Epstein-Barr virus-related encephalitis in a young woman: A case report.

Although infectious mononucleosis due to Epstein-Barr virus (EBV) is a common disease among young individuals, central nervous system (CNS) complications are rare. In this report, we describe a case of CNS complications caused by EBV in a previously healthy young woman. She presented to our hospital with a 9-day history of headache and sore throat, followed by the development of fever and facial edema 6 days prior to admission. On Day 2 of admission, she was confused (Glasgow Coma Scale score: 10 points) and had fever, muscle weakness in her right arm and leg, stiff neck, and roving eye movement. We detected EBV in a cerebrospinal fluid (CSF) sample using a polymerase chain reaction (PCR) test. The magnetic resonance imaging of her brain revealed dural enhancement and right parietal and temporal lobe lesions. She was treated with acyclovir and high-dose steroid therapy. She responded well to treatment, recovered without neurologic sequelae, and was discharged home on Day 12. Our experience suggests that PCR detection of EBV DNA in CSF may be useful in diagnosing EBV encephalitis and that prognosis may be associated with an area of the brain that is affected and the time from symptom onset to starting treatment.

Association between position of the fixed ulnar head and carpal translocation after the Sauvé-Kapandji procedure in patients with rheumatoid arthritis.

OBJECTIVE: The Sauvé-Kapandji procedure is a common surgical procedure for rheumatoid wrist, which involves fixing dissected ulnar head to the distal radius in order to provide "bony support" to the carpus. The purpose of this study was to investigate whether the position of the fixed ulnar head was associated with postsurgical carpus translocation. METHODS: We retrospectively reviewed radiographs of 40 patients who underwent the Sauvé-Kapandji procedure and were subsequently followed up for over two years. The association between the fixed ulnar head position and postsurgical carpus translocation was statistically analysed with a confidence interval of 95% (p < 0.05). RESULTS: Multiple regression analysis suggested that the radial inclination of the fixed ulnar head, the absence of increases in ulnar variance, and wide "bony support" were significantly associated with less postsurgical carpal translocation. CONCLUSION: Our study indicated that good concordance between the "bony support" and the carpus might be important in reducing postsurgical carpus translocation.

Modified metacarpal shortening osteotomy of the midcarpal bone for preserving metacarpophalangeal joints in patients with rheumatoid arthritis.

Recent advances in medication choices have strikingly improved the management of rheumatoid arthritis. However, medication alone cannot place back already deformed joints. Thus, to prevent metacarpophalangeal (MP) joint destruction, joint deformity correction should be considered since mechanical stress induced by finger motions will eventually destruct the undestructed joint, with a possibility of recurrence and future implant arthroplasty in mind since RA still remains as a progressive disease. We report a modified metacarpal shortening osteotomy for correcting MP joint deformity. The advantage of our technique over previous osteotomies is that it easily allows for subsequent implant arthroplasty even after the recurrence of joint deformity/destruction. Major modifications include that the metacarpal is shortened at its mid-shaft and the osteotomy is performed vertical to the shaft and fixed with surgical wiring. We believe that combination therapy consisting of medication and surgery is preferable to prevent joint destruction, even in this age of biological agents.

Rheumatoid Factor Is Associated With the Distribution of Hand Joint Destruction in Rheumatoid Arthritis.

