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Ventricular septal perforation( VSP) leads to a high mortality rate after surgical treatment. The surgical procedure has not been established. Left ventricular (LV) incisions have mainly been performed, while we report a case of right ventricular (RV) approach that resulted in a favorable outcome in a 76-year-old male. The patient was diagnosed with myocardial infarction due to left anterior descending artery (LAD) occlusion. VSP was diagnosed after percutaneous coronary intervention (PCI) and surgery was performed on the 4th day of illness. The perforation site was identified near the anterior septum by epicardiac echography before incision, and a patch made of 3 layers using a pericardial patch, felt, and a Dacron patch was sewn on the perforation with a sandwich technique and closed with bio glue. The RV approach is a useful procedure because it avoids the hemostatic manipulation of left ventricle myocardial necrosis under high pressure and can preserve left cardiac function.

A 67-year-old man visited our hospital with an enlarging abdominal mass several months after he had first noticed his symptoms. An elastic firm tumor was palpated on the left side of the abdomen upon physical examination. The blood test results were normal. Contrast-enhanced computed tomography of the abdomen revealed a 10-cm-diameter homogeneous low-density cystic tumor located at the dorsal portion of the gastric corpus. Enhancement of a few net-like structures was noted, but most of the lesion was not enhanced. Gastroendoscopy revealed the lesion to be a submucosal tumor with a smooth mucosal surface and no ulceration. Endoscopic ultrasonography showed the tumor arising from the fourth layer of the gastric wall. The tumor was completely resected by laparotomy and partial gastrectomy. It was capsulated and contained serous fluid with little solid tissue. Histologically, there were sparse tumor cells within the myxoid interstitium. Immunostaining results were weakly positive for KIT and CD34-positive accompanied by mast cell infiltration. A platelet-derived growth factor receptor alpha (PDGFRA) exon 18 (D842V) mutation was identified, and the lesion was ultimately diagnosed as myxoid epithelioid gastrointestinal stromal tumor (GIST) of intermediate- and low-risk according to Fletcher's classification and Miettinen's classifications, respectively. GISTs with PDGFRA D842V mutations are reportedly resistant to imatinib, and GISTs originating from the stomach are reportedly less malignant than others. The patient was observed without adjuvant therapy after surgery because of the relatively low risk of metastasis or recurrence and the potential risk of imatinib resistance. No recurrence was observed for ≥5 years after the surgery. We herein report this rare case and describe its clinical characteristics.

A 72-year-old man presented with left scrotal swelling. The patient was diagnosed with left testicular hydrocele and underwent the hydrocele aspiration. However, it recurred within a short period. Magnetic resonance imaging (MRI) revealed a tumor in the epididymis and the patient then underwent left high orchiectomy. The histopathological examination revealed an adenocarcinoma of the epididymis. Ten months after the surgery, the patient has been free of disease.

A 30-year-old man who had a painless mass in the right scrotum since 5 years ago underwent biopsy followed by right high orchiectomy. The tumor originating from the epididymis was histologically diagnosed as a malignant lymphoma (a diffuse large B-cell lymphoma). The tumor grew very slowly even though the MIB-1 index was more than 90%. The patient underwent six courses of R-CHOP chemotherapy, intrathecal administration of methotrexate, and radiation therapy to the contralateral testis. Currently, 10 months after surgery, the patient has shown no clinical evidence of recurrence.

We report a case of percutaneous bacillus Calmette-Guérin (BCG) perfusion therapy for carcinoma in situ (CIS) of upper urinary tract after radical cystectomy with ileal neobladder. A 42-year-old man underwent radical cystectomy and ileal neobladder diversion due to the recurrence of CIS in prostatic urethra after transurethral resection of bladder tumor 3 times and 2 courses of intravesical BCG therapy. Final pathological findings showed the presence of CIS in the right distal ureteral margin. After the radical cystectomy, our diagnosis was CIS in the right residual ureter, because of positive urine cytology and negative radiographic findings in the upper urinary tract. We performed the percutaneous BCG perfusion therapy for CIS of the right upper urinary tract after the construction of the percutaneous nephrostomy by intentionally inducing hydronephrosis. No recurrence was found after 3 years of BCG perfusion therapy.

