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This 48-week, randomized, placebo-controlled phase 3 study (DMD114044; NCT01254019) evaluated efficacy and safety of subcutaneous drisapersen 6 mg/kg/week in 186 ambulant boys aged >=5 years, with Duchenne muscular dystrophy (DMD) resulting from an exon 51 skipping amenable mutation. Drisapersen was generally well tolerated, with injection-site reactions and renal events as most commonly reported adverse events. A nonsignificant treatment difference (P = 0.415) in the change from baseline in six-minute walk distance (6MWD; primary efficacy endpoint) of 10.3 meters in favor of drisapersen was observed at week 48. Key secondary efficacy endpoints (North Star Ambulatory Assessment, 4-stair climb ascent velocity, and 10-meter walk/run velocity) gave consistent findings. Lack of statistical significance was thought to be largely due to greater data variability and subgroup heterogeneity. The increased standard deviation alone, due to less stringent inclusion/exclusion criteria, reduced the statistical power from pre-specified 90% to actual 53%. Therefore, a post-hoc analysis was performed in 80 subjects with a baseline 6MWD 300-400 meters and ability to rise from floor. A statistically significant improvement in 6MWD of 35.4 meters (P = 0.039) in favor of drisapersen was observed in this subpopulation. Results suggest that drisapersen could have benefit in a less impaired population of DMD subjects.

SUMMARYStreptococcal toxic shock syndrome (STSS) is a severe invasive infection characterized by the sudden onset of shock, multi-organ failure, and high mortality. In Japan, appropriate notification measures based on the Infectious Disease Control law are mandatory for cases of STSS caused byβ-haemolytic streptococcus. STSS is mainly caused by group A streptococcus (GAS). Although an average of 60–70 cases of GAS-induced STSS are reported annually, 143 cases were recorded in 2011. To determine the reason behind this marked increase, we characterized theemmgenotype of 249 GAS isolates from STSS patients in Japan from 2010 to 2012 and performed antimicrobial susceptibility testing. The predominant genotype was found to beemm1, followed byemm89,emm12,emm28,emm3, andemm90. These six genotypes constituted more than 90% of the STSS isolates. The number ofemm1,emm89,emm12, andemm28isolates increased concomitantly with the increase in the total number of STSS cases. In particular, the number ofmefA-positiveemm1isolates has escalated since 2011. Thus, the increase in the incidence of STSS can be attributed to an increase in the number of cases associated with specific genotypes.

Purpose: To determine the prevalence of macular abnormalities detected by optical coherence tomography (OCT) in patients with retinitis pigmentosa (RP), and to report the results of pars plana vitrectomy (PPV) in five patients with RP. Methods: OCT images of the macula of 622 eyes of 323 patients with RP were evaluated. All patients had a complete clinical examination, and PPV was performed on two RP patients with a macular hole (MH), two patients with vitreomacular traction (VMT) syndrome, and one patient with cystoid macular oedema (CME). Results: A macular lesion was detected by OCT in 46 eyes (7.4%) of 37 patients (11.5%). CME was detected in 34 eyes (5.5%) of 26 patients, an epiretinal membrane in four eyes of four patients, VMT in five eyes of four patients, and full-thickness MH in three eyes of three patients. PPV was performed on five of these eyes. The MH was closed with vision improvement after a single surgery in one eye, and was closed after repeated PPVs with a decrease of vision in another eye. In both patients with VMT, the visual acuity decreased after the surgery. The macular morphology and visual acuity did not improve postoperatively on the one patient with CME who underwent PPV. Conclusion: When OCT is used, macular abnormalities are present in 7.4% of patients with RP, which is lower than the prevalence reported earlier. Although vitrectomy can improve the macular morphology in some patients with RP, improvement of visual function may be limited most likely because of the long-standing retinal dysfunction.

To determine whether a significant correlation exists between the visual acuity or foveal thickness and the status of the inner and outer segment junction (IS/OS) of the photoreceptor in patients with retinitis pigmentosa (RP). Three hundred eyes of 163 patients with RP were examined with the optical coherence tomography (OCT). The IS/OS appeared as a distinct, highly reflective line just vitread of the retinal pigment epithelium in the OCT3 images. The IS/OS line was graded into three groups. The correlations between the grade of the IS/OS and age, best-corrected visual acuity (BCVA), and central foveal thickness (CFT) were determined. Grade 1 included 93 eyes (31.0%) in which an IS/OS line was not seen, Grade 2 included 67 eyes (22.3%) with an abnormal IS/OS, and Grade 3 included 140 eyes (46.7%) with a normal IS/OS. The correlation between the IS/OS grade and age was not significant (P=0.5536). The IS/OS grade was significantly correlated with BCVA and CFT (both P

Purpose To evaluate the efficacy of radial optic neurotomy (RON) on retinal function in patients with central retinal vein occlusion (CRVO). Methods Ten eyes of 10 patients with CRVO underwent pars plana vitrectomy and RON. Standardized combined electroretinograms (ERGs) to full-field bright-flash stimuli were recorded preoperatively and, 3 months postoperatively. The best-corrected visual acuity and foveal thickness were also determined, before and 3 months postoperatively. Results The mean best-corrected visual acuity and mean foveal thickness were both significantly improved after vitrectomy and RON. The mean amplitudes of a- and b-waves did not change significantly, but the mean b/a wave ratio was increased significantly after vitrectomy and RON. An improvement of the b/a ratio was observed in six eyes, however, an improvement of retinal circulation was confirmed on postoperative fluorescein angiograms in two of these six eyes. Conclusions Significant improvements were observed in the b/a wave ratio of the standard combined ERGs after surgery in eyes with CRVO. The improvement of retinal function was most likely due to improved oxygenation of retina caused by vitrectomy and not by RON.

The outcome of anti-tuberculosis treatment varies according to patient factors.To retrospectively identify risks related to the extension of time to negative sputum culture (Tn) and to determine their clinical significance.Patients with bacilli susceptible to isoniazid and rifampicin who received initial standard treatment without cessation were recruited into the study. A total of 630 consecutive in-patients were included in the risk development analysis (development cohort) and another 611 consecutive in-patients in the risk validation analysis (validation cohort).Univariate analysis showed that Tn was related to sex, body mass index (BMI), white blood cell count (WBC), serum albumin, fasting blood sugar, haemoglobin A1c, C-reactive protein and total cholesterol levels and sputum smear positivity (SSP). Multivariate analysis showed that BMI, WBC and SSP were significant risk factors related to extended Tn. Optimal cut-offs of BMI and WBC for predicting good (Tn46 days) and poor responders (Tn ⩾ 46 days) according to each risk were determined by receiver operating characteristics analysis. Risks were verified with the validation cohort. Tn increased according to the number of risks; the median Tn for patients with three risks was 21 days longer than that of patients with none.The nutritional state of a TB patient can be used to predict Tn.