OBJECTIVE: Rheumatoid arthritis (RA) is a chronic disease leading to joint destruction. Although many studies have addressed factors potentially correlated with the speed of joint destruction, less attention has been paid to the distribution of joint destruction in patients with RA. In this study, destruction of the hand bones in patients with RA was classified into 2 anatomic subgroups, the fingers and the non-fingers, with the aim of analyzing which factors are associated with destruction of the finger joints. METHODS: A total of 1,215 Japanese patients with RA were recruited from 2 different populations. The degree of joint destruction was assessed using the total modified Sharp/van der Heijde score (SHS) of radiographic joint damage. The SHS score of joint damage in the finger joints was used as the dependent variable, and the SHS score in the non-finger joints was used as a covariate. Age, sex, disease duration, smoking, C-reactive protein level, treatment for RA, and positivity for and levels of anti-citrullinated protein antibodies and rheumatoid factor (RF) were evaluated as candidate correlates. Overall effect sizes were assessed in a meta-analysis. In addition, associations observed in the Japanese patients were compared to those in a cohort of 157 Dutch RA patients in the BeSt study (a randomized, controlled trial involving 4 different strictly specified treatment strategies for early RA). RESULTS: Not surprisingly, disease duration in Japanese patients with RA was associated with the finger SHS score (P ≤ 0.00037). Both positivity for and levels of RF showed significant associations with the finger SHS score after adjustment for covariates (P = 0.0022 and P = 8.1 × 10(-7) , respectively). These associations were also true in relation to the time-averaged finger SHS score. An association between RF positivity and the finger SHS score was also observed in Dutch patients with RA in the BeSt study (P = 0.049). CONCLUSION: Positivity for and levels of RF are associated with finger joint destruction independent of non-finger joint destruction and other covariates. Our findings suggest that there are different mechanisms of joint destruction operating in the finger joints of patients with RA.

[Anesthetic Management of a Patient with Corrected Transposition of Great Arteries for Cesarean Section].

A 30-year-old woman with corrected transposition of great arteries (c-TGA) was scheduled for elective cesarean section at 37 weeks of gestation. At previous cesarean section, she received general anesthesia for dyspnea and lower cardiac function by severe mitral regurgitation, with a pulmonary catheter inserted. In the current pregnancy, she had tricuspid regurgitation, but she had no signs of heart failure. Cardiac index (CI) and stroke volume variation (SVV) were monitored by the FloTrack, before induction of anesthesia. Because the CI was 3.6 l x min(-1) x m(-2), and the SVV was 18%, we decided to perform combined spinal epidural anesthesia. Epidural anesthesia was performed at L1-2, and spinal anesthesia was performed at L3-4. Hyperbaric 0.5% bupivacaine 2.0 ml with fentanyl 10 µg was given to the subarachnoid space. The total dose of phenylephrine administered was 150 µg, and the CI as well as the SVV were stable during surgery. Her postpartum couse was uneventful. Anesthetic management of c-TGA is discussed, and we should select anesthetic method carefully.

Validity and responsiveness of a self-administered foot evaluation questionnaire in rheumatoid arthritis.

OBJECTIVES: A self-administered foot evaluation questionnaire (SAFE-Q) was developed by the Japanese Society for Surgery of the Foot (JSSF). The aim of this study is to evaluate the validity and responsiveness of the SAFE-Q in patients with rheumatoid arthritis (RA). METHODS: In total, 180 patients with RA answered the SAFE-Q. Of 180 patients, 34 answered the SAFE-Q twice, preoperatively and postoperatively, to assess responsiveness. Construct validity was tested by comparing the 5 SAFE-Q subscales and the JSSF standard rating system for the RA foot and ankle scale (JSSF-RA), a Japanese version of the Health Assessment Questionnaire (JHAQ), disease activity score in 28 joints (DAS28), simplified disease activity index (SDAI), and clinical disease activity index (CDAI). Responsiveness was examined by calculating the standardized response mean (SRM) and effect size (ES) 3 months after surgery. RESULTS: There were moderate correlations between the SAFE-Q and the JSSF-RA and JHAQ. Conversely, a low correlation was observed between the SAFE-Q and DAS28, SDAI, and CDAI. The responsiveness was high, with an SRM of 0.9 and ES of 0.7 for pain subscales. CONCLUSION: SAFE-Q is a useful tool for assessing the foot and ankle in RA patients.

An osteoprotegerin gene polymorphism is associated with an increased risk of hip fracture in Japanese patients with rheumatoid arthritis: results from the IORRA Observational Cohort Study.