A 87-year-old man was diagnosed with prostate cancer (cT2aN0M0 Gleason score 4＋4 with initial prostate specific antigen of 23.4 ng/ml). Prostate cancer was treated with combined androgen blockade (goserelin acetate plus flutamide). He was administered goserelin acetate depot injection without any complications as an outpatient. However, 5 hours after he left the hospital, he came back to the hospital, complaining of lower abdominal pain. Abdominal computed tomography revealed a giant subcutaneous hematoma in the lower abdomen. Hemoglobin was 6.9 g/dl and blood pressure was lower than 80 mmHg. He was admitted and given a blood transfusion. Because of pre-disseminated intravascular coagulation score 6, it was hard to antagonize warfarin by Vitamin K (he had taken warfarin because of atrial fibrillation). Arteriography was performed and injury to a branch of the lower epigastric artery was found. Transcatheter arterial embolization was performed at the same time. Injecting goserelin acetate may cause severe arterial injury.

We report the case of a 57-year-old woman, known to have had allergic sinusitis and bronchial asthma, and had been treated with leukotriene receptor antagonist for one month, at the time of presentation. She complained of bilateral weakness and pain in her lower extremities and her laboratory investigations revealed an elevated absolute eosinophil count of 23,357/μl, elevated biliary enzymes and an IgG4 level of 594 mg/dl. Nerve conduction study revealed mononeuritis multiplex. She was therefore diagnosed with Churg-Strauss syndrome and started on pulse steroids and intravenous immunoglobulins. Her symptoms were alleviated, her absolute eosinophil count and biliary enzymes both improved at the same time and her IgG4 level also decreased. Although elevated biliary enzymes occurring with CSS was thought to be due to a bile duct eosinophilic granuloma, we cannot rule out the possibility of IgG4-related sclerosing cholangitis, because of her elevated IgG4.

We report two cases of prostatic cancer invading the rectum with circumferential rectal wall thickening. In both cases, reddish and uneven edematous mucosa was observed by colonoscopy and the boundary of the lesion was indistinct. No mass or ulcer formation was not observed. CT scan showed irregular prostatic swelling and circumferential rectal wall thickening adjacent to the prostate. The rectal biopsy specimen showed moderately to poorly differentiated adenocarcinoma, and it was positive on PSA immunostaining. Although rectal invasion of prostatic cancer is relatively rare, it is important to be aware that prostatic cancer can cause circumferential stenosis of the rectum.

We report a case of a 64-year-old Japanese man with adenoid cystic carcinoma. An elevated lesion covered by intact epithelium in the thoracic esophagus was found in September, 2007 and been followed. After dysphagia appeared follow-up endoscopy was performed in January, 2010, and morphological change into a protruding tumor was recognized. Since adenoid cystic carcinoma was detected by endoscopic biopsy, the patient underwent esophageal resection. The resected specimen showed a cribriform pattern and a certain amount of mucous substance which was positive for Alcian blue, within a solid nest. The tumor cells were positive for S-100 protein and negative for αSMA, so the tumor was diagnosed as an adenoid cystic carcinoma.

We report the case of a 15-year-old Japanese boy with eosinophilic gastroenteritis. The patient complained of abdominal pain and watery diarrhea and had a history of allergic rhinitis. Laboratory data on admission showed leukocytosis with remarkable eosinophilia. Microscopic examination of the biopsied specimens taken from the esophagus, stomach, duodenum, lower ileum and colon showed eosinophilic infiltration. Especially in the lower esophagus, there was a ring-like discoloration with remarkable eosinophil infiltration. We diagnosed eosinophilic gastroenteritis and his clinical symptoms and eosinophilia improved following starting corticosteroid therapy. After 5 months therapy with prednisolone, discoloration of upper digestive tract disappeared. There have been no reports describing discoloration in the lower end of the esophagus seen by gastroscopy.

