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.

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.

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.

[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.

[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.

Central and peripheral visual interactions in disparity-induced vergence eye movements: I. Spatial interaction.

PURPOSE: To evaluate the interaction between central and peripheral disparities in the initiation of vergence eye movements. METHODS: Eye movements were recorded in eight normal subjects using an infrared limbus tracker. Three-dimensional visual stimuli were back projected onto a tangent screen by using two liquid crystal display (LCD) projectors through crossed polarizers. The central target was a vertical bar, which always jumped from 2 to 1 m. The peripheral target was a random-dot pattern that jumped from 2 to 0.75, 1, or 1.5 m (near planes), 2 m (no change), or 3 m (far plane) simultaneously with the central target jump. Latency, amplitude at 150 ms, and average amplitude over 1 to 2 seconds after vergence onset; peak velocity; and the main-sequence relationship of the initial vergence response were calculated. How far the central target appeared to jump was scored subjectively. RESULTS: In half of the subjects, there was a clear effect of the peripheral disparity on the dynamics of the vergence response to the central disparity. The amplitude of vergence at 150 ms, as an index of open-loop gain, was significantly greater when the peripheral target moved closer, but steady state amplitude (average during 1-2 seconds) did not change, and the vergence latency was significantly greater when the peripheral target jumped away. There was no obvious relationship between the perceived amount of movement of the central target and the parameters of the dynamic properties of the vergence response. CONCLUSIONS: Peripheral disparity can modulate the dynamics of the initial vergence response to a central disparity and is probably independent of the perception of motion in depth.

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.

ERG rod a-wave in Oguchi disease.

We analyzed the change in the ERG rod a-wave waveform during the course of dark adaptation in two patients with Oguchi disease. Two Japanese patients showed a homozygous arrestin 1147delA mutation. Scotopic flash ERGs were recorded after different periods of dark adaptation. ERG rod a-waves were obtained after subtraction of the cone ERG contribution. The rod a-waves were fitted with a model of the rod receptor signal. The parameters, Rm(p3) (maximum a-wave amplitude) and S (sensitivity) were calculated. Longer periods in the dark produced larger rod a-wave but only to the first flash presented. The amplitude of the response to subsequent flashes was essentially independent of the period of dark adaptation. Rm(p3) increased with advance of dark adaptation. However, S was nearly constant. Our results suggest that the cause of delayed dark adaptation is not to be sought in the activation of phototransduction process or the regeneration of rhodopsin per se but rather in the deactivation process of the phototransduction cascade.

Gastritis cystica polyposa associated with a gastric stump carcinoma, with special reference to cell kinetics and p53 gene aberrations.

We report a case of gastritis cystica polyposa (GCP) that developed in association with a small stump carcinoma. The patient had had distal gastrectomy for peptic ulcer 33 years prior to the present illness. Total gastrectomy was carried out for the stump carcinoma of the remnant stomach, followed by Roux-en-Y anastomosis. Histological examination revealed that the cancer was associated with a GCP lesion in its neighborhood. The resected stomach was subjected to a cell kinetics study and p53 gene analysis, as GCPs are thought to have a high potential for carcinogenesis. The GCP mucosae, as well cancer tissues and remnant mucosae obtained from the same specimens, were investigated and compared. We found that cell kinetics, as measured by a Ki-67 labeling index count, was more accelerated in the GCP than in the remnant mucosa, and that p53 gene aberrations, including both mutations and deletions, took place in the GCP lesion. As the p53 gene is considered to be recessive, in principle, its tumor suppressive activity is lost only when gene aberration, either mutation or deletion, occurs concurrently or successively in both alleles. It was of interest to us that a benign lesion such as GCP had, in this instance, already developed both gene aberrations, strongly suggesting a precancerous nature for this disease.

Multifocal electroretinogram in patients with central serous chorioretinopathy.

PURPOSE: To assess local retinal function of macula in patients with central serous chorioretinopathy (CSC). METHODS: Multifocal electroretinograms (ERGs) were recorded in 5 patients with unilateral CSC and 20 normal subjects. The waveforms from the central retina within 5 degrees of visual angle were analyzed. The first negative trough was designated as N1, the first positive peak as P1, and the second trough as N2. RESULTS: The multifocal ERG amplitudes were significantly reduced at the first attack of CSC in all patients compared with the values in the normal controls, for P1-N1 (P <.01) and for P1-N2 (P <.01). Multifocal ERG latencies of the patients significantly increased compared with normal controls, for P1 (P <.01) and N2 (P <.01). After the resolution of retinal detachment, although the multifocal ERG amplitudes increased markedly, they did not improve to the normal level during the follow-up period (4-23 months). CONCLUSIONS: Persistent functional impairment of the retina was found by multifocal ERGs in patients with CSC after the resolution of subretinal fluid. A topographical analysis of the multifocal ERG is useful in the clinical observation of CSC.

Electroretinographic findings in three family members with X-linked juvenile retinoschisis associated with a novel Pro192Thr mutation of the XLRS1 gene.

