The feline fictive startle response and its related potential in the pedunculopontine nucleus.

The human P1/P50 midlatency auditory evoked potential and the auditory startle response (SR) have been used for investigating sensory gating and sensorimotor modulation which is impaired in various psychiatric diseases. In the present study, we demonstrated that auditory stimulation was capable of eliciting excitation of flexor and extensor neurograms from the hindlimb nerves in the paralyzed decerebrate cat, a phenomenon which corresponds to a "fictive" startle response (FSR). Previous studies have shown that the SR consists of distinct excitatory components, "early" and "late", separated by an inhibitory phase. However, in the FSR, unlike the SR in the intact preparation, the "late" excitatory phase never occurred. Recordings from the pedunculopontine nucleus (PPN) simultaneously with the FSR revealed the presence of an auditory evoked potential at a 20-25 ms latency, presumably the depth-recorded equivalent of the vertex-recorded wave A, which has been shown to be the feline equivalent of the human P1 potential. The depth-recorded wave A appeared to share neurological substrates with the excitatory phase of the FSR, since both responses were facilitated in a similar manner by increasing stimulus duration. We previously reported that, in the intact rat, the vertex-recorded P13 potential, the putative rodent equivalent of the human P1 potential, is generated, at least in part, by outputs of the PPN, and that the P13 potential shares neurological substrates with the "early" excitatory phase of the SR. Taken together, the results of the present study indicate that, along with the SR and the P13 potential in the intact rat, the FSR and the depth-recorded wave A in the paralyzed cat may be unique animal models for further examining, in the absence of neural structures rostral to the precollicular decerebration, the cellular basis of startle behavior.

Fundic adenomyomatosis bulged with the subserosal excessive fat of the gallbladder mimicking polypoid carcinoma: a case report with unusual imaging and morphological features.

This report describes a 41-year-old female who presented with adenomyomatosis of the gallbladder mimicking polypoid carcinoma, on the diagnostic imaging findings and revealing unusual histologic features for such a localized adenomyomatosis. The mass was located on the gallbladder liver-side wall at the fundus and papillary hyperechoic growth showed no clear ultrasonographic features of adenomyomatosis. The patient underwent a laparoscopic cholecystectomy with a tentative diagnosis of superficial polypoid carcinoma. Histologically, the tumor bulged due to subserosal excessive fat tissue.

Pedunculopontine stimulation induces prolonged activation of pontine reticular neurons.

Extracellular and intracellular recordings were carried out from neurons in the region of the pontine reticular formation at the transition between the nucleus reticularis pontis oralis and caudalis, and in the pontis caudalis. Responses were studied after stimulation of the mesopontine cholinergic pedunculopontine nucleus in precollicular-postmammillary transected, paralyzed preparations. Recordings of neurographic activity in hindlimb flexor and extensor nerves served to detect changes in fictive locomotion and muscle tone induced by pedunculopontine nucleus stimulation or occurring spontaneously. Short duration trains of pedunculopontine nucleus stimulation induced long lasting responses, on average over 12s in duration, in one-third of pontine reticular neurons. These prolonged responses were stimulation frequency-dependent such that the longest durations were induced by stimulation at 20-60Hz. In some cells, stimulation at lower (10Hz) or higher (100Hz) frequencies induced responses of shorter duration or were absent, while in others, higher frequencies prolonged the excitatory effects of pedunculopontine nucleus stimulation. We conclude that these stimulation frequency-dependent effects may be related to the modulation of postural muscle tone and locomotion by the pedunculopontine nucleus.

Serum KL-6 levels in patients with pulmonary complications after allogeneic bone marrow transplantation.

KL-6, a mucinous high-molecular weight glycoprotein expressed on type 2 pneumocytes, has been shown to be elevated in the serum and bronchoalveolar lavage fluid of patients with interstitial pneumonitis (IP). We measured the serum levels of KL-6 in patients after they had undergone allogeneic bone marrow transplantation (BMT) to determine whether KL-6 could be a clinically useful indicator for the development of IP. The serum concentrations of KL-6 were determined by a sandwich-type enzyme-linked immunosorbent assay using an anti-KL-6 monoclonal antibody. A total of 1028 samples were tested from 76 patients (78 transplantations) who received BMTs. The KL-6 values were markedly elevated in patients with pulmonary complications, but not in those with acute and chronic graft-versus-host disease, hemorrhagic cystitis, herpes encephalitis, sepsis, and veno-occlusive disease. The serum levels of KL-6 from patients with pulmonary complications were significantly higher than from those without pulmonary complications (P < .001) and those with other complications (P < .001). Of the 12 patients with pulmonary complications, 6 had idiopathic IP (IIP). The levels were not high at the onset of IIP. Four of 6 IIP patients showed marked elevations of KL-6 levels in parallel with the severity of IP and died of respiratory failure without response to treatment. Assessment of serum KL-6 levels might not be useful for the early diagnosis of IP, but may be a useful indicator for monitoring the severity of IP after BMT.