A 70-year-old woman presented with abnormal liver function test 3 months after the introduction of daily intake of raloxifene, a selective estrogen receptor modulator, for osteoporosis. She had a history of NAFLD, diabetes mellitus and depression. The ratio of the computed-tomographic value of the liver against that of the spleen decreased remarkably. Pathological examination of the liver biopsy revealed severe steatosis. We thought her NAFLD was aggravated by raloxifene and discontinued the drug. The liver function test results improved and the computed tomographic liver-spleen ratio rose. This is the second case report of NAFLD becoming aggravated after treatment with raloxifene. It still remains unclear how raloxifene affects liver. Monitoring liver function is recommended when treating with raloxifene.

It is widely accepted that the Babinski reflex is the most well-known and important pathological reflex in clinical neurology. Among many other pathological reflexes that elicit an upgoing great toe, such as Chaddock, Oppenheim, Gordon, Schaefer, and Stransky, only the Chaddock reflex is said to be as sensitive as the Babinski reflex. The optimal receptive fields of the Babinski and Chaddock reflexes are the lateral plantar surface and the external inframalleolar area of the dorsum, respectively. It has been said that the Babinski reflex, obtained by stroking the sole, is by far the best and most reliable method of eliciting an upgoing great toe. However, the Chaddock reflex, the external malleolar sign, is also considered sensitive and reliable according to the literature and everyday neurological practice. The major problems in eliciting the Babinski reflex by stroking the lateral part of the sole are false positive or negative responses due to foot withdrawal, tonic foot response, or some equivocal movements. On the other hand, according to my clinical experience, the external inframalleolar area, which is the receptive field of the Chaddock reflex, is definitely suitable for eliciting the upgoing great toe. In fact, the newly proposed method to stimulate the dorsum of the foot from the medial to the lateral side, which I term the "reversed Chaddock method," is equally sensitive to demonstrate pyramidal tract involvement. With the "reversed Chaddock method", the receptive field of the Chaddock reflex may be postulated to be in the territory of the sural nerve, which could be supported by the better response obtained on stimulation of the postero-lateral calf than the anterior shin. With regard to the receptive fields of the Babinski and Chaddock reflexes, the first sacral dermatome (S1) is also considered a reflexogenous zone, but since the dermatome shows marked overlapping, the zones vary among individuals. As upgoing toe responses are consistently observed in the case of pyramidal tract dysfunction by both the Chaddock reflex and the reversed Chaddock method, the external inframalleolar area of the dorsum as a reflexogenous zone is considered very important. In conclusion, this method, termed the "reversed Chaddock method," is not only effective but can also help avoid false positive results due to foot withdrawal or tonic foot responses elicited on stroking the sole. I would like to propose the "reverse Chaddock sign (Tashiro sign)" as one of the 15 pathological reflexes involving the foot.

Because embryonic stem (ES) cells and induced pluripotent stem (iPS) cells can differentiate into various types of cells in vitro, they are considered as a valuable model to understand the processes involved in the differentiation into functional cells as well as an unlimited source of cells for therapeutic applications. Efficient gene transduction method is one of the powerful tools for the basic researches and for differentiating ES and iPS cells into lineage-committed cells. Recently, we have developed an adenovirus (Ad) vector for efficient transduction into ES and iPS cells. We showed that Ad vectors containing the cytomegalovirus enhancer/β-actin promoter with β-actin intron (CA) promoter or the elongation factor (EF)-1α promoter were the appropriate for the transduction into ES and iPS cells. We also found that enforced expression of a PPARγ gene or a Runx2 gene into mouse ES and iPS cells by an optimized Ad vector markedly augmented the differentiation of adipocytes or osteoblasts, respectively. Thus, a gene transfer technique using an Ad vector could be an advantage for the regulation of stem cell differentiation and could be applied to regenerative medicine based on ES and iPS cells.

Induced pluripotent stem (iPS) cells, which are generated from somatic cells by transducing four genes, are expected to have broad application to regenerative medicine. Although establishment of an efficient gene transfer system for iPS cells is considered to be essential for differentiating them into functional cells, the detailed transduction characteristics of iPS cells have not been examined. By using an adenovirus (Ad) vector containing the cytomegalovirus enhancer/beta-actin (CA) promoters, we have developed an efficient transduction system for mouse mesenchymal stem cells and embryonic stem (ES) cells. Also, we applied our transduction system to mouse iPS cells and investigated whether efficient differentiation could be achieved by Ad vector-mediated transduction of a functional gene. As in the case of ES cells, the Ad vector could efficiently transduce transgenes into mouse iPS cells. We found that the CA promoter had potent transduction ability in iPS cells. Moreover, exogenous expression of a PPARγ gene or a Runx2 gene into mouse iPS cells by an optimized Ad vector enhanced adipocyte or osteoblast differentiation, respectively. These results suggest that Ad vector-mediated transient transduction is sufficient to promote cellular differentiation and that our transduction methods would be useful for therapeutic applications based on iPS cells.