The purpose of this study was to determine the morphological and functional changes in the macula after pars plana vitrectomy with the creation of a posterior vitreous detachment in eyes with diabetic macular edema (DME). A simple posterior vitreous detachment was created during pars plana vitrectomy accompanied by simultaneous cataract surgery in 19 eyes of 17 patients with DME. The visual acuity (log MAR units), multifocal electroretinograms (mfERGs), and optical coherence tomographically (OCT)-determined foveal thickness were determined preoperatively and at 6 months postoperatively. The median preoperative log MAR visual acuity was 0.7 (20/100; range, 0.3-1.1), and it improved significantly to 0.4 (20/50; range, 0.15-1.0) postoperatively (p = 0.016). The median foveal thickness was 510 (range, 194-800) microm preoperatively and was significantly reduced to 201 (range, 60-631) microm postoperatively (p =0.0002). The median of response density of the mfERGs in the macular area was 6.3 nV/ deg2 (range, 2.8-14.8) preoperatively, and 6.1 nV/deg2 (range, 2.4-14.3) postoperatively, a non-significant change (p = 0.27). The median of peak latency of the macular response was 30.0 ms (range, 25.0-44.2) preoperatively and it decreased significantly to 28.3 ms (range, 26.7-35.0) postoperatively (p = 0.003). In conclusion, vitrectomy with the creation of a PVD in eyes with DME improved the visual acuity and foveal thickness significantly. In addition, a decrease of the peak latency of the macular mfERGs suggested an improvement of the physiological function of the macula although the amplitude of the mfERGs was not changed significantly.

Aim: To evaluate the morphology and visual function of the macula in eyes with adult onset vitelliform macular dystrophy (AVMD). Methods: 12 eyes of six patients with AVMD were examined by ophthalmoscopy, scanning laser ophthalmoscopy (SLO), optical coherence tomography (OCT), and multifocal electroretinography (mfERGs). The macular lesions were bilateral in all patients and varied from the typical vitelliform (five eyes), faded vitelliform changes with retinal pigment epithelium (RPE) atrophy (five eyes), and a normal fovea associated with small flecks around the macula (two eyes). Results: SLO demonstrated small abnormal bright spots in the deep retina throughout the posterior retina in all cases. OCT showed a highly reflective fusiform thickened layer at the level of the RPE and choriocapillaris in patients with a submacular yellow vitelliform lesion. A well circumscribed, optically clear space was observed beneath the retinal layer in the macular lesions with RPE atrophy. The mfERGs were significantly reduced not only in the macular area but also in the outermost ring (20–30°) of the mfERGs. Conclusions: The submacular materials that accumulate within the RPE or subepithelial layers reported in previous histopathological studies of vitelliform lesions can be detected by OCT. In the macular lesions with RPE atrophy, the material may have disappeared leaving a subretinal or subepithelial optical clear space. These SLO and mfERG observations suggest that the morphological and functional abnormalities may not be localised just in the macular area but may be present throughout the posterior pole in eyes with AVMD.

We describe a 72-year-old man with mycosis fungoides (MF) followed up at our hospital for more than 20 years, who has developed 14 eccrine poromas (EPs) in the past 12 years. Twelve of these tumours were ascertained as EP histopathologically without any findings of malignancy and the other two were clinically diagnosed and are not yet resected. To our knowledge, this is the first case report of MF complicated with multiple EPs. In our patient, all EPs arose in skin areas previously irradiated with an electron beam for the treatment of MF, suggesting that electron beam irradiation might have an effect on the development of EPs.

BACKGROUND Although Japan is categorised as an intermediate tuberculosis (TB) burden country, the incidence of TB is nevertheless decreasing. OBJECTIVE To identify metabolic and nutritional risk factors for the development of TB. DESIGN We compared 522 TB in-patients (350 males, 172 females) with age- and sex-matched 522 controls randomly selected from among individuals undergoing comprehensive medical examinations. Patients with relapsed or multidrug-resistant TB were excluded. RESULT The proportion of males with glucose intolerance was significantly higher in TB patients than in controls (34.2% vs. 14.4%). Among females, the proportion with glucose intolerance was higher in TB patients than in controls (18.3% vs. 10.0%). In male and female controls and female TB patients, individuals with glucose intolerance had significantly higher body mass index (BMI) than those without glucose intolerance. Low BMI was prevalent in male TB patients; there was no significant difference between the BMIs of TB patients with glucose intolerance and those without. Significant associations were found between BMI, peripheral white blood cell count, serum albumin concentration and glucose intolerance and the development of TB in males. Albumin was a parameter significantly associated with TB development in females. CONCLUSION In Japan, the development of TB is still associated with glucose intolerance and malnutrition.

Objectives. This study was undertaken to investigate the incidence of posttransplant recoarctation of the aorta, delineate the mode of presentation, identify risk factors that predict recoarctation and examine the results of intervention for posttransplant recoarctation.Background. Patients with aortic arch hypoplasia require extended arch reconstruction at transplant, with an inherent possibility of subsequent recoarctation of the aorta.Methods. This was a retrospective review of all children (age

Approaching the problem of how close the sporadic diarrhea diseases correlate with epidemics of infectious diarrheas, including food poisonings, bacteriological studies were made on the characteristics of sporadic diarrheas in the past 10 years and analyzed. In collaboration with physicians, mostly pediatricians, in Oita district during 1985 to 1986 and 1989 to 1996, a total of 1707 specimens was obtained from diarrhea patients, suspected of bacterial cause. We have isolated 717 strains from 670 specimens (ca 40% of the total). The majority were; Campylobacter, Salmonella and EPEC with the rate of about 40, 24 and 23% respectively. On the other hand, isolation of Vibrio spp. and Staphylococcus were very few. The yearly trend of detection rates of such major agents were as follows: Campylobacter had the highest rate in the early period, 1989, but thereafter tended to decrease. In contrast, Salmonella continued to increase in rate through the study period until the present when it exceeds that of Campylobacter. This alternation is due primarily to the rapid increase of S. Enteritidis since its recent appearance. VTEC stays at a low rate with a slow increase showing no prevalence so far. From the above results the following may meet with attention. Vibrios and Staphylococci, known as popular agents for food poisonings, seem unlikely to correlate with sporadic diarrheas. Instead, the evidence that recent food poisoning is often represented by Salmonella, especially S. Enteritidis, suggests that epidemics of infectious diarrhea may be attributed to the background of common sporadic diarrheas.

