Measurement of various parameters of supra- and infratentorial brain atrophy in computerized tomographs of 142 cases of spinocerebellar degeneration (SCD) and 100 age & sex matched controls was carried out in order to investigate whether these parameters would correspond to the subtypes of this disease and differing grades of various clinical manifestations. One supra- and all infratentorial parameters of SCD showed statistically significant atrophy with a risk of P<0.005. Among the subtypes, OPCA had a more severely atrophied pons than LCCA (P<0.005), Menzel (P<0.05) and SSP (P<0.01). There was a correlation between the distribution of symptoms like gait, speech, ataxia of extremities and ocular movement disorders, and distribution and degree of infratentorial atrophy with statistical significance (P<0.05 - P<0.005), The degree of atrophy of the pons and the width of the IV ventricle were directly proportional to the duration of the illness in cases of less than 10 years, but not to those of over 10 years. Follow-up CT scan was done for 24 patients, 12 within 3 years, 12 after the lapse of 3 years. The latter group showed statistically significant atrophy between the 1st and 2nd scans in several parameters, but there was no significance between those of the former group.

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Five cases of rheumatoid arthritis (RA), two cases of systemic lupus erythematosus (SLE), a case of mixed connective tissue disease (MCTD), and a case of cold urticaria were treated with double filtration plasmapheresis (DFP). Each aliquot of plasma was obtained at three different points of the DEP circuit during the treatment and concentrations of thyroid hormones as well as thyroxine binding globulin (TBG) were measured. Despite the removal of considerable amounts of triiodothyronine (T3), thyroxine (T4), and TBG from the plasma, levels of plasma free T3 (FT3) and free T4 (FT4) before and immediately after DFP treatment were not significantly different. These results indicate that DEP therapy rarely affects plasma concentration of active thyroid hormones in patients who undergo such therapy.

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The phenotypic and functional characteristics of the cells that mediate "natural killer" (NK) cytolysis were examined by using anti-Leu 7 monoclonal antibody specific for NK cell in peripheral blood mononuclear cells (PMC) and pleural effusion mononuclear cells (PEMNC) from the tuberculous pleurisy patients. The proportion of Leu 7+ cells in PEMNC was observed significantly lower than in PMC, whether the patients were treated or not. Before treatment NK cell activity, precisely cytotoxity against K 562, in PEMNC was apparently higher than in PMC. There was little relationship between proportion of leu 7+ cells and NK cell activity. Further, the mechanism of high NK cell activity in PEMNC was analysed by coculture with a healthy control's PMC and a tuberculous pleural effusion. The NK cell activity of Leu 7+ cells was augmented by low molecular soluble factor(s).

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Various volumes of saline labelled with indocyanine green (ICG) and urea were injected into the pleural cavity of the dogs. The osmolarity of the saline was exactly adjusted to be equal to that of normally occurring pleural liquid. In all experiments ICG remained constant. This suggested that the injected saline were removed via the lymphatics. The lymphatic removal rate increased linearly with the increase in volume of injected saline. Based on the change in urea concentration in the pleural liquid, the turnover of the pleural liquid was studied. The fraction of exchange of pleural liquid is thought to be constant when the volume of the pleural liquid is small but when the volume is increased, this fraction correspondingly decreases. This method is also available in clinical cases.

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The in vitro Fc receptor function of monocytes as measured by using soluble immunecomplexes (IC) consisting of ovalbumin and ¹²⁵ I-labeled antibody to ovalbumin was examined in serum-free medium. Adherent monocytes were obtained from peripheral blood of 18 patients with systemic lupus erythematosus (SLE; 6 active, 12 inactive) and 13 normal controls. 5x10⁵ monocytes were incubated with soluble IC containing 20 ng IgG for 16 hr at 4°C, and for 4 hr and 16 hr at 37°C, and then cell-associated and acid-soluble radioactivities were counted. After 16-hr incubation at 4°C, there was no significant difference in the amount of cell-associated IC between monocytes from SLE patients and those from normal controls. After 4 hr incubation at 37°C, monocytes from patients with active SLE bound and phagocytized more IC than those from patients with inactive SLE or from normal controls (P<0.01). After 16 hr incubation at 37°C, degradation of soluble IC by monocytes from patients with active SLE greatly increased (P<0.003). These findings were discussed in relation to the possible pathophysiologic role of the mononuclear phagocyte system in SLE.