A 50-year-old man had been treated for hemorrhagic duodenal ulcer. Although the patient was treated by proton pump inhibitors (PPI), the ulcer did not show any sign of improvement. Therefore, we also examined the pancreas, and obtained CT findings suggestive of arteriovenous malformation (AVM) from the head to the body of the pancreas. Pancreatic AVM was diagnosed based on angiography. He was readmitted for high fever and abdominal pain. On day 2, CT demonstrated a cystic lesion containing gas at the pancreatic head, and CT showed pneumobilia on day 15. The next day, pancreaticoduodenectomy was carried out and a common bile duct-duodenal fistula was confirmed. This report describes a rare case of pancreatic AVM complicated with a common bile duct-duodenal fistula.

To clarify the whole picture of epidermal growth factor (EGF) signaling pathway, we identified proteins from the EGF-stimulated A431 cells by anti-phospho-tyrosine antibody column chromatography. Over 150 proteins were detected including previously unidentified proteins as well as well-studied proteins. Among these proteins, we picked up four proteins that had not been known in EGF signaling pathway and analyzed their functions. We report the functions of these proteins in this article. 1) CFBP interacts with CD2AP family proteins and functions as a key component in downregulation of EGF receptor protein level following EGF stimulation. 2) Ymer is found to be phosphorylated and ubiquitinated upon EGF stimulation, and functions as a regulator for the downregulation and endocytosis of EGF receptor. 3) CLPABP binds to mitochondria-specific phospholipids, cardiolipin, through its PH domain, and its complex includes various proteins related to mRNA metabolism. 4) GAREM is associated with Grb2 and Shp2. Each association affects the ERK activity. Finally, we discuss the possibilities that these proteins can be used as a novel biomarker protein for cancer and other diseases.

Carcinoid tumors are low-grade malignant tumors that arise from neuroendocrine cells. Primary renal carcinoid tumors are extremely uncommon. A 63-year-old woman presented with a right abdominal mass and fever. Abdominal computed tomography demonstrated a mass in the right kidney; the mass measured 120 mm in diameter and showed hemorrhage. The patient underwent an uneventful right radical nephrectomy, and histological appearance was typical of carcinoid tumor. Immunohistochemistry demonstrated strong cytoplasmic labeling for neuron-specific enolase and synaptophysin. Additional examinations of the gastrointestinal tract did not show any evidence of carcinoid tumors. The patient remains free from disease recurrence at 8 months after the operation. The prognosis for primary renal carcinoid tumor is relatively optimistic. Complete surgical excision is the only recommended treatment for localized renal carcinoid tumor.

A 74-year-old woman was admitted to our hospital with recurrent massive lower gastrointestinal bleeding. She had a history of type C liver cirrhosis and appendectomy, and had undergone endoscopic ligation of esophageal varices one year before. Three-dimensional CTA revealed ileal varices in the right lower quadrant of the abdomen. Superior mesenteric arteriography demonstrated varices at the corresponding area and collateral veins from the superior mesenteric vein to the right ovarian vein. Ileal varices were diagnosed and ileal resection was performed. At surgery, exposed vessels were present at the mucosal surface of the resected specimen and they were thought to be the origin of hemorrhage. In conclusion, bleeding from small intestinal varices, though uncommon, should be considered when the origin of melena is unidentified in a patient with liver cirrhosis.