The aim of this study was to evaluate the accuracy of reduced-dose ¹³C-urea breath test (¹³C-UBT) and early sampling of exhaled breath for the detection of Helicobacter pylori infection in children and adolescents.Patients up to 20 years old that underwent upper gastrointestinal endoscopy with gastric biopsies were included. The ¹³C-UBT was performed after a 4-hour fasting period with 4 points of collection: baseline (T₀), and at 10, 20, and 30 minutes (T₁₀, T₂₀, and T₃₀) after ingestion of 25 mg ¹³C-urea diluted in 100 mL of apple juice. The infection status was defined through 3 invasive methods, and a patient was considered infected with a positive culture or concomitant positive histology and rapid urease test. The absence of infection was defined by all negative histology, rapid urease test, and culture. Analysis of exhaled breath samples was performed with an isotope-selective infrared spectrometer. A receiver-operating characteristic curve analysis was done to define cutoff delta over baseline (DOB) values.A total of 129 patients between the ages of 2.1 and 19 years (median 11.6 years; mean age ± standard deviation 11.5 ± 3.8 years; F:M 85:44) were included. The prevalence of infection was 41.1%. The sensitivity (S) and specificity (Sp) were at T₁₀ (cutoff DOB 2.55‰), S 94.7% (95% confidence interval [CI] 90.9-98.5) and Sp 96.8% (95% CI 93.4-100); at T₂₀ (DOB 2.5‰), S 96.2% (95% CI 92.9-99.5) and Sp 96.1% (95% CI 93.7-99.8); and at T₃₀ (DOB 1.6‰), S 96.2% (95% CI 92.9-99.5) and Sp 94.7% (95% CI 90.8-98.6).Low-dose ¹³C-UBT with early sampling is accurate for diagnosing H pylori infection in children and adolescents.

Duct-ligated submandibular and sublingual glands of rats were evaluated immunohistochemically for changes in keratin (MoAb 1164), actin, S-100 protein and rat-EGF (rEGF). Normal salivary glands were reactive for keratin, S-100 protein and rEGF in the granular convoluted tubule (GCT) and duct cells, and for actin in the myoepithelium. Submandibular glands showed a marked reduction of S-100 protein and rEGF staining following duct ligation, and no increased staining of proliferating epithelial cells of the late stage in duct ligated glands. Sublingual glands revealed no marked changes for actin staining in myoepithelial cells, irrespective of atrophic changes occurring in acinar and duct cells after duct ligation. Immunohistochemical patterns differed for each type of gland; changes associated with the obstructive lesion were more prominent in the submandibular gland.

To examine the early changes of retinal sensitivity by fundus-related microperimetry after subthreshold micropulse diode laser photocoagulation (SMDLP) for diabetic macular oedema (DMO). Twenty-eight eyes of 28 patients with diffuse DMO were treated with SMDLP. The mean retinal sensitivity within the central 10 degrees measured with a fundus-related microperimeter, MP1, best corrected visual acuity (BCVA) in logarithm of minimum angle of resolution units, and optical coherence tomography-determined foveal thickness (FT) were examined before and 3 months after SMDLP. The pretreatment values of the retinal sensitivity, FT, BCVA, and funduscopic findings were compared with the corresponding values at 3 months after SMDLP. At 3 months, the BCVA was significantly improved (P=0.03), and the FT was significantly reduced (P=0.0043). The mean retinal sensitivity within the central 10 degree, however, did not change significantly (P=0.70). The correlation between the changes in the retinal sensitivities and the decrease in the FT was not significant. The correlation between the changes in the retinal sensitivities and the BCVA was also not significant. Significant improvements in retinal sensitivities within the central 10 degrees were not observed even though the decrease in FT and the improvement of BCVA were significant. On account of this difference of changes in retinal sensitivity and BCVA, the combination of retinal sensitivity by MP1 and BCVA may be beneficial in assessing the visual function from various angles after SMDLP for eyes with DMO.

To determine the vision-related quality of life (VRQOL) with the National Eye Institute Visual Function Questionnaire-25 (NEI VFQ-25) in patients with retinitis pigmentosa (RP), and to examine the relationship between VRQOL and peripheral visual field defects. Prospective study. The Japanese version of the NEI VFQ-25 was used to study 40 patients with typical RP whose visual acuity was ⩾0.7 (better than 0.15 in log MAR). For control, 40 volunteers with normal vision were studied in the same way. The peripheral visual field was evaluated by Goldmann's perimetry, and the degree of field loss was classified into seven grades. The correlation between the mean of the total composite score of the NEI VFQ-25 and the degree of the visual field loss was determined. The mean NEI VFQ-25 score was 68.4 in RP patients and 90.1 in normal controls. This difference was highly significant (P=0.00004). Among RP patients, there was a significant negative correlation between the mean NEI VFQ-25 score and the degree of visual field loss (r=−0.519, P=0.0006). The significant correlation between the peripheral visual field loss and VRQOL score obtained with the NEI VFQ-25, indicates that a good estimate of the QOL can be determined by the degree of visual field loss in RP patients.

We report a case of aortic valve replacement and repeat coronary artery bypass grafting in a patient with a patent internal thoracic artery bypass graft. A 77-year-old man, who underwent coronary artery bypass grafting (CABG) 10 years ago, was admitted to the hospital for aortic stenosis and angina pectoris. Transthoracic echocardiography demonstrated severe aortic calcification with an 86 mmHg gradient across the aortic valve. Coronary angiography demonstrated the patency of all grafts and 90% stenosis of the left circumflex coronary artery. At surgery, a full median sternotomy was performed. The adhesions around the heart and great vessels were severe and the left internal thoracic artery (LITA) was found adherent to the heart and sternum. The LITA and saphenous vein graft (SVG) graft were dissected carefully. Retrograde coronary sinus (CS) perfusion was performed with antegrade myocardial protection for cardiac arrest. We performed aortic valve replacement (AVR) with a 17 mm St. Jude Medical Regent Valve and CABG to the circumflex artery with the saphenous vein under cardiopulmonary bypass. His postoperative course was uneventful. He was discharged from our hospital without major complications.

Three different polypeptides capable of stimulating histamine release from mast cells were isolated from human whole saliva, using heparin-gel chromatography followed by reversed-phase HPLC. The amino acid sequences of these peptides were shown to be identical to those of histatins 1, 3 and 5.