PURPOSE: To present ocular findings in three family members with X-linked juvenile retinoschisis (XLRS) associated with a novel Pro192Thr mutation. CASES: We examined 21- (Case 1), 17- (Case 2), and 10-year-old (Case 3) male patients who showed wheel-like cystic lesions in the macula and a silver-gray reflex in the peripheral retina. Case 2 was a cousin of Case 1. Case 3 was a brother of Case 2. METHODS: Scotopic electroretinogram (ERG) (dim and bright flash), oscillatory potentials, photopic ERG, and 30-Hz flicker responses were recorded in each patient. The XLRS1 gene was analyzed in patient blood samples by a direct sequencing method. RESULTS: A novel missense mutation (Pro192Thr) was identified in the XLRS1 gene in each patient. Variable b/a ratios upon scotopic bright flash stimulation were evident (Case 1: right 1.16, left 1.20; Case 2: right 0.98, left 1.01; Case 3: right 0.81, left 0.83). Only Case 3 showed the typical "negative" waveform. The amplitude of rod b-waves was significantly decreased in all patients. CONCLUSIONS: Three cases with a novel Pro192Thr mutation showed the phenotypic variation in ERG, especially in b/a ratio, which has been considered an important diagnostic parameter.

[Multifocal electroretinograms in patients with retinal vein occlusion].

PURPOSE: To investigate waveform changes of multifocal electroretinograms(mERG) in patients with retinal vein occlusion(RVO). METHODS: Nine eyes of 8 patients with RVO and 29 eyes of 29 normal subjects were examined using mERG. An array of 103 hexagonal elements was displayed on a monitor. mERG latencies(ms) and response densities (nV/deg2) were measured for center area(Ct) and for each of four quadrant areas. The peak and troughs were named N1, P1 and N2, consecutively. RESULTS: In pathological quadrants, although the response densities were abnormal in only one eye, latencies of the N2 and P1 were prolonged in 7 eyes and in 2 eyes, respectively(over 1 SD-2 SD). The latencies were significantly prolonged compared with those of normal eyes(p < or = 0.03, U-test). On the other hand, in the central area, although the latencies were abnormal in 3 eyes, the response densities were reduced in 6 eyes(over 1 SD-2 SD). The response densities were significantly reduced compared with those of normal eyes(p < or = 0.024, U-test). CONCLUSIONS: Both peak latencies in pathological quadrants and response densities in the central retinal area can be sensitive indicators of retinal dysfunction caused by RVO. The mERG system is useful for detecting local retinal dysfunction in patients with RVO.

[A case of membranous nephropathy with ANCA-associated necrotizing glomerulonephritis during oral administration of PTU for Graves' disease].

We experienced a coincidental case of two types of glomerulopathy associated with Graves' disease. A 64-year-old man, who had been treated with propylthiouracil(PTU) for Graves' disease for 15 years, was admitted to our hospital for macroscopic hematuria and rapidly progressive deterioration of renal function. Although his thyroid function had been within the normal range during treatment, the level of thyrotropin receptor antibody(TRAb) gradually increased from a year before admission. Serological tests revealed that he was positive for myeloperoxidase-antineutrophil cytoplasmic antibody(MPO-ANCA). The renal biopsy specimen showed necrotizing and crescentic glomerulonephritis(GN) superimposed on membranous nephropathy(MN). This is a rare case of MN complicated with ANCA associated crescentic GN in a Graves' disease patient. Association of these two renal alterations was not clearly defined. MN involved with Graves' disease also has been rarely reported. Some reports demonstrated deposition of thyroglobulin and other thyroid related antigens in the glomeruli. In the present case, long-term impairment of Graves' disease and elevation of TRAb might have been responsible for the formation and deposition of thyroid-associated immune complex in the glomeruli. As for crescentic GN, PTU might have induced ANCA-associated GN independently of MN. This case is instructive for considering the relation between Graves' disease and renal injury.

[A surgically resected case of advanced gastric carcinoma with peritoneal dissemination after treatment with combined chemotherapy of TS-1 and CDDP].

A 60-year-old female had undergone laparoscopic oophorectomy for right ovarian tumor. At the time of surgery, peritoneal dissemination and ascites was observed. Histological examination revealed that the resected ovary, peritoneal nodes and floating cells in the ascites were metastatic adenocarcinomas. Later, the primary malignant lesion was found to be a type 4 gastric carcinoma. The carcinoma was judged to be unresectable and treated by combination chemotherapy with TS-1 and CDDP every 6 weeks. After 3 courses of treatments, upper gastrointestinal series and endoscopic examinations were conducted and revealed a marked reduction of the tumor size. No carcinoma cells were detected by endoscopic biopsy. CT-scan showed complete disappearance of metastatic lesions. Staging laparoscopy was performed for evaluation of the effects of chemotherapy, and no adenocarcinoma cells at peritoneal nodes or ascites were found histologically. We performed total-gasterectomy with D1 + alpha lymph node dissection. Histopathologically, resected specimens showed severe fibrosis in most parts of the stomach. Following chemotherapy, the carcinoma was judged to be Grade 2 by histopathological examination.

[Sustained NC status for a long period after combined chemotherapy of TS-1 and CDDP for residual lesions following gastrectomy for gastric cancer].

A 71-year-old man with nausea and epigastralgia was referred to our hospital and was diagnosed with type 3 gastric cancer. Palliative resection was performed since radical surgery was impossible due to massive tumorous invasion to the retroperitoneum. Chemotherapy with TS-1 100 mg p.o. was started for the residual cancer lesions, with CDDP i.v. 50 mg added from the 3rd course. We did not find any remarkable side effects except for grade 1 loss of appetite. This chemotherapy was ended after the eighth course. The residual tumor did not disappear but did not grow, and a no change status was maintained for twelve months after the gastrectomy.

[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.