A 28-year-old woman with ascites was admitted to our hospital. We diagnosed peritonitis carcinomatosa caused by colon cancer complicated by ulcerative colitis. We performed peritoneal tap and infusion of mitomycin C, and administered 5-fluorouracil. Her clinical status gradually worsened, and she died 5 months later. At autopsy, the histological examination showed many mucinous adenocarcinoma and signet ring cell carcinoma with dysplasia. There were also some areas of squamous cell carcinoma with squamous metaplasia and dysplasia far from rectum. Squamous cell carcinoma and adenosquamous cell carcinoma of the colon are rare complications of ulcerative colitis. We reported this case as an addition to the literature on the subject.

We reported a 20-year-old woman with fibromuscular dysplasia (FMD) of the basilar artery presenting multiple cerebral infarctions. A sudden onset of consciousness disturbance and right hemiparesis was experienced. A neurological examination on day 2 revealed an absence of light and corneal reflexes on the left side, homonymous left upper quadrant anopsia and right hemiparesis with Babinski sign: she was also somnolent. On head MRI, multiple high signal intensity lesions were seen in the right occipital lobe, bilateral thalami and left pons on T2- and diffusion weighted images. Brain angiogram revealed the string of beads sign of the basilar artery, suggesting FMD. Neurological deficits gradually improved in the 2 months that followed, leaving slight hemiparesis and homonymous left upper quadrant anopsia In the following 3 years, no recurrence was seen with aspirin (81 mg/day). FMD in the head and neck usually affects extracranial segments of the carotid and vertebral arteries, while FMD of the basilar artery is extremely rare. To the best of our knowledge, 12 cases with FMD of the basilar artery have been reported; of these, 11 were symptomatic and 5 died. Since FMD of the basilar artery has poor prognosis, attention needs to be paid for FMD in young adults as a differential diagnosis of cerebral infarction in the territory of the basilar artery.

A 26-year-old woman with heterotopic pregnancy in the 8th week of gestation was scheduled for surgery for ectopic pregnancy. To reduce the effect on the intrauterine pregnancy, awake laparoscopic surgery was performed under spinal anesthesia with pneumoperitoneum pressure of 6 mmHg. Bilateral shoulder pain occurred when pneumoperitoneum pressure was increased to 8 mmHg. The pain disappeared after the pneumoperitoneum pressure was decreased to 6 mmHg. Attention should be paid to shoulder pain due to physical and chemical stimulation of the diaphragm by pneumoperitoneum. Postoperative pregnancy and delivery were uneventful.

A 65-year-old female presented to our hospital with a 6-month history of pollakuria, low-grade fever and urgent incontinence. Cystoscopy revealed a nonpapillary bladder tumor that was 50 mm in diameter in the trigon. Computed tomography showed the abscess between the urinary bladder and sigmoid colon. Transurethral resection was performed and the histology consisted of inflammatory lesions with inflammatory cell infiltration, which was diagnosed as an inflammatory pseudotumor due to diverticulitis of the sigmoid colon. Sigmoidectomy was subsequently performed. A fistula between the urinary bladder and the sigmoid colon was not detected. Cystoscopy 2 months after the operation revealed no signs of a bladder tumor.

Owing to progress of assisted reproduction technology in recent years, it has become possible for couples with infertility problems to have children. Between March 1998 and May 2003 testicular sperm extraction (TESE) was performed on 30 men with male-factor infertility in our hospital. Consequently, we succeeded in recovering 20 spermatozoa. Intracytoplasmic sperm injection was subsequently performed in 15 couples and resulted in 8 pregnancies. There was a statistically significant difference in follicle-stimulating hormone, luteirizing hormone and Johnsen's score between the non-obstructive groups with successful TESE and those with unsuccessful TESE.