To clarify the clinical aspects of penetrating thoracic injury.Eighteen patients with penetrating thoracic injury treated from 1987 to 2005 were evaluated. There were 13 men and 5 women. The age distribution was 8 to 69 years, with an average of 36.7 years.There were 14 patients with stab wound and 4 with impalement injury. Five patients with stab wound were those who attempted suicide. In 4 patients with impalement injuries, the cause was fall in 2, traffic accident in 1 and sports injury in 1. The calculated injury severity score (ISS) was over 15 in 4 patients, 6 to 14 in 12, and under 5 in 2. Thoracotomy was performed in 2 patients with cardiac tamponade, 3 with massive hemothorax and 1 with an impalement injury caused by an iron bar. All of them were rescued and got well. In the other cases, after cleansing and debridement, the wound was closed and thoracic drainage was performed. Only 1 patient with cardiac arrest on arrival died within 24 hours after reviving.Emergent thoracotomy is indicated for patients with massive bleeding including shock, continuous air leakage and cardiac tamponade. Since cardiac arrest is difficult to cure, appropriate cooperation with the rescue team is necessary to avoid preventable trauma death.

Type I neurofibromatosis (NF-I), also referred to as von Recklinghausen's disease, is an autosomal dominant disease characterized by neurofibromas and abnormal cutaneous pigmentation (café-au-lait spot). We studied retrospectively the 8 cases operated in our hospital between January 1979 to December 2002, which were complicated with von Recklinghausen's disease and a thoracic surgical disease. The patients were 6 males and 2 females and the age from 16 to 70 (the averaged age was 36 +/- 22). The thoracic diseases were consist of mediastinal tumors (n = 7) and esophageal cancer (n = 1). The operative procedures were tumorectomy (n = 6), subtotal esophagectomy (n = 1), and pericardial cystectomy (n = 1). The mediastinal tumors were neurofibroma (n = 3), malignant schawannoma (n = 1), ganglioneurinoma (n = 2), and pericardial cyst (n = 1). Malignant neoplasms were recognized in 2 cases (25%). The postoperative survival was 10 months for malignant schwannoma, and 8 months for esophageal cancer, and the others were alive. For 1 case of neurofibromas, there was observed to be the reoperated one after the postoperative recurrence. von Recklinghausen's disease are apt to be complicated with thoracic surgical neoplasms, it should be required a careful and systemic exploration especially for malignant neoplasms.

We report 2 cases of postoperative pseudoaneurysm of the descending thoracic aorta, repaired successfully by temporary bypass graft without extracorporeal bypass. One patient presented with a large recurrent pseudoaneurysm that developed 30 years after ligation of a patent ductus arteriosus. The other patient presented with a pseudoaneurysm 18 years after thoracic aortic reconstruction for a traumatic aneurysm. In both cases, a temporary bypass graft was created from a major branch of the aortic arch to the femoral artery prior to definitive reconstruction. The postoperative course was uncomplicated by organ dysfunction or neurologic deficit. Creation of a temporary bypass graft can avoid the need for cardiopulmonary bypass in selected patients. By avoiding heparin use, blood loss is decreased, especially when dense pulmonary adhesions from previous surgery are present.

The aim of this study was to investigate the relation between clenching strength and occlusal force distribution in primary dentition. Twenty healthy children with normal occlusions: 11 boys and 9 girls, ages 3.2-5.8 years (avg. 4.5 years) were selected. Setting the bilateral masseter muscular activity at maximum clenching in full intercuspation as 100%, the occluding forces at 20, 40, 60, 80 and 100% clenching were recorded with pressure-sensitive sheets (Dental Prescale 50H, type R, Fuji Photo Film Co.), and the force of each primary tooth was analysed by computer (Occluzer FPD703). Occlusal force distribution was expressed as a percentage of the total occlusal force of each tooth and was compared between each clenching. There were no significant differences between various clenching strengths in the occlusal force distribution in primary dentition [one-way repeated-measures analysis of variance (ANOVA)]. Thus, the results of the present study suggest that the distribution of occluding forces on a primary dental arch had its own pattern and that the clenching strength had no effect on that pattern. These patterns may be useful in determining occlusal function in children.

Arterial reconstructions for ischemia in patients with Buerger's disease are technically challenging. This retrospective review was conducted to identify the critical factor for a successful outcome in bypass surgery for Buerger's disease.retrospective review.University hospital, hospitalized patients.since 1993, we performed 10 arterial reconstructions in 8 patients with Buerger's disease. There were 8 tibial artery bypasses and 2 collateral artery bypasses. One bypass was performed in the upper extremity.bypass surgery with autogenous vein graft.graft patency.Over a mean follow-up period of 41.8 months, there were 3 graft occlusions. Of these, 2 were of bypasses to a patent but diseased tibial artery. One graft was occluded due to a toe stenosis which had been previously detected. The 2 collateral artery bypasses were patent at the last follow-up.In Buerger's disease, distal arterial reconstruction is frequently necessary to prevent ischemic limb loss. Collateral artery bypass is an option when the main arteries are affected by the disease. A patent but diseased artery should be avoided as a target for reconstruction.

A 39-year-old man has complained of palpitation and dyspnea since 8 months ago. With aortic regurgitation pointed out in another hospital, he was referred to our hospital for examination. Preoperative ultracardiography (UCG) showed a dilated noncoronary sinus of Valsalva and aortic regurgitation. A daughter aneurysm was found by aortography. There were 2 perforations and 1 pouch at the noncoronary sinus cusp (NCC). The noncoronary sinus of Valsalva was dilated and the orifice to the daughter aneurysm was noticed. The noncoronary sinus of Valsalva was obliterated by suturing a dacron patch on the orifice. The aortic valve was replaced with ATS 23 mm valve. Postoperative computed tomography (CT) showed clot formation outside the patch.

To examine the electroretinograms (ERGs) of the short-wavelength-sensitive (S-) and the mixed long- and middle-wavelength-sensitive (L,M-) cones, and the ON- and OFF-responses of the cone ERGs in three patients with X-linked juvenile retinoschisis (XLRS).Cone ERGs elicited by different color flashes and those elicited by long duration stimuli under Ganzfeld conditions were recorded from three patients with XLRS.The S-cone b-waves were undetectable to short-wavelength stimuli in all three XLRS patients, while the L,M-cone ERG b-waves were within the normal range. To long-duration white stimuli, the ON-response (b-wave) was reduced and delayed in all patients compared with that of the normal subjects, while the d-wave or OFF-response appeared normal in amplitude and implicit time.These results support the hypothesis that the normal S-cone ERG arises primarily from the ON-pathway of the cone ERGs and the hypothesis that ON-bipolar cells are predominant in the S-cone system.

