Pupillography of relative afferent pupillary defects in amblyopia associated with peripapillary myelinated nerve fibers and myopia.

Pupillary responses to light were recorded in three patients with unilateral extensive myelinated nerve fibers and amblyopia by means of binocular infrared video pupillography. All of the patients had an afferent pupillary defect in the eye with myelinated nerve fibers. This finding supports the notion that this type of amblyopia is a severe form of anisometropic amblyopia that is often resistant to treatment.

[A case of sigmoid colon cancer in a patient undergoing hemodialysis treated by tegafur/uracil/folinate].

The patient was a 59-year-old man who had undergone hemodialysis because of chronic renal failure. The patient was diagnosed as colonic perforation due to stricture of sigmoid colon cancer, and sigmoidectomy was performed. Since colonic perforation and imperfect lymphnode dissection might increase the risk of recurrence, the patient underwent 6 courses of tegafur/uracil/folinate(UFT/LV)chemotherapy. No adverse events occurred in the course of chemotherapy. UFT/LV chemotherapy may be administered safely with careful management even for a patient on hemodialysis.

Pupillography of automated swinging flashlight test in amblyopia.

Relative afferent pupillary defects (RAPDs) in amblyopia have been reported, and it is widely accepted that amblyopes can have an RAPD. We investigated whether or not this could be confirmed by the use of binocular pupillography. We examined twelve patients (6 males and 6 females, aged 7-57 years) with unilateral amblyopia associated with anisometropia and/or strabismus, using binocular infrared video pupillography (Newopto, Kawasaki, Japan). Eight normal subjects were also tested in the same manner. Two patients' data had to be excluded because of poor recording quality. Only one patient with moderate anisometropic amblyopia was found to have reduced contraction amplitude in the amblyopic eye, and one patient with a borderline pupillary defect. The other amblyopes, some of whom showed even denser amblyopia, did not have a pupillary defect. This study has confirmed that only a small proportion of amblyopes have a reduced pupillary contraction amplitude in the affected eye, as established by pupillographic recordings, and even these amblyopes are not necessarily associated with dense amblyopia.

Negative scotopic ERG and photopic ERG ON response impairment in a patient with normal dark adaptation.

PURPOSE: To present ocular findings in a patient who showed negative scotopic electroretinogram (ERG) and reduced ON response, but normal dark adaptation. CASE: An 18-year-old Japanese male patient who complained of severe asthenopia. His corrected visual acuities were 1.2 in both eyes. His fundi were normal. He had normal contrast sensitivity and normal dark adaptation. METHODS: The patients underwent ERG (including the standard protocol and photopic long flash recordings). RESULTS: The amplitudes of the rod ERG b-wave were reduced. The scotopic standard combined ERG response showed negative configuration. The photopic response to long flash revealed the reduced b-wave (ON response), while the amplitude of the first peak of the d-wave (OFF response) was within the normal range. CONCLUSIONS: Postsynaptic abnormalities in both the rod and cone ON-pathways, which are often found in patients with night blindness, were suggested in the ERG findings, but the dark adaptation of our patient was normal. Neuromuscular evaluation of the patient and ophthalmological evaluation, including ERG, of his parents were normal. To our knowledge, the ophthalmological and electrophysiological findings of our patient cannot be attributed to any known clinical entity.

Pupillography of relative afferent pupillary defect contralateral to monocular mature cataract.

CASE REPORT: Few previous reports have documented a relative afferent pupillary defect (RAPD) in the eye contralateral to a dense unilateral cataract. We report pupillographic findings of a 55-year-old man with a mature cataract in the left eye and an RAPD in the right eye, whose RAPD disappeared after cataract surgery in his left eye. Using binocular infrared video pupillography, we recorded the pupillary responses of the two eyes simultaneously during an automated swinging flashlight test before and after the cataract surgery. The average contraction amplitude in both eyes was significantly larger when the unaffected left eye was stimulated before the cataract surgery, but this difference in contraction amplitude disappeared after surgery on the left eye. COMMENTS: An RAPD was shown quantitatively with a pupillographic technique in the eye contralateral to a mature cataract, confirming previous studies that indicate a dense cataract may produce a small but definite RAPD in the contralateral eye. Such an RAPD associated with a dense cataract must be taken into consideration when evaluating patients with unilateral visual loss.

