To study the epidemiology of human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in Japan, we conducted two nationwide surveys between October 1986 and March 1989. A total of 710 patients with HAM (definite HAM, 589; probable HAM, 121) were reported. Of the 589 patients with definite HAM, 69% were residents of the areas with the highest prevalence HTLV-I in Japan. To determine the importance of blood transfusion in the pathogenesis of HAM/TSP, we performed a case-control study in the Kagoshima district in southern Japan. Significantly more patients with HAM reported a history of blood transfusion (26/129, or 20%) than did subjects in a health survey of the general population (41/1,290, or 3%; odds ratio = 7.7, p less than 0.001) or than did hospitalized neurological patients (6/119, or 5%; odds ratio = 4.8, p less than 0.001). Furthermore, the cumulative percentages of the intervals between blood transfusion and the onset of the symptoms of HAM fit a lognormal curve, suggesting that transfusion was an important common exposure. Blood transfusion probably transmitted HTLV-I to the patients with transfusion-associated HAM because there was a significant decrease in the number of patients with the transfusion-associated HAM who received blood after implementation of nationwide screening of blood donors in 1986 (p = 0.004). In the first 2 years, screening the blood supply in Japan appears to have decreased the number of reported patients with HAM by 16%.

Abstract• Histological examinations of the intracerebral and retinal arteries were performed in patients who had cerebrovascular disease and in those cases who did not. Fibrinoid degeneration, fibrous nodule, and splitting, which are most frequently found in putamen, thalamus and pons, are thought to be the main changes in cerebral hemorrhage and infarction. Fibrous and fibro-hyalinoid thickenings of the retinal arteries were found mainly in the neighboring region of the optic disk, which reflects the changes of the intracerebral arteries. Hyalinoid thickening was found in the ora serrata, which does not reflect the changes of the intracerebral arteries.Our results suggest that patients with these retinal artery changes in the region near the optic disk, if moderate to severe, have an increased risk of having or incurring cerebral hemorrhage and infarction, but the arterial changes in the ora serrata do not always indicate risk of cerebral hemorrhage and infarction. Additional Key Wordscerebral hemorrhage fibrinoid degeneration cerebral infarction splitting fibrous nodule putamen pons atherosclerosis thalamus hypertension• The significance of the changes of the retinal arteries in various disorders, particularly cerebrovascular diseases, has been of interest to physicians, and controversy exists as to whether the degenerative changes of the intracerebral arteries are parallel to those of the retinal arteries. Several attempts have been made to show the relationship between the changes of these arteries. 15 However, the comparative analyses of the changes between intracerebral and retinal arteries were not sufficient.The purpose of this paper is to describe the intracerebral and retinal vascular changes in various types of cerebrovascular diseases (CVD) in the Japanese and the relationship of various degenerative changes between intracerebral and retinal arteries. MethodsHistological examinations were performed in 22 patients with cerebrovascular diseases. Of these, 11 patients (age range: 37 to 76 years with an average of 58 years) had cerebral hemorrhage. There were eight cases with cerebral infarction (age range: 62 to 85 years with an average of 73 years). The remaining three cases had subarachnoid hemorrhage, their ages being 29, 68 and 75 years. An additional 21 cases without cerebrovascular diseases (non-* Present address: Department of Neurology, Neurological Institute, Kyushu University, Faculty of Medicine, Fukuoka City, Japan 812 (reprint requests).tSecond Department of Internal Medicine, and {Department of Ophthalmology, Kyushu University, Faculty of Medicine, Fukuoka City, Japan 812. CVD) were studied (age range: 21 to 69 years with an average of 49 years). An age-matched control study was performed in 12 cases (cerebral hemorrhage in nine, and cerebral infarction in three) with an age range from 37 to 68 years (average of 57 years), and in 12 non-CVD cases, ranging in age from 37 to 69 years (average of 57 years).In each case 15 samples were taken of the basal ganglia at levels through the mamillary...

Using the polymerase chain reaction, we quantitated the amount of human T-lymphotropic virus type I (HTLV-I) proviral DNA in peripheral blood mononuclear cells from 18 patients with HTLV-I--associated myelopathy/tropical spastic paraparesis; 17 HTLV-I carriers without HTLV-I--associated myelopathy/tropical spastic paraparesis, with or without other autoimmune or inflammatory diseases; and 19 seronegative control subjects. The HTLV-I proviral DNA was 10- to 100-fold higher in the patients and in the HTLV-I carriers without HAM/TSP who had autoimmune or inflammatory diseases than in the carriers without autoimmune or inflammatory diseases. The patients who had had onset of myelopathy at a younger age (15 to 39 years) had an extremely high level of HTLV-I proviral DNA in the early phase, as compared with findings in those with a late onset of myelopathy (at 44 to 61 years). The large increase in HTLV-I proviral DNA in peripheral blood mononuclear cells is presumably closely related to the development of autoimmune or inflammatory processes in HTLV-I carriers, including HTLV-I--associated myelopathy/tropical spastic paraparesis.

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