Inclusion body myositis (IBM) is a chronic progressive inflammatory myopathy in elders. Three patients with chronic hepatitis C developed IBM. Their muscle biopsies were examined by polymerase chain reaction (PCR) for two patients and immunohistochemistry for three patients. The hepatitis C virus (HCV)-RNA genome was detected in one of the two patients. The degenerated and atrophic muscle fibers were immunoreactive for an anti-HCV antibody in all three patients. Immunoreactivity for an anti-HCV antibody was not apparent in the muscle specimen from an IBM patient without HCV infection. Our findings suggest that HCV infection is related to the pathogenesis in some cases of IBM.

Although dysfunction of the sensory systems in sporadic amyotrophic lateral sclerosis (ALS) has been reported, the clinical characteristics of such cases still remain unknown. We therefore performed a clinico-electrophysiological analysis of sporadic ALS patients.Twelve ALS patients (aged 36-66 years), who had their somatosensory evoked potentials (SEPs) evaluated, were reviewed and their clinical characteristics were delineated. In addition, needle EMG, sensory nerve conduction velocities, motor evoked potentials (MEPs) and cervical MRI or plain X-ray of the neck were also recorded.Three cases were segregated from the other 9 patients because of predominant upper motor neuron signs with pseudobulbar palsy and abnormal posterior tibial nerve and/or median nerve SEPs. The MEPs were also abnormal in these 3 patients and the brainstem auditory evoked potentials were abnormal in one patient. EMG revealed less involvement in the lower motor neurons.Sporadic ALS with a predominant upper motor neuron sign and also demonstrating pseudobulbar palsy with abnormal SEPs, may therefore form a clinical subtype of ALS.

Kinetic resistance is assumed to be one of the main mechanisms of drug resistance in acute myeloid leukemia (AML), but the relationship between cell cycle status at diagnosis and achievement of complete remission (CR) is controversial. Based on the possibility that the cell cycle data after starting induction chemotherapy are more important than the pretreatment data, we used 3-color flow cytometry to examine the cell cycle (G0, G1, S, and G2/M phases) of AML cells on days 0, 5, and 9 of the first induction chemotherapy in 20 patients. Cell cycle data at these 3 time points were compared in the patients who achieved CR (CR cases) and in the patients who had persistent leukemia (non-CR cases) after the induction chemotherapy. In the CR cases, there was a tendency for the percentages of G0-phase AML cells on days 5 and 9 to be smaller than that on day 0, while the opposite tendency was observed in the non-CR cases. When cell cycle data were compared between the CR and non-CR cases, the percentage of G0-phase AML cells on day 9 differed significantly (CR cases 6.9% +/- 10.9%, non-CR cases 50.1% +/- 38.4%; P = .0024). This significance remained when the patients' AML subtype was taken into consideration. None of the other cell cycle data at each time point or the hematologic parameters, which may be related to CR achievement, showed differences between the CR and non-CR cases. We emphasize the importance of cell cycle analysis after initiating therapy and suggest that such analysis can identify refractory AML subjects. The identified subjects may be candidates for clinical trials of cell cycle modulators.

There is little information available regarding immunophenotypic monitoring of minimal residual disease (MRD) in acute myeloid leukaemia (AML). We investigated leukaemic cells co-expressing CD15 and CD117 (CD15+CD117+) in 72 adult AML cases at diagnosis. In 22 cases (31%) with various AML subtypes, more than 5% of leukaemic cells showed the CD15+CD117+ phenotype (range 5.22-55.48%). These 22 cases were younger and had a higher complete remission (CR) rate than the other AML cases, but the CD15+CD117+ cell percentage at diagnosis showed no correlation with the CR duration among the 72 cases. The CD15+CD117+ cell percentage showed a range of 0.00-0.08% in bone marrow cells from 10 haematologically normal subjects. We also investigated CD15+CD117+ cells in sequential bone marrow samples from 17 AML patients who achieved CR and who had had more than 5% CD15+CD117+ leukaemic cells at diagnosis. Because the CD15+CD117+ cell percentage varied among these AML cases, we calculated the percentage of MRD ¿MRD% = [CD15+CD117+ cells (%) in each sequential marrow sample] / [CD15+CD117+ cells (%) at diagnosis of the corresponding case] x 100¿. A high MRD% after 10 months of CR was significantly associated with a short CR duration (P = 0.0004), whereas continuation of a well-reduced MRD% was associated with a long CR duration. The leukaemic cells conserved the CD15+CD117+ phenotype in all of the eight cases who relapsed. Flow cytometric monitoring of CD15+CD117+ cells is simple and can be applied to a substantial fraction of AML cases. This monitoring may be useful for predicting relapse of adult AML.

The objectives of our study were to assess psychiatric distress of caregivers who had been caring for the demented elderly at home and to examine the association of caregivers' psychiatric distress with putative risk factors. Subjects were 294 caregivers living in Amakusa, Kumamoto Prefecture of Japan, whose spouses, parents or other family members were registered at Amakusa Public Health Center as demented elderly. In 1998, Survey on Caregivers' Mental Health was conducted using the General Health Questionnaire (GHQ12) as a measurement for general psychiatric state of caregivers. Two hundred and eighty-two caregivers responded to interviews with complaints of the following psychological symptoms: feelings of unhappiness (55.7%), of stress (41.8%), insomnia (29.4%) and depressed mood (29.1%). Seventy-six caregivers (27.2%) were identified as being above the cut-off point 4 for psychiatric distress caseness. Multivariate logistic regression analysis indicated caregivers' psychiatric distress was statistically associated with caregivers' age, the caregivers' perception of the severity of dementia, the number of years devoted to caregiving at home and perceived financial state. Being 50 to 69 years (OR = 0.37, 95% CI: 0.17-0.81) and being 70 years or older (OR = 0.35, 95% CI: 0.14-0.83) were negatively associated with caseness as compared to being 20 to 49 years. Caseness was positively related to the severity of the elderly's demented state (OR = 6.93, 95% CI: 1.99-24.19), 1 year to 2 years devoted to caregiving at home (OR = 3.26, 95% CI: 1.02-10.38), no family or social support (OR = 2.99, 95% CI: 1.12-7.96) and lower perceived financial state (always OR = 6.99, 95% CI: 2.77-17.64, sometimes OR = 2.41, 95% CI: 1.19-4.85). Reduction of caregivers' psychiatric distress is important for not merely the enhancement of quality of care for demented elders and caregivers' life but for the prevention of elder abuse or neglect. Our study suggests that a comprehensive assessment and intervention program is needed for the demented elderly and their caregivers, in order for caregivers to be able to adapt well to the challenges of caregiving and find personal meaning through caregiving.

