proven AL systemic amyloidosis developed rapidly progressive dyspnea. Chest roentgenogram and CT scan revealed a large right pleural effusion in addition to nodular lesions with bilateral hilar lymphadenopathy. The patient's serum showed IgG lambda type monoclonal gammopathy and she also had Bence Jones proteinuria. The pleural effusion was an exudate that contained many mononuclear cells and a high concentration of protein. Cardiac function was not seriously disturbed. Except for amyloidosis, no other causes for the severe pleural effusion were found. This patient was treated with chemical pleurodesis using Picibanil and a low dose of prednisolone. Eighteen months after this treatment, her right pleural effusion did not recur. Bronchopulmonary tissues are known to be frequently involved by AL systemic amyloidosis, but a nodular pattern of pulmonary amyloid deposition and a unilateral large pleural effusion are rare clinical manifestations in this disease.