Effect of the background on the disparity-induced ramp vergence response in humans.

PURPOSE: Our purpose was to investigate the changes in the dynamic property of vergence eye movements caused by changes in the co-existing stationary background in the central visual field. METHODS: Disparity-driven target movement was presented virtually by a head-mounted liquid-crystal display. Two targets were used: a bar-shaped target that moved between 2 and 0.5 m along the mid-sagittal line at a speed of 50 cm/s (vergence target) and a background image of a cross-shaped target that stayed at a distance of 2 m (background target). Eight normal subjects participated in the experiments. The subject was asked to follow the vergence target while the configuration of the background target was randomly changed among four conditions in each experiment: the length (experiment 1) or the width (experiment 2) of the horizontal and vertical lines composing the cross of the background target was each randomly changed among four conditions. A limbus tracker was used to measure eye movements. RESULTS: In experiment 1, there was a negative correlation between the amplitude of the vergence eye movements and the lengths of the lines of the cross in each of five subjects (mean r = 0.018, n = 48 in each subject). Similarly, in experiment 2, there was a negative correlation between the amplitude of the vergence eye movements and the width of the lines of the cross in each of 8 subjects (mean r = -0.12, n = 48 in each subject). CONCLUSION: The vergence response to a target object significantly differs depending on the texture of background objects on the visual axis.

PIII and derived PII analysis in a patient with retinal dysfunction with supernormal scotopic ERG.

PURPOSE: To present electroretinographic (ERG) findings in a patient with retinal dysfunction with supernormal scotopic ERG, and to analyze rod and cone PIII components and rod inner nuclear layer (derived PII) responses. PATIENT: A Japanese 11-year-old girl complained of poor visual acuity. There was no parental consanguinity in her family. The corrected visual acuity was 0.7 in both eyes. No abnormal finding was observed in both fundi. METHODS: The patient underwent full-field ERGs. Rod and cone a-waves were analyzed using photoreceptor models. The derived PII responses were analyzed using a technique described by Hood and Birch. RESULTS: In the photopic ERG, responses to single flash and 30-Hz flicker were attenuated. In the scotopic ERG, b-wave was supernormal in amplitude in response to intense flashes, but smaller than normal and markedly delayed over a lower range of flash intensities. By the PIII analysis, phototransductions (values of S) of both rod and cone were remarkably decreased. The derived PII responses for this patient were larger than the responses for normal subjects, and the onset of the PII responses in this patient are significantly delayed compared to those in normal subjects. CONCLUSIONS: The ophthalmological findings in this patient are consistent with previous publications of this disease. Although it has been reported that the sites of disease action were beyond the outer segment (values of S were within the normal range), our results suggest that photoreceptors could be involved in sites of disease action in at least some patients with this disease.

Rod and cone a-waves in central retinal vein occlusion.

PURPOSE: To evaluate rod and cone a-waves in cases with unilateral central retinal vein occlusion (CRVO). METHODS: Scotopic and photopic flash electroretinograms (ERGs) were recorded in seven patients aged 54-84 with unilateral hemorrhagic CRVO. Rod and cone a-waves were analyzed using photoreceptor models, and Rm(p3) (maximum a-wave amplitude) and S (sensitivity) were calculated. RESULTS: Decreased rod log S was found in all seven cases, and decreased cone log S was found in five cases. In only one case, rod log S in the fellow eye was decreased. The alterations in rod and cone log Rm(p3) were smaller than those in rod and cone log S. Of three cases in which ERGs could be recorded again after a certain follow-up period, rod log S and cone logS became larger in two cases and smaller in one case. CONCLUSIONS: The change in the phototransduction cascade was confirmed not only in rods but also in cones in five of our seven cases of CRVO. The ERG findings might reflect the functional change in the photoreceptor layer after the onset of CRVO.