We studied validity and clinical applicability of the Japanese version of amyotrophic lateral sclerosis (ALS) assessment questionnaire 40 (ALSAQ-40). The original version contains forty questions measuring five areas (domains) of health status: Physical Mobility, ADL/Independence, Eating and Drinking, Communication and Emotional Functioning. Data were obtained from 39 ALS patients and from their physicians at 15 centers in Japan. Patients completed the ALSAQ-40 and the SF-36, and provided information on their age and their status of ventilator use. Their physicians provided information on the date of diagnosis, type of disease and clinical characteristics, and ALSFRS-R. The patients' average age was 58.5 years, and 64% were men. The mean duration since diagnosis was 39.1 months. Forty four percent were classical ALS patients and 46% were receiving a respiratory intervention. Although there was much heterogeneity, the scores for Physical Mobility and ADL/Independence were higher(indicating worse health status)than the scores for the other domains. Item-scale correlations were strong, except for the item "felt embarrassed in social situations" in the Emotional Functioning domain. All the domains had very high internal consistency: Cronbach's alphas ranged 0.95 to 0.97. With regard to the cluster structure of the forty items, the Eating and Drinking domain and the Communication clustered together. The reason might be that the former consisted of only three items and both domains measure bulbar symptoms. Domain scores correlated significantly with scores of related dimensions in the SF-36 and ALSFRS-R, and did not correlate strongly with unrelated domains. The five items of the ALSAQ-5 correlated with all five domain scores on the ALSAQ-40. These results should be interpreted with caution because we analyzed together data from ALS patients with various characteristics. In conclusion, although we may need to add and remove some items and modify the wording of others, the Japanese version of the ALSAQ-40 had high validity and is likely to be useful in evaluating of QOL in ALS patients. Whether the ALSAQ-5 can be used in place of the ALSAQ-40 is a matter for further study.

Multiple lines of evidence support the role of genetic factors for susceptibility to multiple sclerosis (MS), although unknown environmental factors must play an important role in developing MS. In this paper, we review the reports studied for the possible associations between Japanese MS and candidate genes by using case-control method. As for HLA alleles, HLA-DRB1*1501 and -DPB1*0501 allele have been confirmed to be associated with conventional MS and opticospinal MS, respectively. Some polymorphisms of vitamin D receptor gene, estrogen receptor gene, CTLA-4 gene, and osteopontin gene were reported to be associated with conventional MS. Appropriate case ascertainment and collection as well as proper statistical method are needed to isolate the susceptibility genes for MS.

We report a case of signet-ring cell carcinoma of the urinary bladder. A 48-year-old female was hospitalized because of general fatigue, pollakiuria and residual sensation. Renal ultrasonography disclosed bilateral hydronephrosis. The serum level of cretinine was 3.1 mg/dl, and we diagnosed the patient with post-renal failure. Cystoscopic examinations revealed non-papillary sessile tumors in the trigone. Histopathological findings of the biopsy specimen demonstrated signet-ring cell carcinoma. There was no evidence indicating bladder metastasis from the cancer in other organs. The patient died of cachexia 5 months after the diagnosis. She was autopsied, and the diagnosis of signet-ring cell carcinoma of the urinary bladder with metastases in various organs was confirmed. This disease is extremely rare and has a poor prognosis. We review the previous cases reported in the literature.

The purpose of this study was to investigate the age-related working memory(WM) decline in patients with Parkinson's disease(PD) using Baddeley's WM model. This model consists of the central executive (CE) and two slave systems, the phonological loop (PL) for the storage of verbal materials, and the visuospatial sketchpad(VSSP) for the storage of visuospatial information. The participants of this study were 22 PD, 11 old (age of 68-78, mean age, 70.5) and age of onset, duration of illness, medication time, and Yahr stage, global cognitive status-matched 11 young(age of 39-58, mean age, 51.5) PD, age- and educational years, global cognitive status-matched 22 normal control (NC), 11 old(age of 65-78, mean age, 70.4) and 11 young(age of 45-57, mean age, 52.4). Mental calculation span of digit sequences, digit forward and backward span, visual memory span were carried out. Age related decline of WM was found in both groups, but processing related differences were revealed between the two groups. NC group showed significant decline with aging in digit backward span. In contrast, in mental calculation span, PD groups showed significant deficit revealed in young PD group and declined significantly with aging and significant decline was not found in digit backward span. In term of the processing and difficulty of WM tasks, digit backward span that needs maintenance of digit sequences and re-ordering, was more difficult than mental calculation that needs maintenance of digit sequences, summation of the digit and updating of the results. There were not significant differences between four groups in digit forward span, visual memory span. The results indicated that the WM span in normal aging declined as task difficulty increased. Their performance decline may be caused by the CE dysfunction. On the other hand, PD showed a characteristic CE deficit observed in mental calculation even in young age and decline with aging. Such decline may be caused by peculiar processing related dysfunction of CE that assumes to be essential deficit of PD.