We evaluated a method of mitral valve plasty (MVP) for ischemic mitral regurgitation (IMR) by examining the morphological changes of the mitral valve. From November 1992 to May 1998, 8 patients (M : F = 4/4, age 44-79 years, mean age 65.1 years) with IMR underwent surgical repair. Preoperative mitral regurgitation (MR) was grade III of Sellers classification in 7 patients and grade IV in 1 patient. The cause of MR was mitral valve annular dilatation in 4 patients, mitral valve prolapse due to papillary muscle elongation in 2 patients, and partial papillary muscle rupture (PMR) in 2 patients. Cardiac surgery consisted of CABG + MVP in 7 patients and MVP in 1 patient. Mitral valve repair was separated into three types. Repair for annular dilatation consisted of commissuroplasty in 3 patients (2 patients Kay method, 1 patient Reed method) and ring annuloplasty using a Carpentier-Edwards ring (C-E ring) in 1 patient. Repair for papillary muscle elongation consisted of papillary muscle shortening and ring annuloplasty using a C-E ring. Repair for partial PMR consisted of papillary muscle implantation and ring annuloplasty for anterior leaflet prolapse in 1 patient, and quadrangular resection, posterior leaflet plasty (McGoon method) and ring annuloplasty in 1 patient. There was no hospital death. Postoperative outcome was 6 patients with no MR and 2 patients with grade II MR, but they were well-controlled with medication. Based on the morphological changes of the mitral valve, it is considered that MVP for IMR is an effective and recommended procedure.

Progressive muscular dystrophy patients often show progressive body weight loss in early adolescence. This severe body weight loss frequently causes superior mesenteric artery syndrome which may result in a fatal outcome. We performed prandial pulse oximetry and found 12 out of 35 Duchenne muscular dystrophy patients, 1 out of 2 Becker muscular dystrophy patients and 1 out of 3 Limb-Girdle muscular dystrophy patients showed prandial hypoxia with tachycardia. The patients became tired and eating habits were easily interrupted resulting in progressive body weight loss. Nasal administration of 0.2L/min oxygen to 2 Duchenne muscular dystrophy patients, and nasal intermittent positive pressure ventilation to 2 Duchenne muscular dystrophy patients, 1 Becker muscular dystrophy patient and 1 Limb-Girdle muscular dystrophy patient for 15-30 minutes before eating improved the prandial hypoxia and halted the progression of body weight loss. This prandial hypoxia is one of the earliest signs of respiratory failure in progressive muscular dystrophy.

Here we report a case of acute cerebellitis, in which the patient developed right peripheral facial palsy during the recovery phase of cerebellar ataxia. A 67-year-old man developed truncal and limb ataxia following a fever, general fatigue and anorexia. He was diagnosed to have acute cerebellitis. While the ataxia symptoms were improving without any treatment, right peripheral facial nerve palsy developed and an MRI revealed an enhancement of the right facial nerve proximal to the geniculate ganglion. After treatment with acyclovir and corticosteroids, his facial nerve palsy and ataxia both gradually improved. There has been no previous report of an adult case who developed peripheral facial nerve palsy during the recovery phase of acute cerebellitis. This case indicates that a wide spectrum of neurological complications may develop in association with a varicella-zoster virus infection.

Myotonic dystrophy (MD) is an autosomal dominant systemic disorder with an unstable expansion of the CTG triplet repeat in the 3'-untranslated region of the gene encoding myotonine protein kinase (DMPK) which maps to chromosome 19q13.3. Somatic mosaicism of CTG repeats in MD has been reported; and it has been observed that CTG repeats in tumor tissues associated with MD are more expanded than the other tissues. It is not rare that parotid tumors are found in patients with MD. We performed Southern blot analysis for tissues from the parotid tumor, the normal parotid gland, the skeletal muscles, and the leukocyte from a 60-year-old patient with MD. CTG repeat was most expanded in the parotid tumor, and the normal parotid gland had longer expansion of CTG repeat than the skeletal muscles. The leukocyte had the shortest expansion of CTG repeat. The expansion of CTG repeat in the parotid tumor may be related to active cell division and may underlie the occurrence of tumors in MD.

Nocturnal periodic hypoxemia occurring in a 25-year-old Duchenne muscular dystrophy patient under NIPPV control was successfully treated with monoamine oxydase inhibitor (MAOI), safrazine hydrochloride. Five mg of safrazine hydrochloride was administered before sleep, and the periodic hypoxemia disappeared within 14 days. The effect lasted almost seven months without notable side effect. MAOI may be effective for nocturnal hypoventilation through suppression of REM sleep as in the case of tricyclic antidepressants. The effect of tricyclic antidepressants appears immediately. However, it usually fades away within forty days. Safrazine hydrochloride was effective obviously longer than tricyclic antidepressants. Consequently MAOI may be a hopeful candidate of medication for a treatment of nocturnal periodic hypoxemia in Duchenne muscular dystrophy.

We read with interest the article by Arevalo et al 1 about the development of a tractional retinal detachment (TRD) following the use of intravitreal bevacizumab (Avastin; Genetech Inc., San Francisco, California, USA) as an adjuvant to vitrectomy for the management of severe proliferative diabetic retinopathy (PDR). They warned that TRD might occur or progress shortly after intravitreal bevacizumab. We present a case of a retinal detachment with macular hole (MH–RD) that developed after intravitreal bevacizumab that was used for neovascular glaucoma as a result of severe PDR. A 40-year-old man with diabetes of 19 years presented with PDR and neovascular glaucoma in the right eye that was refractory to panretinal photocoagulation (PRP). He was being treated with insulin but had …

Data on endogenous thrombopoietin (TPO) levels and their regulation in myelodysplastic syndromes (MDS) are sparse. We examined the plasma TPO level of 85 MDS patients by a sensitive enzyme immunoassay and the platelet expression of TPO receptor (TPO-R) protein, which metabolizes endogenous TPO, in 19 MDS patients with an equilibrium binding assay using 125I-TPO. The MDS patients had higher plasma TPO levels (7.0 +/- 9.3 fmol/ml) than 52 normal subjects (P0.0001). Refractory anaemia (RA) patients (n = 39) had higher plasma TPO levels than patients (n = 28) with RA with excess blasts (RAEB) or RAEB in transformation (RAEB-t) (P = 0.0002), irrespective of similar platelet counts in these groups. The plasma TPO level correlated inversely with the platelet count in RA patients (P = 0.0027) but not in RAEB and RAEB-t patients (P = 0.7865). These data suggest that the physiological pathway for TPO production and metabolism is conserved, at least partially, in RA, but deranged in RAEB/RAEB-t. The number of TPO-R per platelet was significantly smaller in 19 MDS patients (17.5 +/- 13.3) than in normals (P = 0.0014), but similar between RA patients and patients with RAEB and RAEB-t. Further, the bone marrow megakaryocyte count, determined in 31 MDS patients, was quite similar between RA patients and patients with RAEB or RAEB-t. Thus, in addition to thrombocytopenia, a reduced platelet TPO-R number may contribute to elevated plasma TPO levels in MDS, and a regulatory pathway for circulating TPO other than platelet TPO-R and marrow megakaryocytes, such as blasts expressing TPO-R, may operate in RAEB/RAEB-t.