[Multifocal visually evoked responses in two children with optic neuritis].

PURPOSE: Central visual functions of two children with idiopathic optic neuritis were analyzed and followed in the course of the disease by using multifocal visually evoked potentials (mVEP) and other ophthalmological tests. SUBJECTS AND METHODS: Two girls 10 and 11 years of age with unilateral optic neuritis participated in this study. At the initial onset of the disease, visual acuity of the patients was below 20/400 and severe central visual field impairment was found in the affected eyes. There were no abnormal neurological or radiological findings suggesting multiple sclerosis in these patients. The mVEPs were recorded with a stimulus of 37 hexagons composed of black and white triangles subtending 35 degrees of visual angle. RESULTS: The amplitude of mVEPs from many stimulating locations was severely reduced in the course of the recovery of these patients. Although visual acuity and perimetric sensitivity in the affected eyes recovered to normal after steroid pulse therapy, the amplitude of mVEPs still remained 1/3 to 1/2 of that of the opposite healthy eye. The mVEPs gradually recovered to near the level of the opposite healthy eyes at the latest examination. CONCLUSIONS: Recovery from the central visual impairment due to infantile optic neuritis is more gradual than that suggested by subjective ophthalmological examinations. There is still optic nerve dysfunction after visual acuity and visual field have recovered to normal. The mVEP is one of the most sensitive tools for detecting optic nerve dysfunction in patients with optic neuritis.

[Evaluation of macula in patients with branch retinal vein occlusion using multifocal electroretinogram and optical coherence tomography].

PURPOSE: To evaluate the functions of the macular area in patients with branch retinal vein occlusion (BRVO) by using multifocal electroretinograms (mERG) and optical coherence tomography(OCT). METHODS: mERGs were recorded from 18 eyes of 18 patients with BRVO. An array of 103 hexagonal elements was displayed on a monitor. The latency (ms) and response density (nV/deg2) of mERGs were measured for 7 central locations. The peak and troughs were labeled N1, P1 and N2, respectively. OCT was used to measure the foveal retinal thickness. RESULTS: Statistically significant correlation was found between visual acuity(log MAR) and P1-N1 response density (r = - 0.629, p < 0.05) and N2 latency(r = 0.619, p < 0.05). Foveal retinal thickness had the significant statistic correlations with P1-N1 response density (r = -0.750, p < 0.0001), P1-N2 response density (r = -0.520, p < 0.05) and N2 latency (r = 0.488, p < 0.05). CONCLUSIONS: In 18 BRVO patients, mERGs from the central retinal area were significantly correlated with foveal retinal thickness measured by OCT and with visual acuity. The mERG recording is sensitive to morphological changes and functional disorders induced by BRVO.

Complete obstruction of the lower common bile duct caused by autoimmune pancreatitis: is biliary reconstruction really necessary?

Recent observations suggest that an immune response is involved in the development of chronic pancreatitis. We report a case of autoimmune pancreatitis in a patient who showed complete obstruction of the lower common bile duct. A 63-year-old man was admitted to a local hospital, complaining of appetite loss and back pain. The patient had obstructive jaundice, and percutaneous transhepatic gallbladder drainage was performed. Fluorography through the biliary drainage catheter showed complete obstruction of the lower common bile duct. The patient had no history of alcohol consumption and no family history of pancreatic disease. Physical examination revealed an elastic hard mass palpable in the upper abdomen. Abdominal ultrasound and abdominal computed tomography (CT) scans showed enlargement of the pancreas head. While autoimmune pancreatitis was highly likely, due to the patient's high serum immunoglobulin level, the possibility of carcinoma of the pancreas and/or lower common bile duct could not be ruled out. Laparotomy was performed, and wedge biopsy samples from the pancreas head and body revealed severe chronic pancreatitis with infiltration of reactive lymphocytes, a finding which was compatible with autoimmune pancreatitis. Cholecystectomy and biliary reconstruction, using choledochojejunostomy, were performed, because the complete bile duct obstruction was considered to be irreversible, due to severe fibrosis. After the operation, prednisolone (30 mg/day) was given orally for 1 month, and the entire pancreas regressed to a normal size. Complete obstruction of the common bile duct caused by autoimmune pancreatitis has not been reported previously; this phenomenon provides an insight into autoimmune pancreatitis and provokes a controversy regarding whether biliary reconstruction is needed for the treatment of complete biliary obstruction caused by autoimmune pancreatitis.

