A case of lymphomatoid granulomatosis/angiocentric immunoproliferative lesion with long clinical course and diffuse brain involvement

Lymphomatoid granulomatosis (LYG)/angiocentric immunoproliferative lesions (AIL) consist of angiocentric and angiodestructive lymphoreticular proliferation predominantly involving the lungs and other extranodal sites, such as the central nervous system (CNS). This clinical entity is considered as a B cell process related to Epstein-Barr virus (EBV) infection and EBV positive diffuse large B-cell lymphoma. The CNS is involved in 20% of cases of LYG, but initial involvement is rare. In cases without pulmonary symptoms, diagnosis may be difficult. We report a rare case involving initial progression of CNS symptoms followed by a pulmonary abnormality.A 14-year-old girl suffered from high fever, ataxic gait and paraparesis. MRI revealed diffuse T2 high signals with multiple gadolinium enhancements in the cerebellum, brain stem and cerebral white matter. Her symptoms briefly improved after steroid therapy, but ataxia gradually progressed. Dyspnea due to pulmonary interstitial involvement appeared when she was 18 years old. Steroid therapy proved effective for respiratory symptoms. At 20 years old she suffered from disseminated intravascular coagulopathy (DIC) and hemophagocytic syndrome (HPS) with respiratory symptoms and repeated seizures. Her symptoms improved after the administration of cyclophosphamide. Mild hemiparesis and gait disturbance appeared when she was 22 years old. MRI revealed new lesions at the basal ganglia and subcortical white matter, brain atrophy and diffuse T2 high intensity of cerebral white matter. Cyclophosphamide was effective and there has been no recurrence of symptoms in the last 5 years. We reviewed the non-tumorous LYG/AIL involving the CNS, and discussed the clinical features, MRI imaging and diagnosis of the LYG/AIL.     

Periodic leg movements during sleep in Japanese community-dwelling adults based on the assessments of their bed partners

BACKGROUND: There is little known about epidemiologic evidence on periodic leg movements during sleep (PLMS) for the Japanese. The present study was a cross-sectional epidemiologic study to estimate the prevalence of PLMS and examine the associated factors of PLMS in Japanese community-dwelling adults. METHODS: The subjects were 884 with bed partners or bedroom mates of 1,889 Japanese adults aged 20 years and over randomly selected from the general population. The case ascertainment of PLMS was based on the assessments of their bed partners or bedroom mates using the Pittsburgh Sleep Quality Index. Multiple logistic regression analyses were used for investigating the associated factors. RESULTS: The age-adjusted prevalences (95% confidence interval) were 5.8% (4.7-6.8%) and 1.3% (0.8-1.9%) for 1 to 2-times, and 3-times or greater of PLMS per week during the preceding month, respectively. Those with PLMS were more likely to experience difficulty in initiating sleep, snore during sleep, be depressed, and suffer from peptic ulcer. Sex, age, difficulty in maintaining sleep, excessive daytime sleepiness, medication use to aid sleep, and any psychoactive substances (tobacco, alcohol, and caffeine) were not identified as significant associated factors of PLMS. CONCLUSIONS: The results suggest that the prevalence of PLMS in Japanese community-dwelling adults is not so high as those reported from Western countries, and that PLMS is correlated with some sleep and health disturbances.     

Dynorphin A inhibits NMDA receptors through a pH-dependent mechanism

Dynorphin A (DynA), an endogenous agonist of kappa-opioid receptors, has also been reported to directly interact with the NMDA receptor. DynA inhibition of NMDA receptor function has been suggested to be involved in its neuroprotective action during ischemic and acidic conditions. However, the effect of external pH on DynA inhibition of the NMDA receptor has not been reported. Here, we show that DynA inhibition of the NMDA receptor is dependent on extracellular pH over the range of pH 6.7-8.3, and the inhibition by 10 microM DynA increases at low pH by three- to four-fold in hippocampal neurons and in Xenopus oocytes expressing NR1-1a/2B subunits. Molecular studies showed that the interacting site for DynA on the NMDA receptor is distinct from that of proton or redox sites. Peptide mapping demonstrated important contributions of positively charged residues and specific structural organization of the peptide to the potency of DynA inhibition. Thus, DynA inhibits NMDA receptors through an allosteric mechanism, which is pH dependent and involves the specific structural features of the peptide.     

Remitting seronegative symmetric synovitis with pitting edema: scintigraphic and magnetic resonance imaging findings

Remitting seronegative symmetric synovitis with pitting edema (RS3PE) was first reported by McCarty et al in 1985 and refers to a rheumatologic set of symptoms with acute onset, with no erosive bone lesions, with seronegative findings, affecting the elderly more frequently, and showing an excellent prognosis with low-dose steroid therapy. Although these characteristics make it possible to differentiate it from rheumatoid arthritis and rheumatic polymyalgia, there have been very few reports on the imaging findings. The authors present scintigraphic and magnetic resonance images in a case of RS3PE.     

