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.     

Adequacy of Pacing Rate During Exercise in Rate Responsive Ventricular Pacing

Our objective was to determint; the adequate pacing rate during exercise in ventricular pacing by measuring exercise capacity, cardiac output, and sinus node activity. Eighteen patients with complete AV block and an implanted pacemaker underwent cardiopulmonary exercise tests under three randomized pacing rates: fixed rate pacing (VVJ) at 60 beats/min and ventricular rate-responsive pacing (VVIR) programmed to attain a heart rate of about 110 beats/min ar 130 beats/min (VVIR 110 and VVIR 130, respectively) at the end of exercise. Compared with VVI and VVIR 130, VVIR 110 was associated with an increased peak oxygen uptake(VVIR 110:20.3 ± 4.5 vs VVI: 16.9 ± 3.1; P < 0.01; and VVIR 130: 19.0 ± 4.1 mL/min per kg, respectively; P < 0.05) and a higher oxygen uptake at anaerobic threshold (15.3 ± 2.7, 12.7 ± 1.9; P < 0.01, and 14.6 ± 2.6 mL/min per kg; P < 0.05). The atrial rate during exercise expressed as a percentage of the expected maximal heart rate was lower in VVIR 110 than in VVI or VVIR 130 (VVIR 110: 75.9%± 14.6% vs VVI: 90.6%± 12.8%; P < 0.01; VVIR 110 vs VVIR 130: 89.1%± 23.1%; P < 0.05). There was no significant difference in cardiac output at peak exercise between VVIR 110 and VVIR 130. We conclude that a pacing rate for submaximal exercise of 110 beats/min may be preferable to that of 130 beats/min in respect to exercise capacity and sympathetic nerve activity.     

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.     

A Case of Pachydermoperiostosis with Watery Diarrhea,Giant Gastri Rugae,and Endocrine Disorder

Abstract: A 31-year-old man, cook, who had had persistent watery diarrhea for about a month visited our hospital. He had already been diagnosed as having pachydermoperiostosis. An examination of the upper gastrointestinal tract revealed that he had giant gastric rugae. The histology was compatible with hypertrophic gastritis accompanied with marked hyperplasia of the fundic gland and foveolar epithelia. The output of gastric juice was high in volume, although its acidity normal. The barium transit time of the small intestine was reduced to 15 minutes. His diarrhea was, thus, considered to be induced by gastrointestinal hyperfunction. The patient's glucose tolerance was abnormal and basal Cortisol secretion level was high. Pachydermoperiostosis has been the focus of attention for skin and bone changes, and the frequency of this disease with endocrine disorders seems rather high, but accompanying gastrointestinal disorders have so far not often been reported in Japan. The results of our investigation strongly suggest that pachydermoperiostosis is a systemic disease.     

Laparoscopic Follow-up of Asymptomatic Primary Biliary Cirrhosis with Negative Anti-mitochondrial Antibody

Abstract: The case of a 59 year-old female with asymptomatic primary biliary cirrhosis with negative anti-mitochondrial antibody is presented. According to the results of the first laparoscopy and liver biopsy, reddish patch was observed but chronic non-suppurative destructive cholangitis could not histologically be confirmed. A follow-up laparoscopy with liver biopsy conducted 39 months after the first laparoscopy revealed progress in the laparoscopic findings, i. e. a focal appearance of mesh-like white marking and undulation, when chronic non-suppurative destructive cholangitis was histologically demonstrated. Following the diagnosis of primary biliary cirrhosis, ursodeoxycholic acid treatment was begun, and the patient's serum levels of transaminases and biliary tract enzymes showed a rapid improvement. The importance of a laparoscopy with liver biopsy for the diagnosis of asymptomatic primary biliary cirrhosis in a case with negative anti-mitochondrial antibody is emphasized, and the follow-up of such a case discussed.     

Anti-Ro/SSA-associated annular erythema in childhood

Summary We report lour children with anti-Ro/SSA positive who presented with an annular erythema as a manifestation of Sjögren's syndrome. One patient had an aseptic meningoencephalitis as well as cutaneous lesions. Children with annular erythema should he carefully followed for features of Sjögren's syndrome, which is believed to be a rare condition in children.     

A Case of Gallbladder Perforation in Which the Developmental Pattern was Demonstrated by Laparoscopy

Abstract: A 58-year-old man with diabetes mellitus was referred to our clinic because of epigastric colicky pain of sudden onset with fever. An ultrasonography (US) and endoscopic ultrasonography (EUS) demonstrated marked thickening of the gallbladder wall and a pericholecystic echo-free space. A laparoscopy showed tight adhesion between the greater omenturn and the parietal peritoneum, and pooling of bile on the liver surface, the greater omenturn, and in the perisplenic area. The gallbladder itself could not be seen. A surgical laparotomy revealed perforation of the gallbladder with a pericholecystic abscess. No laparoscopic observation of free bile has been reported in cases of gallbladder perforation. In the present case, US, EUS and laparoscopy were useful for early diagnosis of gallbladder perforation. In addition, laparoscopy played an important role in determining the type of gallbladder perforation.     

Night-time hypnopompic visual hallucinations related to REM sleep disorder

Abstract We encountered three patients who had experienced hypnopompic visual night-time hallucinations. Their clinical manifestations resembled Charles Bonnet's syndrome and the content of their experiences were understood as attempts at wish fulfillment. However, abnormal REM findings were recognized on polysomnogram at the occurrence of visual hallucination in two cases. We speculated that dysfunction of REM sleep mechanism might contribute to the night-time occurrence of such kind of visual hallucination and that their visual experiences might be reflected by dream content.