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In order to study the relationship between hyperinsulinemia and atherosclerosis, an examination was made on the relationship of hyperinsulinemia to the risk factors of atherosclerosis, using as subjects a total of 2, 927 composed of Japanese resident in Hawaii and Los Angeles and Japanese resident in Hiroshima whose age ranged from 40 to 79 and who underwent oral glucose tolerance test in our Hawaii - Los Angeles - Hiroshima medical study between 1978 and 1982. The subjects were classified into five groups of comparable size according to fasting insulin level, studied on the various risk factors of atherosclerosis in each group. The results of this study showed that the hyperinsulinemia group had in comparison with the four other groups a higher mean body mass index, mean serum total cholesterol value and mean serum triglyceride value, and further had a higher prevalence of hypertension, diabetes mellitus, ECG abnormalities, hypercholesterolemia and hypertriglyceridemia. Moreover, the mean serum HDL-cholesterol value was lower in the hyperinsulinemia group than in the other four groups. A similar examination was made with subgroups with normal body weight, and almost the same results were obtained. The above results suggest that hyperinsulinemia is rather strongly implicated in the development of atherosclerosis via a number of relationships though not specifically indicating that hyperinsulinemia independently is a risk factor of atherosclerosis.

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Serum CA19-9 levels were measured in 60 diabetic patients and 40 healthy volunteers. Serum CA19-9 concentration was correlated with hemoglobin Al (HbA1) (r=0.4368 P<0.005) and fasting plasma glucose levels (r=0.3410 P<0.01). None of the 40 healthy subjects showed elevated CA19-9 concentrations over 37 units/ml as the upper normal value. The percentage of positive serum CA19-9 levels in poorly controlled patients (fasting plasma glucose > 200 mg/dl or HbA1>13%) and moderately to well controlled patients was 50% and 10%, respectively. No correlation was found between the level of CA19-9 and those total cholesterol, and triglycerides, or the duration of diabetes. In patients who had diabetic retinopathy or persistent proteinuria, the CA19-9 concentration was significantly elevated when compared with those without these complications. It has been shown that patients with adenocarcinoma of the gastrointestinal tract have high plasma CA19-9 levels and those who have benign disease have normal CA19-9 levels. Even though diabetes mellitus is not a malignant disease, serum CA19-9 levels were increased in diabetic patients. These results indicate that HbA1 and fasting plasma glucose should be examined in patients with high CA19-9 levels.

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A pedigree of C1 inhibitor (C1 INH) deficiency associated with positive LE cell and an elevated titer of DNA antibodies and antinuclear factor (ANF) and nephropathy was presented. The proband of this family was diagnosed as having definite systemic lupus erythematosus (SLE) after a clinical course of several year since her first visit to our hospital and because of the lack of hemolytic activity of complement (CH50), in spite of the absence of idiopathic edema, C1 INH levels were 1.2 mg/dl (3.8% NHS) determined as antigen and 65 site forming unit (SFU) (5.8% NHS) determined by hemolytic assay in her blood. Her mother and brother had characteristic idiopathic edema of her face, larynx, hand and bowel and they had low levels of C1 INH of 1.8 mg/dl (5.8% NHS) and 4.8 mg/dl (15.5% NHS) respectively in their blood. On the basis of these findings, this family was diagnosed as having a pedigree of hereditary angioneurotic edema (HANE) which is supposedly an inherited autosomal positive trait. Actually, however, the proband's serological and hematological indices became positive and progressed year by year, which implies that SLE was absent for the first several years. It might be said that this interesting clinical course indicates that SLE appeared chronologically as a hereditary deficiency in one of the complement components in this case. In concurrence with the general observation that recurrent viral infections due to the deficiency of complement components are presumed to be responsible for SLE-like disease. Her levels of several kinds of anti-virus antibodies were high. Methylprednisolone helped normalize her level of C1 INH and ameliorate the clinical course.