Impact of interstitial lung disease on surgical morbidity and mortality for lung cancer: analyses of short-term and long-term outcomes

BACKGROUND: This study investigated postoperative morbidity, mortality, and the long-term survival for patients with lung cancer who have interstitial lung diseases. METHODS: A retrospective chart review of 931 patients with lung cancer who underwent pulmonary resection at Chiba University Hospital between 1990 and 2000 was undertaken. Interstitial lung disease was defined by medical history, physical examination, and abnormalities compatible with bilateral lung fibrosis on chest computed tomography or high-resolution computed tomography (36 patients: 3.9%, interstitial lung diseases group). The remaining 895 patients (96.1%) were categorized as non-interstitial lung disease group. RESULTS: The incidence of postoperative pneumonia and acute or exacerbation of interstitial pneumonia was higher in the interstitial lung disease group (all P <.05). Thirty-day mortality was statistically equivalent between the interstitial lung disease and the non-interstitial lung disease groups (P =.30). The 5-year overall survivals were 62.5% (non-interstitial lung disease) and 35.6% (interstitial lung disease). Respiratory failure was the second main cause of death after the recurrence of primary cancer in the interstitial lung disease group. The risk factors for long-term mortality were interstitial lung diseases, advanced pathologic stage, male sex, high age, and positive smoking history (all P <.05). CONCLUSIONS: Interstitial lung disease was a risk factor for developing postoperative morbidity and mortality and poor long-term survival due to the occurrence of respiratory failure.     

Reductions in bone turnover, mineral, and structure associated with mechanical properties of lumbar vertebra and femur in glucocorticoid-treated growing minipigs

The present study was designed to determine the effects of glucocorticoid (GC) on bone turnover, minerals, structure, and bone mechanical properties in minipigs. Six 8-month-old G?ttingen minipigs were subcutaneously injected with prednisolone (PN, 0.5 mg/kg body wt (BW)/day, 5 days/week for 26 weeks (Group GC)), 6 were treated with vehicle alone (Group VC), and 4 were sacrificed at start of the study for baseline controls (Group BC). The increase in BW was similar in all groups. PN significantly reduced serum osteocalcin and urinary type-1 collagen N-telopeptide levels at 13 weeks and thereafter, compared with baseline and control, and also reduced serum bone specific alkaline phosphatase levels relative to baseline. At 26 weeks, the longitudinal axis of the lumbar bone and length of femur were smaller in Group GC than Group VC. The total cross-sectional area of femur, but not the lumbar bone, in Group GC was significantly different from Group VC. BMD of the femur, but not L2, measured by DXA, was lower in Group GC than in Groups BC and VC. The cortical shell structure measured by 2D-micro-CT deteriorated and age-dependent increases in trabecular bone structure 3D micro-CT were reduced by PN. PN also caused deterioration of the cortical structure of the mid-femur. In L2 and femur, PN significantly reduced the ultimate load and maximum absorption energy of the femur and L2 compared with Group VC. The structural modulus in Group GC was lower than in Group BC. Regression analyses revealed that bone minerals, bone structure, and chemical markers correlated with mechanical properties of L2 and mid-femur. Our results indicate that PN reduced systemic bone formation and resorption and suppressed the age-dependent increases in bone minerals, structure, and mechanical properties of L2 and mid-femur. Reduced bone turnover seemed to be associated with a reduction in mechanical properties. The growing minipig could be a suitable model of GCs-induced osteoporosis in humans.     

Optical coherence tomography and focal macular electroretinogram in eyes with epiretinal membrane and macular pseudohole

PURPOSE: To determine the morphology of macular pseudoholes (MPHs) and the relationship of morphology to macular function. DESIGN: Observational case series. METHODS: Optical coherence tomography (OCT) was performed on 42 eyes of 42 consecutive patients with an epiretinal membrane (ERM) and an MPH. The diameters of the MPH, and the thickness of the foveal and parafoveal retina were measured. Of these 42 eyes, focal macular electroretinograms (FMERGs) were recorded from 22 eyes of 22 patients with a 15 degree stimulus; FMERGs were also recorded with a 5 degree stimulus from 9 eyes of these 22 eyes. RESULTS: In 42 eyes, the mean +/- Standard deviation (SD) diameter (437.7 +/- 172.8 microm) and geometrical shape of the MPHs were not significantly correlated with the visual acuity. The MPHs were divided into 2 types from the OCT images at the base of MPHs; group A had normal thickness (100-199 microm; n = 29), and group B (n = 13) had thicknesses of >or= 200 microm, or thickness < 100 microm, or irregular base. The visual acuity in group A (logarithm of the minimum angle of resolution [log MAR] mean +/- SD:.083 +/-.144) was significantly better than group B (log MAR,.407 +/-.212, P <.0001). There was a significant reduction in the amplitude of all components of FMERGs elicited by the 15 degree stimulus in the affected eyes (mean +/- SE, A-wave: 1.26 +/-.12 microv, B-wave: 3.07 +/-.27 microv, oscillatory potentials: 1.23 +/-.25 microv) compared with the normal fellow eyes (A-wave: 1.58 +/-.13 microv, B-wave: 4.14 +/-.27 microv, oscillatory potentials: 2.35 +/-.29 microv). A significant correlation was found between the relative amplitudes of the B-wave elicited by the 5 degree stimulus and the visual acuity (r =.918, P =.0005). CONCLUSIONS: In eyes with an ERM and an MPH, the visual acuity is generally correlated with the OCT images. Macular function of eyes with an MPH resembles eyes with an ERM without an MPH. The effect of the ERM appears to be different on the base and parafovea of the MPHs.