An extraosseous aneurysmal cyst in the pelvic cavity: report of a case.

A 51-year-old Japanese female was referred to us with a left lower quadrant pain and palpable mass. The CT and MRI study showed a 10x8x8 cm of well-circumscribed, multicystic mass adjacent to the left iliac bone. The mass was excised and consisted of multiple cysts containing bloody viscous material surrounded by thin-ring of eggshell-like tissue. The histological findings correlated with those of an aneurysmal bone cyst. This extraosseous case might be the first reported case observed in the pelvic cavity.

Locus coeruleus involvement in the effects of immobilization stress on the p13 midlatency auditory evoked potential in the rat.

1. Adult male rats were prepared for recording midlatency auditory evoked responses from the vertex (Vx, P13 potential) and auditory cortex (ACx, P7 potential). 2. The P13 potential is the rodent equivalent of the human P1 or P50 potential, which exhibits decreased sensory gating in posttraumatic stress disorder. 3. Immobilization (IMB) stress for 60 min led to a significant decrease in P13 potential amplitude and sensory gating of the potential for the first 30-40 min of IMB. 4. The effects of IMB on the P13 potential were reduced by pre-treatment with the alpha-2 adrenergic receptor blocker yohimbine (YOH). 5. Injections of corticotropin releasing factor (CRF) into the locus coeruleus (LC), but not injections dorsal or ventral to the LC, induced a dose-dependent decrease in P13 potential amplitude and sensory gating. 6. The effects of CRF were blocked by cotreatment with the CRF receptor antagonist alpha-helical CRF (alpha-h CRF). 7. The effects of IMB on the P13 potential were mimicked by injections of the alpha-2 adrenergic receptor agonist dexmedetomidine (DEX) into the pedunculopontine nucleus (PPN). 8. The effects of DEX injections into PPN were reduced by pre-treatment with the alpha-2 adrenergic receptor blocker YOH. 9. The effects of IMB on P13 potential amplitude and sensory gating may be mediated in part via CRF activation of LC, which sends inhibitory alpha-2 adrenergic projections to PPN, a major source of the P13 potential.

Cholinergic modulation of the sleep state-dependent P13 midlatency auditory evoked potential in the rat.

Injections into the pedunculopontine nucleus (PPN) of the cholinergic receptor agonist, carbachol (CAR), were found to reduce the amplitude of the vertex-recorded, sleep state-dependent P13 midlatency evoked potential in a dose- and time-dependent manner. This effect was blocked or reduced by pretreatment with the muscarinic receptor antagonist, scopolamine, injected into the PPN.

A case of adult-onset Still's disease complicated by non-Hodgkin's lymphoma.

Left cervical lymphadenopathy developed in a 50-year-old male who had a history of adult-onset Still's disease for the preceding 18 months. Still's disease is characterized by rash, fever, and leukocytosis. Lymphadenopathy has been reported in about 60% of the patients, and most histopathologic studies have shown non-specific reactive hyperplasia. However, in this case, an open biopsy of the cervical node revealed a histology of diffuse large B-cell lymphoma. The B-cell malignant lymphoma that developed may have resulted from a sequential progression of a previous stage of benign lymphoproliferative lesion. Our case suggests that the pathophysiology of adult-onset Still's disease involves the stimulation of lymphoid systems to the point of progression towards lymphoma. Malignant lymphoma should be added to the list of life-threatening complications which, although rare, are associated with this disease.

Granular cell tumor of the rectum: a case report and review of the literature.

A 47-year-old Japanese woman with a 5-year history of alcoholism was admitted to the Ryukyu University Hospital for the treatment of the alcoholism. For evaluation of observed changes in her bowel habits, she underwent colonoscopy, which revealed seven small polyps spread throughout the entire large intestine. Six of the polyps were in the colon; one was an adenoma and five were hyperplastic polyps. The remaining polyp, in the rectum, was an 8-mm submucosal tumor. Pathological analysis of a biopsy of the lesion in the rectum indicated a possible diagnosis of adenocarcinoma. Endoscopic ultrasonography (EUS) demonstrated a submucosal hypoechoic nodule, involving the mucosa and the muscularis propria. Subsequently, the patient underwent a radical low anterior resection of rectum. The lesion was a submucosal tumor with ulceration. The tumor consisted of granular tumor cells which were positive for S-100 protein, neuron-specific enolase, and periodic acid schiff (PAS) stain, but negative for desmin and vimentin. Granular cell tumor is rare in the gastrointestinal tract. As a result, such tumors can be misinterpreted to indicate a possible malignancy on either a biopsy or EUS.

Serotonergic modulation of the P13 midlatency auditory evoked potential in the rat.