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A case of respiratory myoclonus in a 45 year-old male was studied by electromyography. He complained of anorexia, involuntary swinging movement of the thorax and trunk, and epigastric pulsation. Electromyographic studies showed synchronous rhythmic grouped discharges at the rate of 2 to 3 Hz from the bilateral 8th to 10th intercostal muscles, the right iliocostalis lumborum muscle, and the left hemidiaphragm only during the expiratory phase. They disappeared on inspiration and forced expiration. Anesthetic block of the left phrenic nerve abolished both the voluntary and myoclonic movements of the left hemidiaphragm. Abnormal excitation of the central nervous system and possible irritation of the afferent pathways were postulated.

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A 34 year old man suffering from oral and genital ulceration with uveitis (the complete type of Behcet's syndrome) developed fluctuating radiological opacities in the right lung and showed recurrent hemoptyses. Pulmonary angiography showed multiple aneurysms and obstruction of the pulmonary arteries. After he was treated with predonisolone, the symptoms and pulmonary manifestation improved temporarily. However, he died suddenly from a massive hemoptysis. An autopsy revealed the aneurysmal rupture of right basilar branch of pulmonary artery into the right B6 bronchus. Histopathological findings in the lung showed multiple organized thrombi, inflammatory infiltration and vascular wall destruction of pulmonary arteries of various sizes. Only 47 previously resported cases of Behcet's syndrome which showed pulmonary involvements were revealed by a search of the published literature.

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A 56-year-old male was admitted to a hospital because of gastric ulcer in 1967, and by chance he was diagnosed right milk of calcium renal stone associated with hydronephrotic kidney by plain abdominal X-ray film. In 1979, he was admitted to our hospital complaining of hematuria, and died from renal pelvic cancer on the 49th hospital day. Milk of calcium renal stone associated with hydronephrotic kidney is rare, with only 13 cases were reported previously. This is the first report, a case of milk of calcium renal stone associated with hydronephrosis died from renal pelvic cancer.

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A case of familial hyperlipidemia incidentally found through a 16 year-old high school girl with type V hyperlipoproteinemia and abdominal bouts consistent with this type of hyperlipemia is reported for the first time in Japan. The laboratory findings of the plasma of her father revealed typical hyperlipoproteinemia of type IIa. Nineteen of her 26 kindred were investigated. Type V was seen only in the proband, type Ila in father, paternal grandmother, two paternal aunts, and two paternal cousins, type IV in three paternal cousins. The serum apolipoprotein (apo A-I, A-II, B, C-II, C-III, and E) concentrations were determined by the single radial immuno diffusion technique. The apolipoprotein concentrations were not different from those of normolipidemic control subjects except for apo B, which was higher in the hyperlipidemic members, and apo C-II, C-III, and E, which were higher in the proband.

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A 58-year-old female who had a serum cholinesterase activity four times higher than normal is described. Investigation of her family revealed that five out of seven tested had high level of serum cholinesterase activity. The family distribution of the increased cholinesterase suggests that the inheritance is transmitted in a autosomal dominant manner. Analysis of the isoenzyme of serum cholinesterase of five cases including the patient showed that none of them had extracomponent (C5) of the cholinesterase. Examination of anti-cholinesterase antibodies in her serum was negative. Molecular weight of her serum cholinesterase by HPLC analysis was not different from that of a healthy subject. It was speculated that the overproduction of usual components (Cl, C2, C3, C4), decreased clearance of cholinesterase by a certain mechanism(s), and/or the presence of cholinesterase which has more active catalysis could be responsible for her and her family's hypercholinesterasemia.

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Nephrotic range proteinuria occurred in a 42-year-old woman with renal arterial occlusion and hyperreninemia. The administration of captopril, an angiotensin converting enzyme inhibitor, led to an amclioration of the proteinuria and the decrease of blood pressure, without surgical treatment. From the present observation, it is highly probably that the increased activity of the renin-angiotensin system plays an important role in massive proteinuria. Conservative treatment for renovascular hypertension with nephrotic syndrome was effective in this patient.

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