Gene regulatory networks developed from full genome expression libraries from gene perturbation variant cell lines can be used to quickly and efficiently identify the molecular mechanism of action of drugs or lead compound molecules. We developed an extensive yeast gene expression library consisting of full-genome cDNA array data for over 500 yeast strains each with a single gene disruption. Using this data, combined with dose and time course expression experiments with the oral antifungal agent, we used Boolean network discovery techniques to determine the genes whose expression was most profoundly affected by this drug. Our system identified the gene as the most significantly suppressed target molecule due to exposure to the antifungal agent. This process for network based drug discovery can significantly decrease the time and resources necessary to make rational drug targeting decisions.

The Department of Neurology, Hokkaido University, has been established since 1987, however the bases for neurology had been cultivated through psychiatry and neurosurgery in Hokkaido, especially under the pioneering work by Professor Mitsuo Tsuru. Hokkaido, the northernmost island of Japan, is located between 42 and 46 degrees, and the people have ancestors from various parts of Japan, since this island was reclaimed about 130 years ago. Three major neurological disorders, such as multiple sclerosis(MS), spinocerebellar degeneration(SCD) and Parkinson disease (PD), were discussed on the bases of Hokkaido island. We proved the existence of definite north-south gradient of MS, prevalence rate of 8.6 in Tokachi, Hokkaido, compared with 1-2 in the southern island. SCD, especially hereditary SCD, such as SCA1, SCA2 and SCA14 were reported from Hokkaido. The epidemiological studies of PD showed the prevalence rate of 104.6 at Iwamizawa, Hokkaido in 2000, and we proposed the prevalence rate of PD was close to those of Western countries under the collaborative works with Yonago, Kyoto, and Kagoshima by 2001. Neurology, and its specialization and training programs were also discussed to propose the importance of Neurology in Japan.

A 85-year-old woman was admitted to our hospital because of progressive hoarseness and dysphagia of two days' duration. Neurological examination on admission revealed right pharyngeal and vocal cord palsies. After admission, gradual swelling of her right ear was noted, and on day 6, vesicular eruptions in her right geniculate zone, the right VII and the VIIIth cranial nerve palsies were added. On the basis of these findings, she was diagnosed as Ramsay Hunt syndrome. Varicella zoster virus (VZV) infection was confirmed by the elevation of serum anti-VZV-antibody titer, and detection of VZV DNA from cerebrospinal fluid. Ramsay Hunt syndrome associated with multiple cranial neuropathy is not frequently reported. Reviewing Japanese literatures, we found that the IX and the Xth cranial nerves were most frequently affected, and the half of these cases were initiated with cranial neuropathy other than the VIIth. Additionally, spreading mechanisms of cranial neuropathy, and the early diagnostic problems of these conditions were discussed.

In Japan, freeze-dried live attenuated mumps vaccine has been used optionally since 1981. The effectiveness of mumps vaccination has been established by worldwide research since 1971. On the other hand, because of it's live activity several untoward effects have been reported. Vaccination-related mumps orchitis is a rare adverse effect of mumps vaccine. Only 9 cases of vaccination-related mumps orchitis have been reported in Japan. We describe a case of orchitis following mumps vaccination in adolescence. A 16 years-old male has admitted because of acute orchitis with high fever and painful swelling of right testis. The patient had received vaccination with freeze-dried live attenuated mumps vaccine 16 days before admission. After admission, the bed-rest had completely relieved the symptoms on 6th hospital day. The impaired testis has maintained normal size and consistency 6 months after discharge.