The vertex-recorded, sleep state-dependent P13 midlatency auditory evoked potential in the rat may be generated, in part, by pedunculopontine nucleus (PPN) projections. Injections into the PPN of the 5-HT(1A) serotonin receptor agonist, 8-hydroxy-2-di-n-propylaminotetralin hydrobromide (DPAT), were found to reduce the amplitude of the P13 potential in a dose- and time-dependent manner. The suppressive effect of DPAT was blocked or reduced by pretreatment with the 5-HT(1A) serotonin receptor antagonist, Pindobind. These results show that the P13 potential can be modulated by known inhibitory serotonergic inputs to the PPN.

Combined endoscopic and surgical treatment for multiple polyps of the small intestine in Peutz-Jeghers syndrome: a case report.

Combined endoscopic and surgical treatment in a 14-year-old girl with Peutz-Jeghers syndrome is reported herein. The patient was diagnosed with Peutz-Jeghers syndrome because of mucocutaneous pigmentation and hamartomas of the small intestine at 10 years of age, when she underwent an emergency laparotomy for an intussusception of the small intestine. Since this diagnosis, she has undergone follow-up, and barium radiologic and endoscopic studies have shown multiple polyps of various sizes from the stomach throughout the small intestine and to the colon. This time, with the use of combined endoscopic and surgical treatment for polyps of the small intestine, 26 polyps were removed endoscopically by performing only one enterotomy. This combined technique may allow for a longer interval between laparotomies, therefore reducing the complications associated with multiple laparotomies and resections.

Successful resection for advanced hepatoblastoma, combined with perioperative chemotherapy.

The aim of this study was to evaluate our results of surgical treatment with intensive perioperative chemotherapy for hepatoblastoma in infants and children. Seven patients (mean age, 30 months; range 1 month to 6 years) with hepatoblastoma who were followed-up for more than 3 years were reviewed. All patients underwent hepatectomy, performed using a microwave tissue coagulator, after they had received neoadjuvant chemotherapy comprising up to four cycles of cisplatinum and doxorubicin. The main outcome criteria were the clinical response rates to neoadjuvant chemotherapy and the overall survival. Neoadjuvant chemotherapy markedly reduced the tumor volume on computed tomography (mean regression rate, 73%). Alpha-fetoprotein (AFP) levels also decreased, from a mean value of 138 x 104 to 990 ng/ml (excluding values for one patient with tumor thrombus in the portal vein). The surgical procedures included extended right lobectomy in one patient, extended left lobectomy in two patients, hepatic left trisegmentectomy in one patient, and hepatic subsegmentectomy in three patients. The postoperative clinical courses in all seven patients were good, and no serious complications were observed. No relationship was observed between the DNA ploidy pattern and the histopathological findings of the resected specimens regarding survival. Six patients (excluding the patient with a tumor thrombus in the portal vein) who underwent complete resections survived without any signs of recurrence during a follow-up period ranging from 47 to 150 months. In conclusion, the perioperative chemotherapy greatly improved both the resection rate and overall survival in patients with hepatoblastoma. DNA ploidy pattern analysis may be useful when predicting the prognosis of patients with hepatoblastoma. The use of the microwave coagulator was safe for performing hepatectomy, even in infants.

Expression of p53, bcl-2, and bax as predictors of response to radiotherapy in esophageal cancer.

The sensitivity of cancers to radiotherapy or chemotherapy may be influenced by susceptibility to apoptosis. We evaluated whether expression of three proteins regulating apoptosis, p53, bcl-2, and bax, could predict the effect of radiotherapy in esophageal cancers. We used immunohistochemical staining for these protein regulators of apoptosis to study biopsy specimens obtained from 25 patients with esophageal squamous cell carcinoma before they underwent preoperative radiotherapy. Effectiveness of radiotherapy was assessed by barium esophagography, esophagoscopy, and computed tomography. Radiotherapy was effective in 12 patients and ineffective in 13 patients. Biopsy specimens from the 25 patients showed expression of p53, bcl-2, and bax to be 48.0%, 32.0%, and 76.0% respectively. Effectiveness of radiotherapy was correlated with p53 expression (p = 0.047), but bcl-2 and bax expression showed no relationship to effectiveness of radiotherapy. Expression of p53 protein in biopsy specimens may predict effectiveness of preoperative radiotherapy in esophageal cancers.

Course and outcome of tubulointerstitial nephritis and uveitis syndrome.