Night blindness with depolarizing pattern of ON/OFF response in electroretinogram: a case report.

PURPOSE: To present a patient who has night blindness with a depolarizing pattern of ON/OFF response by electroretinography (ERG). CASE: A 43-year-old woman had had night blindness and poorly corrected visual acuity since childhood. Parental consanguinity was noted. The patient had suffered from mental retardation, epilepsy, and mild cerebellar ataxia. Corrected visual acuity was 20/30 in the right eye and 20/25 in the left. Goldmann perimetry showed no scotoma but slight depression with internal isoptors. No evidence for rod activity was observed by Goldmann-Weekers adaptometry. The ocular fundi appeared normal. METHODS: Conventional full-field ERGs to scotopic (dim and bright flash) and photopic (bright flash and flicker) stimuli were recorded. Photopic ERG responses to long flash stimulation (200 ms) were also examined. RESULTS: The scotopic responses to dim flash were non-recordable, while those to bright flash were severely reduced. The photopic responses to bright flash were decreased. The amplitudes of flicker responses also were significantly decreased, and the implicit times of those responses were prolonged. Although the amplitudes of a- and d-waves to long flash stimulation were reduced, those of the b-wave were within normal range. The implicit times of a-, b- and d-waves were significantly prolonged. The patient showed a normal rise for the photopic b-wave but lacked a normal falling slope after the b-wave peak. An OFF-response late-negativity was also noted. CONCLUSIONS: The abnormal ON/OFF response found in the patient could be diagnosed as depolarizing pattern, characterized by elevation of the plateau to a positive potential above the prestimulus baseline. Since the ERG waveforms and clinical features found in our patient were different from those in previous reports, her ERG findings might reflect another retinal physiological mechanism.

Ocular fundus images by scanning laser ophthalmoscopy in a patient with enhanced S-cone syndrome.

PURPOSE: To present ocular fundus images in a patient with enhanced S-cone syndrome by scanning laser ophthalmoscopy. A 34-year-old Japanese woman whose parents were consanguineous showed mismatched electroretinographic responses to photopically balanced single-flash stimuli, with a larger signal to blue light than to red light. The central macula lacked a foveal reflex, and the surface was dull. Yellowish flecks and retinal pigment epithelium atrophy were evident in a ring at and around the vascular arcades. Faint black pigmentation was deposited in the mid peripheral retina. METHODS: The ocular fundus of the patient was observed by scanning laser ophthalmoscopy with the use of an argon blue laser (wavelength, 488 nm), a helium-neon laser (633 nm), and an infrared laser (780 nm). RESULTS: The argon blue laser showed numerous black spots of pigment, which were observed as faint pigmentation by conventional ophthalmoscopy. The spots were more enhanced with the argon blue laser than with the helium-neon laser. The white spots, which corresponded to the yellowish flecks in a ring at and around the vascular arcades, were more enhanced with the helium-neon laser than with the argon blue or infrared laser. Hypopigmentation of the retinal pigment epithelium was best shown with the infrared laser. CONCLUSION: An abnormality of the retinal structure in enhanced S-cone syndrome may exist in the inner and outer retinal levels, in at least some patients.

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung.

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vater's papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vater's papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.

Changes in thymidylate synthase and its inhibition rate and changes in dihydropyrimidine dehydrogenase after the administration of 5-fluorouracil with cisplatin to nude mice with gastric cancer xenograft SC-1-NU.