The aim of this paper was to investigate whether a correlation between dyspeptic symptoms and infection due to Helicobacter pylori could be established. We conducted a prospective study with 27 children who had Helicobacter pylori associated antral gastritis with endoscopic and histologic alterations, without any other peptic lesions. Helicobacter pylori was detected by rapid urease test and histology with hematoxylin and eosin and Giemsa stain. Endoscopy revealed antral nodularities in 20/27 (74%) and enantema in six cases (23%). During histological examination active chronic superficial gastritis was confirmed in 26 cases (96%). Twenty seven children were treated with antimicrobial therapy, eight of which presented infection eradication, six presented nodularities regression and symptoms remission in four. Of the 19 non-eradicated patients, eight (42%) became asymptomatic and 11 maintained symptoms. Statistical analysis to compare symptoms remission was not significant. Taking into account the results presented herewith, we concluded that antral nodularity was the most common endoscopic finding, and there was no sufficient evidence of a correlation between Helicobacter pylori infection and dyspeptic symptoms.

We report an unusual case of T 0 N 2 M 0 small cell lung cancer in a patient with Lambert-Eaton myasthenic syndrome (LEMS). A 52-year-old man began to notice muscle weakness in a left limb in January 1996, which was followed by muscle weakness in his left arm and fingers, appetite loss, and general fatigue. An electromyogram (EMG) showed the waxing phenomenon in response to high-frequency repetitive stimulation. Lambest-Eaton myasthenic syndrome was diagnosed, based on his symptoms and EMG findings. Chest computed tomography (CT) was done, and left paratracheal, tracheobronchial, subaortic, and hilar lymphadenopathy were found. No mass was seen in either lung field. Cytologic examination of the sputum and bronchial lavage fluid were done, but no malignant cells were found Small cell lung cancer was diagnosed after thoracoscopic resection of the subaortic lymph nodes. No metastases were detected by bone scintigraphy, abdominal CT, or magnetic resonance imaging of the brain. Complete response and resolution of symptoms were obtained by chemotherapy and irradiation.

A phase II trial combining cisplatin, carboplatin and etoposide was conducted in previously untreated patients with stage IIIB and IV small-cell lung cancer, in an attempt to increase response rates and prolong survival.Previously untreated patients with small-cell lung cancer, with measurable disease, agedor = 72 years, performance statusor = 2, and adequate hematologic, hepatic and renal function were enrolled in the study. They were treated with 80 mg/m2 cisplatin on day 1, 100 mg/m2 carboplatin on days 2, 3 and 8, and 50 mg/m2 etoposide on days 1, 2, 3 and 8.A total of 46 patients (20 with stage IIIB and 26 with stage IV disease) were enrolled in the study. A total of 186 courses of chemotherapy were given, and the dose was reduced in 27 courses (15%). The chemotherapy was repeated for four or more courses in 30 patients. There were 10 complete responses and 32 partial responses, for a total response rate of 91% (95% confidence interval, 79% to 98%). The median survival time and 2-year survival rates were 18 months and 22% for stage IIIB disease, and 14 months and 15% for stage IV disease. Major side effects were hematologic: leukopenia, anemia, and thrombocytopenia of grade 3 or more occurred in 48%, 46%, and 43% of patients, respectively.The three-drug regimen of cisplatin, carboplatin and etoposide is feasible and active against small-cell lung cancer.

The present study was carried out to determine whether neutrophils could be activated to increase superoxide and myeloperoxidase production during liver surgery in clinical settings. We measured superoxide production in polymorphonuclear leukocytes (PMNs) obtained from the radial artery and hepatic vein during hepatectomy. We also determined plasma myeloperoxidase (MPO) as a marker of activation of the neutrophil. Blood samples were obtained from radial artery and from hepatic vein before operation and immediately after hepatectomy. Superoxide generation in PMNs from radial artery showed no significant change during hepatectomy, while oxidant generation in PMNs from hepatic vein increased after hepatectomy (from 40.5 +/- 4.20 to 44.8 +/- 4.80 nmol/10(6) cells/30 min; p0.05). MPO in the plasma obtained from hepatic vein also increased significantly after hepatectomy (from 166.6 +/- 23.0 to 225.4 +/- 26.2 micrograms/L; p0.05). The results show that neutrophils are activated locally in the liver for enhanced release of superoxide during hepatectomy, suggesting that these oxidant species may be involved in post hepatectomy liver damage.

Response-oriented individualized induction chemotherapy (ROIIC) has been reported to achieve a high complete remission (CR) rate in acute myeloid leukemia (AML). In ROIIC, drug dose is modified for each patient to achieve hematologic target points, based on the concept that this achievement, not a preset drug dose, increases the likelihood of attaining CR. However, the existing target points lack objectivity, therefore prohibiting worldwide use of ROIIC. In addition, the achievement of these target points does not statistically relate to CR, which contradicts the concept of ROIIC. To solve these problems, we evaluated various parameters of 117 AML cases treated by ROIIC to identify the factors relating to CR. Of 117 AML subjects, 75 achieved CR with ROIIC and most cases achieving CR did so with one ROIIC course. Not the administered dose of drugs but several hematologic parameters at the end of ROIIC correlated significantly with CR. A total of four parameters, all related to the leukemic cell mass, were independent predictive factors of CR. A formula consisting of these parameters predicted CR cases with high sensitivity (98%) and non-CR cases with high specificity (96%). Based on this formula, an objective target point (a bone marrow blast mass ratio between before and after ROIIC of below 0.15), valid for dose modification during ROIIC, was established. These results validate the concept of ROIIC and enable objective dose modification in ROIIC and universal application of this therapy. Further, the formula predicting non-CR cases at the end of ROIIC is useful for deciding early therapeutic intervention for such cases.

