Marchiafava-Bignami disease treated by mianserin hydrochloride in short-term evaluated by neuropsychological analysis

Since 1903, Marchiafava‐Bignami disease has been recognized as a rare syndrome with focal demyelination and necrosis in the corpus callosum, which is usually found in chronic alcoholics. It extends into the neighboring white matter and occasionally as far as the subcortical regions. We report a Japanese patient with Marchiafava‐Bignami disease associated with alcohol abuse, who had traveled around Western Europe, North America and China for more than 30 years. As he suffered extreme delirium in the early stages we administered a low dose (10 mg) of mianserin hydrochloride. He was very irritable and uncooperative on admission, after 20 days his delirium had disappeared and his temper had become very calm and mild. After 40 days, his intelligence level increased substantially as measured by various neuropsychological tests.     

PREVALENCE OF RAYNAUD'S PHENOMENON AND SPECIFIC CLINICAL SIGNS RELATED TO PROGRESSIVE SYSTEMIC SCLEROSIS IN THE GENERAL POPULATION OF JAPAN

Background. In order to evaluate the prevalence rates of Raynaud's phenomenon (RP) and specific clinical signs related to progressive systemic sclerosis (PSS) in the general population of Japan, inquiries were made concerning RP in the hands and dermatologic examinations were also conducted. Methods. One thousand and sixty-three subjects (332 men and 731 women) over 30 years of age who underwent inhabitants' health examinations in 1990 were considered for this study. Results. The prevalence of RP was 3.0% in men and 3.4% in women. In 8 men and 17 women with RP who received the blood tests, the positive rates of antinuclear antibody (ANA) were 12.5% and 35.3% in men and women, respectively. The prevalence rates of all five specific clinical signs related to PSS, sclerodactyly, pitting scars of the fingers, brown pigmentation of the body, shortened frenulum of the tongue, and flexion contracture of fingers, were under 2% in men and 3% in women. In women with RP the prevalence rates of sclerodactyly, pitting scars of the fingertips, brown pigmentation of the body, and shortened frenulum of the tongue were 16.0, 4.0, 4.0, and 16.0%, respectively. These values were significantly higher than those of persons without RP. Conclusions. Because some persons with primary RP may become typical cases of PSS within several years, a follow-up study, particulary for women who have positive titers of ANA with RP, should be carried out to find out whether the persons suffer from PSS or not.     

Expression of Sialylated LewisGangliosides in Cultured Lens Epithelial Cells from Rhesus Monkey

Monkey and human lenses contain essentially the same glycosphingolipids, and Lewis^xand sialylated Lewis^xepitopes are expressed on the terminal structure of neolactotetraosylceramide. However, monolayer cultures of lens epithelial cells from rhesus monkey expressed gangliosides GM3, GD3 and a small amount of GM1, but not sialylated Lewis^xepitopes. Eight-week-old cultures on various extracellular matrices resulted in morphological changes in lens epithelial cells. Monolayer of cells cultured on vitronectin or polylysine assembled into aggregates after 4 weeks of culture. Cells cultured on vitronectin expressed sialyl-Lewis^xgangliosides and did not exhibit GD3. On collagens, fibronectin and laminin elongated cells were observed in cells cultured for 8 weeks. Thus, the interaction between cells and extracellular matrices influenced morphology and glycosphingolipid composition in lens epithelial cells.     

Small renal oncocytoma with central cystic degeneration

A case of a small renal oncocytoma with central cystic degeneration, 15 mm in diameter, is reported. Contrast-enhanced computed tomography showed the tumor contained a central hypoattenuating region and had an irregular, heterogeneously enhanced wall. Magnetic resonance images showed a well-circumscribed lesion and the T(1)-weighted image indicated medium signal intensity, whereas the T(2)-weighted image indicated slight hypointensity. Both T(1)- and T(2)-weighted images showed central hyperintensity. Our preoperative diagnosis was renal cell carcinoma originating in a renal cyst wall or cystic renal cell carcinoma. Nephrectomy was performed because frozen-section examination did not completely rule out malignancy. The final pathological diagnosis of the entire surgical specimen was renal oncocytoma with cystic degeneration. To our knowledge, this is the 14th case of renal oncocytoma with central cystic degeneration reported in the published works. We discuss herein the variant forms of oncocytoma and difficulties with their preoperative diagnosis, especially when the tumor is small.     

Hepatic HCV-RNA as a predictor of outcome after interferon therapy in patients with chronic hepatitis C

Measurement of serum HCV-RNA is a useful index for evaluating the antiviral effect of interferon therapy in chronic hepatitis C. In the present study, we investigated whether the detection of hepatic HCV-RNA after interferon treatment, using a polymerase chain reaction assay, predicted long-term response to therapy in patients with chronic hepatitis C. Thirty-three patients underwent liver biopsies before and after interferon therapy. Histology and clinical courses were compared after treatment. Before therapy, serum and hepatic HCV-RNA was detected in specimens from 32 (97%) and 33 (100%) patients, respectively. Serum HCV-RNA became undetectable in samples from 22 (67%) patients; however, in 10 of these patients (45%), serum HCV-RNA levels relapsed after therapy. Hepatic HCV-RNA became undetectable in 14 patients after therapy and the serum aminotransferase concentration remained within normal limits during and following (24–92 weeks) therapy in 12 of these patients (86%). All 11 patients with detectable hepatic HCV-RNA also had serum HCV-RNA and elevated aminotransferase concentrations refractory to therapy. The absence of hepatic HCV-RNA at the end of interferon treatment thus predicted a long-term complete response to therapy with a sensitivity of 100%, a specificity of 90% and an accuracy of 94%. We conclude that hepatic rather than serum HCV-RNA is a more useful index for the prediction of the long-term efficacy of interferon therapy.     

"Trench Ulcer" of the Stomach

The clinical, endoscopic and histological findings of 14 cases with unusual gigantic ulcers of the stomach were reported. The patients were mainly aged, complicated by cardiovascular diseases. The ulcer was designated as "trench ulcer" from the characteristic endoscopic features, which ran from the cardiac portion to the angle along the lesser curvature.     

Pancreatic Arteriovenous Malformation in a Patient with Gastrointestinal Hemorrhage

A 39-year old woman, with a pancreatic arteriovenous malformation (AVM) which ruptured into the pancreatic duct, was treated successfully with distal pancreatectomy and splenectomy. Histological examinations revealed an AVM with extravasation into the pancreatic ducts. A review of the cases reported to date emphasizes the importance of physical signs, individual histories and angiographic studies for early diagnosis and surgery.     

FK506 promotes liver regeneration by suppressing natural killer cell activity

We examined the mechanism of promotion of liver regeneration by tacrolimus hydrate (FK506), a potent immunosuppressant, after partial hepatectomy. The administration of FK506 significantly increased the bromodeoxyuridine (BrdU) labelling index at 36 and 48 h after 70% hepatectomy compared with the placebo group. Using the same model, we examined the effect of FK506 on the expression of hepatocyte growth factor (HGF) and transforming growth factor-β1 (TGF-β1) and found no changes in HGF and TGF-β1 mRNA expression in the liver or in the HGF protein concentration in plasma. We found that pretreatment with FK506 markedly reduced the activity and number of liver-resident natural killer (NK) cells at the time of partial hepatectomy. Our observations suggest that the promotion of liver regeneration by FK506 may be attributable to a reduction in the number of liver-resident NK cells and to inhibition of their activity.