Since bladder injury has no specific clinical symptoms, accurate diagnosis at first consultation is relatively difficult. To elucidate the clinical characters type of injury, clinical symptoms, laboratory findings, methods of therapy and diagnosis, we reviewed 15 patients with bladder injury over a 9-year-period 1990-1998 (10 were traumatic injuries and 5 spontaneous injuries). We found no specific clinical symptom of bladder injury. Bladder injury may occur anywhere in the bladder wall, but most commonly occurred at the dome of the bladder (60.0%). Gross hematuria was not seen in 40.0% of the cases. The accuracy of diagnosis at first consultation was relatively low (46.7%) and the tendency to make a misdiagnosis as acute abdomen on digestive organs was found. Of the traumatic injuries 60% were afflicted in the drunken state, so alcohol intoxication was considered as an important enviromental factor of bladder injury. Surgical repair of injury sites was employed in 11 cases (73.3%: 7 were intraperitoneal injuries, 4 were extraperitoneal injuries), 4 cases were managed with indwelling urethral catheter. With appropriate treatment, the prognosis is excellent.

Malignant mesothelioma is a neoplasm which tends to develop along serosal surfaces, such as the pleura and peritoneum. We report a rare case of malignant retroperitoneal mesothelioma in a 27-year-old woman. The patient was admitted with a chief compliant of intermittent high fever once a month, and left retroperitoneal tumor was detected by dynamic abdominal computed tomography. Since an adrenal tumor was suspected, laparoscopic tumor extirpation was performed. Immunohistochemically tumor cells were positive for calretinin and thrombomodulin, but negative for carcinoembryonic antigen (CEA) and Ber-Ep4. Based on these findings, primary malignant retroperitoneal mesothelioma was diagnosed.

A total of 465 patients with primary and multiple or recurrent, stages Ta and T1 superficial bladder cancer were included in this randomized multicenter trial to compare the prophylactic effect by 17 times instillation of 40 mg doxorubicin or 40 mg epirubicin with no instillation after transurethral resection of tumor(s). The primary endpoint was first recurrence after transurethral resection. Endoscopic examination as well as urinary cytology was performed in each case every three months. It became evident that the recurrence rate in the doxorubicin or epirubicin instillation arm was lower that in the no instillation arm. Toxicity was mainly restricted to bladder irritation in about 10% of patients in each instillation arm.

There have been few effective chemotherapeutic regimens for scirrhous type gastric cancer. A 62-year-old male patient was admitted to our hospital because of anorexia and abdominal discomfort. Gastroendoscopy showed a type 4 advanced gastric cancer in the upper gastric body. Histologic study of biopsy specimens from the tumor revealed poorly differentiated adenocarcinoma. Examination by computed tomography and ultrasonography revealed swollen paraaortic lymph nodes and peritonitis carcinomatosa. The patient was diagnosed as having a nonresectable scirrhous type gastric cancer with peritonitis carcinomatosa and paraaortic lymph node metastasis. This patient was treated weekly with an intraarterial 5-FU (500 mg) and MTX (100 mg) including AT-II by a subcutaneously implanted port system placed into the thoracic aorta. Furthermore, he was administered tegafur/uracil (400 mg/day) 5 days weekly as a pharmacokinetic modulating chemotherapy (PMC). After eight courses of treatment of PMC, paraaortic lymph node swelling and ascites decreased. This chemotherapy produced a partial response in the peritonitis carcinomatosa and paraaortic lymph nodes. This chemotherapy was repeated preoperatively. We reconsidered this case to show indications for operation. The patient died suddenly of acute heart failure before the operation. This therapy was considered an effective treatment for nonresectable gastric cancer.

Image-guided stereotactic surgery of the ventralis intermedius nucleus of the thalamus, globus pallidus, and subthalamic nucleus is a prevailing modality as a treatment of movement disorders. This technical note describes a method of minimally-invasive stereotactic functional surgery for patients with parkinsonian symptom or various tremors. Patients were administered propofol, an intravenous anesthetic, during placement of a burr hole and a stereotactic frame, but not the period when the patients were necessary to be awake. The intravenous anesthesia was very beneficial to relieve local pain and mental stress of the patients. Radionics ImageFusion and AtlasPlan were used for defining the target localization without an intraoperative ventriculography. ImageFusion efficiently fused high-resolution MR images on CT images. AtlasPlan accurately corrected the localization of the tentative target point after measurements from the midpoint of the anterior commissure-posterior commissure line on the modified MRI, and enabled us to reduce the degree of an intraoperative correction to fix a final target. Stereotactic functional surgery has been thought to be less-invasive, however further modifications of surgical procedure and intraoperative medication can make this type of surgery much more less-invasive.