We report here the clinical features and outcomes of 10 patients, aged 11 to 21 years (median, 13.0), with idiopathic tubulointerstitial nephritis (TIN) and uveitis syndrome (TINU syndrome). The initial symptoms were visual impairment in 7 patients and prolonged fever, anemia, or asthenia in 4 patients. An increase in urinary beta(2)-microglobulin was noticed at the initial checkup in all patients, including 2 patients who showed the normal ranges of 24-hour protein excretion. Creatinine clearance was decreased in 8 patients. TIN was found simultaneously with ocular symptoms in 7 patients and preceded these symptoms in the remaining 3 patients. Percutaneous renal biopsy indicated tubulointerstitial lesions in varying degrees. The histological grade of TIN was correlated with urinary beta(2)-microglobulin levels. Systemic steroid therapy was performed in 7 patients because of the progression of uveitis. The 10 patients were followed-up for 16 to 94 months (median, 31.0 months). In all patients, creatinine clearance recovered to the normal ranges (>/=70 mL/min/1.73 m(2)) mostly within 1 year. Urinary beta(2)-microglobulin excretion gradually declined but was slightly elevated in 4 patients at the latest checkup. Uveitis recurred in all 10 patients, which did not affect the renal status. Our findings indicate that early referral of patients from ophthalmologists and determination of beta(2)-microglobulin in the urine is helpful for the early discovery of TINU syndrome. In children and adolescents with this syndrome, TIN spontaneously resolves and its long-term prognosis is good, but uveitis often relapses. Systemic steroids may be required for uveitis, but not for TIN.

Familial skeletal myopathy with atrioventricular block.

OBJECTIVE: We studied familial cases of skeletal myopathy with atrial fibrillation (Af) and atrioventricular (AV) block to compare the clinical features to other myopathies associated with cardiac abnormalities. METHODS: Neurologic, cardiologic, electrophysiologic, muscle pathology, and genetic studies were performed on the patients showing muscle weakness. PATIENTS: Four patients (a 63-year-old mother, 30 and 32-year-old sisters, and their maternal grandmother) and three healthy family members from three generations were studied. The mode of inheritance was suspected as autosomal dominant. RESULTS: Two sisters with congenital myopathy without rigid spine developed Af and AV block at the age of 28 and 18, respectively. The mother showed AV block, and underwent pacemaker implantation at the age of 63. The maternal grandmother had dilated cardiomyopathy, Af and severe lordosis. She died of stroke attacks and congestive heart failure at the age of 78. Muscle biopsy obtained from the mother and sisters showed myopathic changes without characteristic abnormalities. No mitochondrial DNA mutations were found. Other inherited myopathies with cardiac complications were not suspected in this family. CONCLUSION: This Japanese family appears to belong to a new genetically heterogeneous group of autosomal dominant skeletal myopathy with severe AV block and Af.

Results of retrograde transhepatic biliary drainage after a common bile duct exploration for choledocholithiasis.

BACKGROUND/AIMS: The purpose of this study is to assess the benefits of retrograde transhepatic biliary drainage (RTBD) and a primary closure after a common bile duct (CBD) exploration for patients with choledocholithiasis. METHODOLOGY: We analyzed 143 patients with choledocholithiasis who had been managed by RTBD after undergoing a CBD exploration retrospectively over a 12-year period. The main outcome criteria were frequency of occurrence of post-operative complications which needed a relaparotomy and the clinical long-term results. In addition, the radiographic diameter changes of the CBD at the site of the primary closure and liver function tests after RTBD were also evaluated. RESULTS: The frequency of bile peritonitis in the patients undergoing the RTBD procedure was only 0.7% (1 out of 143 cases). Cholangiography via the RTBD tube revealed no severe stenosis at the site of primary closure. Liver function returned to normal on day 3 after RTBD (p<0.05). Recurrence of common bile duct stones developed in 2 patients in this series during the follow-up (1-12 years). CONCLUSIONS: RTBD and a primary closure of the CBD after CBD exploration appears to be a clinically safe and effective method for such patients with choledocholithiasis who had undergone a complete stone removal intra-operatively.

Analyses of microsatellite instability and the transforming growth factor-beta receptor type II gene mutation in sporadic human gastrointestinal cancer.

Microsatellite instability (MSI) and transforming growth factor-beta receptor type II (RII) gene mutation have been reported in many types of tumors and their instance seem to vary among the tumors investigated. To determine the relation between MSI and RII gene mutation in sporadic gastrointestinal cancer development, 21 esophageal, 19 gastric, and 27 colorectal cancers were investigated. The presence of MSI was screened by single strand conformation polymorphism (SSCP) method using six microsatellite markers. RII gene mutations were detected by SSCP method and direct sequencing. MSI was detected in seven of 21 (33.3%) esophageal cancers, three of 19 (15.8%) gastric cancers and seven of 27 (25.9%) colorectal cancers. However, RII gene mutations were observed in only two of seven (28.6%) MSI-positive colorectal cancers. Our data suggest that among sporadic gastrointestinal cancers, colorectal cancers seem to be the most frequent target organ involved in carcinogenesis through RII gene mutation, which thus appears to be related to organ specificity.