We evaluated the effect of controlled hypotension induced by PGE1 on evoked spinal cord potential (ESCP) and spinal cord blood flow (SCBF) in 14 patients undergoing laminectomy or laminoplasty. They were divided into two groups: hypotensive group (group H), non-hypotensive group (group N). Controlled hypotension was induced with PGE1 to maintain mean arterial blood pressure at 55-60 mmHg for 45 min. The amplitude and latency of the N 1 potential were analyzed, and the SCBF was estimated by laser doppler flowmeter. There were no significant differences in ESCP and SCBF. These results suggest that controlled hypotension by PGE1 maintained normal local spinal cord blood flow autoregulation.

Tension of a suture placed to the patella to close the medial capsule during 35 primary total knee arthroplasties was measured. The increase in tension with flexion after arthrotomy was significantly smaller in 10 knees with a subvastus incision (subvastus group) than in 25 knees with a standard medial parapatellar incision (standard group). With the prosthesis in place, the patella showed maltracking with the no-thumb technique in 1 knee (10%) of the subvastus group and in 9 knees (36%) of the standard group. A lateral retinacular release was performed in 5 of these 10 knees but not in the remaining 5 knees because the increase in tension was a minimum. There was no case of patellar maltracking at an average follow-up period of 2.1 years after surgery, suggesting that a lateral release is not always needed if retinacular tension shows no significant increase, even cases where the patella dislocates with the no-thumb technique.

Gnathostomiasis is an important food-borne parasitic zoonosis caused mainly by ingesting uncooked or undercooked flesh of freshwater fishes. Although four distinct species of the genus Gnathostoma were identified as the causative agents for human gnathostomiasis, human infections with G. doloresi have been found only in Japan, concentrated in Miyazaki Prefecture. So far we have found 25 cases in Miyazaki Prefecture. Although most of these patients were of cutaneous gnathostomiasis, two patients presented to the hospital with unusual clinical manifestations; one case was a pulmonary gnathostomiasis diagnosed by immunoserological methods, and the other was an ileus caused by migration of the late 3rd stage larva in the colonic tissue, which was found by post-operative histopathological examination. Although cutaneous lesions such as creeping eruption or mobile erythema are the common clinical features of gnathostomiasis, caution should be paid to the presence of such unusual cases.

Aims: Isopropyl unoprostone (IU), a maxi-K channel activator, is used topically to treat glaucoma, and has been reported to have neuroprotective effects on retinal neurons in vitro and in vivo . The purpose of this non-comparative pilot study was to determine whether topical IU will alter the sensitivity of the central retina in patients with retinitis pigmentosa (RP). Settings and Design : Non-comparative pilot study. Materials and Methods : IU was given topically twice a day for 6 months to both eyes of 30 patients with typical RP. The visual acuity was measured with a Japanese Snellen chart, and the mean retinal sensitivities were obtained by fundus-related microperimetry (MP-1). The mean deviation (MD) of the visual field was determined with a Humphrey field analyzer (HFA). All measurements were made before and 6 months after the treatment. Statistical Analysis Used : Wilcoxon and the Mann-Whitney U tests (SPSS, SPSS Inc., Chicago, IL). Results: After the treatment, the mean retinal sensitivity within the central 2° and 10° improved significantly from 12.3 ± 4.8 dB to 14.7 ± 5.5 dB ( P = 0.001) and from 9.1 ± 5.4 dB to 11.0 ± 6.2 dB ( P = 0.001), respectively. Conclusions: These short-term results suggest topical IU can improve the central retinal sensitivity in RP patients. It will be necessary to examine longer treatment periods in a controlled study to determine the effectiveness of topical IU in RP patients.

The bcl-2 gene belongs to a new category of oncogene that inhibits programmed cell death (apoptosis). No data are available on the frequency or clinical importance of its expression in patients with small cell lung cancer (SCLC), although its expression is reported in SCLC cell lines. We investigated the correlation between bcl-2 expression and prognosis, including response to chemotherapy, in SCLC patients and report our findings here.Tumor specimens biopsied bronchoscopically in 38 SCLC patients were used for immunohistochemical study. Bcl-2 oncoprotein was detected by obtaining an immunostain using a specific monoclonal antibody, DAKO-bcl-2, 124. All patients received more than two courses of chemotherapy with 3- to 4-week intervals.We detected bcl-2 expression in 21 of the 38 SCLC patients (55%). In 17 patients with bcl-2-negative tumors, the objective response to chemotherapy was 76% with 12% complete response and in 21 patients with bcl-2-positive tumors it was 62% with 8% complete response. The difference in response rate was not significant. In patients with bcl-2-positive tumors, survival time tended to be shorter than in those with bcl-2-negative tumors. There was no significant correlation between bcl-2 expression and clinical factors (gender, age, smoking, performance score, staging).In 21 of the 38 SCLC patients bcl-2 oncoprotein was abnormally expressed and its expression may be associated with shorter survival times and poor response to chemotherapy.

Respiratory insufficiency is a major cause of death in Duchenne muscular dystrophy (DMD). The earliest sign is a hypoventilation which appears in REM sleep. We administered 20 mg of clomipramine hydrochloride before sleep to two DMD patients with periodic nocturnal hypoxemia. The complaints such as morning headache, nausea and somnolence disappeared promptly. Polysomnography revealed complete suppression of REM sleep and disappearance of periodic hypoxemia in both cases. The most important untoward effect was intractable constipation observed in one patient. Although clomipramine dramatically improved the nocturnal periodic hypoventilation, the therapeutic effects lasted less than three months. Permanent REM sleep suppression is requisite for optimal drug therapy of periodic nocturnal hypoventilation in DMD.

We describe a 19-year-old patient who developed mononeuritis multiplex associated with measles virus infection. On April 30th, 1995, he suffered from fever and skin eruption, which disappeared ten days later. Thirteen days later, he noticed weakness of the fingers of both hands and forearms, and pain on both elbow joints. He was admitted to our hospital on June 12th, 1995. Neurologic examination revealed weakness and sensory disturbance, confined to the distal aspects of the right radial nerve and left median nerve. The electrodiagnostic findings confirmed those of axonal neuropathy in the right radial and left median nerves. Routine laboratory studies were normal. The cerebrospinal fluid test and EEG were normal. The IgG and IgM antibody titers against measles virus continued to be elevated in serum over four months after the onset, while they were not elevated in the cerebrospinal fluid. The corticosteroid therapy was mildly effective. Although the pathogenesis of this case is unclear, we speculate that the mononeuritis multiplex was caused by vasculitis related to the persistent measles virus infection in both elbow areas.