BACKGROUND: Although 5-fluorouracil (5-FU) and cis-diamminedichloroplatinum (cisplatin) in combination have synergistic cytotoxicity against both murine and human neoplasms, the precise mechanism of the synergism, and the effects on thymidylate synthase and its percent inhibition, and the effects on dihydropyrimidine dehydrogenase (DPD) remained to be elucidated. METHODS: Experimental chemotherapy was performed using SC-1-NU, a human gastric carcinoma xenograft. SC-1-NU was maintained by serial transplantation in male BALB/c nude mice. The nude mice received various chemotherapeutic regimens consisting of 5-FU and/or cisplatin, with different dosages and periods of administration. After the treatment, we examined the in vivo effects of 5-FU and cisplatin in each regimen on thymidylate synthase and its percent inhibition, and the effects on DPD, in addition to the observation of tumor growth inhibition. RESULTS: The combined use of 5-FU (20 mg/kg per day) and cisplatin (either 1.5 or 7.5 mg/kg per day) showed a synergistic antitumor effect, regardless of the different doses of cisplatin. The long-term administration of 5-FU significantly increased both total thymidylate synthase and the percent thymidylate synthase inhibition rate. The short-term administration of 5-FU significantly decreased DPD. Nevertheless, these changes showed no relation to the combined use of cisplatin. CONCLUSION: Combined administration of cisplatin with 5-FU did not further increase thymidylate synthase inhibition over that occurring with 5-FU alone, which does not support the hypothesis that cisplatin combined with 5-FU modulates thymidylate synthase inhibition in enhancing the anticancer effect of 5-FU. Changes in DPD after the administration of 5-FU may provide an insight into tumor sensitivity and resistance to 5-FU.

Perforated duodenal diverticulum successfully diagnosed preoperatively with abdominal CT scan associated with upper gastrointestinal series.

Although duodenal diverticulum is not uncommon, precise preoperative diagnosis is occasionally difficult. We report a patient with perforated duodenal diverticulum successfully diagnosed preoperatively by an upper gastrointestinal series followed by abdominal computed tomography (CT) scanning. An 81-year-old Japanese woman visited a local hospital because of right-sided abdominal pain. Physical examination revealed diffuse muscle guarding localized in the entire right-side of the abdomen indicative of peritonitis. While plain abdominal X-ray film revealed no free air, abdominal ultrasound and abdominal CT scanning revealed fluid collection and gas in the anterior perirenal space. An emergency upper gastrointestinal series, using water-soluble contrast media, demonstrated multiple diverticula in the descending portion and the horizontal portion of the duodenum. Leakage of the contrast material was found by the upper gastrointestinal series followed by the abdominal CT scanning, suggesting that the peritonitis was caused by the perforated duodenal diverticulum, and an emergency laparotomy was performed. The diverticulum in the descending portion of the duodenum was mobilized from the retroperitoneum and complete resection and peritoneal drainage were performed. The resected specimen showed that the diverticulum was 42 x 23 mm in size, and two separate sites of perforation were identified. The present case suggests that upper gastrointestinal series followed by CT scan is useful for the preoperative diagnosis of perforated duodenal diverticulum.

[A case of Barrett's esophageal carcinoma successfully treated with TS-1 in an elderly patient].

A 92-year-old man was admitted to the hospital for rehabilitation. Upper gastrointestinal endoscopy was performed 2 weeks after admission because of vomiting which was found to be due to a Barrett's esophageal carcinoma at the lower esophagus. We chose chemotherapy with TS-1 at the dose of 50 mg/day in consideration of his age and performance status. His difficulty in swallowing disappeared rapidly. After 7 days of treatment, grade 3 anorexia appeared, so the dosage of TS-1 was reduced to 25 mg/day. After the reduction of TS-1, anorexia soon disappeared. The regimen consisted of TS-1 every 6 weeks as 1 cycle. After 7 cycles of treatment, upper gastrointestinal endoscopy and upper gastrointestinal series revealed that the tumor had a completely disappeared. The effects of chemotherapy were judged as CR. This case suggests that administration of TS-1 is an effective choice of chemotherapy not only for gastric carcinoma but also Barrett's esophageal carcinoma.