INTRODUCTION: Patients with rheumatoid arthritis (RA) have a higher prevalence of osteoporosis and hip fracture than healthy individuals. Multiple genetic loci for osteoporotic fracture were identified in recent genome-wide association studies. The purpose of this study was to identify genetic variants associated with the occurrence of hip fracture in Japanese patients with RA. METHODS: DNA samples from 2,282 Japanese patients with RA were obtained from the DNA collection of the Institute of Rheumatology Rheumatoid Arthritis cohort (IORRA) study. Six single nucleotide polymorphisms (SNPs) that have been reported to be associated with fractures in recent studies were selected and genotyped. Forty hip fractures were identified with a maximum follow-up of 10 years. The genetic risk for hip fracture was examined using a multivariate Cox proportional hazards regression model. RESULTS: The risk analyses revealed that patients who are homozygous for the major allele of SNP rs6993813, in the OPG locus, have a higher risk for hip fracture (hazard ratio [95% CI] = 2.53 [1.29-4.95], P = 0.0067). No association was found for the other SNPs. CONCLUSIONS: Our results indicate that an OPG allele is associated with increased risk for hip fracture in Japanese patients with RA.

Recent trends in orthopedic surgery aiming to improve quality of life for those with rheumatoid arthritis: data from a large observational cohort.

OBJECTIVE: To describe current trends in the numbers of rheumatoid arthritis (RA)-related surgeries. METHODS: The number of operations was determined for patients with RA in a large observational cohort [Institute of Rheumatology, Rheumatoid Arthritis (IORRA)] enrolled from 2001 to 2012. RESULTS: The total number of operations peaked in 2002 and gradually decreased thereafter, but began to increase again in 2008. The number of total knee replacements has decreased since 2003, while the number of wrist and foot arthroplasties and the number of artificial finger prosthesis surgeries have increased gradually. CONCLUSION: Our results suggest that the number of orthopedic surgeries may change in response to changes in the drug therapy for RA.

Number of ruptured tendons and surgical delay as prognostic factors for the surgical repair of extensor tendon ruptures in the rheumatoid wrist.

OBJECTIVE: Extensor tendon ruptures in the rheumatoid wrist are usually restored by extensor tendon reconstruction surgery. However, the factors significantly correlated with the outcomes of extensor tendon reconstruction have not been defined. We examined factors showing a statistically significant correlation with postoperative active motion after tendon reconstruction. METHODS: Spontaneous extensor tendon ruptures of 66 wrists in patients (mean age, 52.6 yrs) with rheumatoid arthritis (RA) were evaluated. All patients underwent tendon reconstruction surgery with wrist arthroplasty or arthrodesis. Active ranges of motion of the affected fingers were evaluated at 12 weeks postsurgery. Statistical significance was determined using multiple and single regression analyses. RESULTS: Forty-six (69.6%) wrists had "good" results, while 13 (19.7%) and 7 (10.6%) wrists had "fair" and "poor" results, respectively. In multiple regression analysis, an increased number of ruptured tendons and the age at operation were independent variables significantly correlated with the postoperative active motion of reconstructed tendons (p = 0.009). Single regression analysis also showed a significant association between the number of ruptured tendons and surgical delay (p = 0.02). CONCLUSION: The number of ruptured extensor tendons was significantly correlated with the results of tendon reconstruction, and the number of ruptured tendons was significantly correlated with preoperative surgical delay. Our results indicate that, in patients presenting with possible finger extensor tendon rupture, rheumatologists should consult with hand surgeons promptly to preserve hand function.

Genetics of rheumatoid arthritis contributes to biology and drug discovery.

A major challenge in human genetics is to devise a systematic strategy to integrate disease-associated variants with diverse genomic and biological data sets to provide insight into disease pathogenesis and guide drug discovery for complex traits such as rheumatoid arthritis (RA). Here we performed a genome-wide association study meta-analysis in a total of >100,000 subjects of European and Asian ancestries (29,880 RA cases and 73,758 controls), by evaluating ∼10 million single-nucleotide polymorphisms. We discovered 42 novel RA risk loci at a genome-wide level of significance, bringing the total to 101 (refs 2 - 4). We devised an in silico pipeline using established bioinformatics methods based on functional annotation, cis-acting expression quantitative trait loci and pathway analyses--as well as novel methods based on genetic overlap with human primary immunodeficiency, haematological cancer somatic mutations and knockout mouse phenotypes--to identify 98 biological candidate genes at these 101 risk loci. We demonstrate that these genes are the targets of approved therapies for RA, and further suggest that drugs approved for other indications may be repurposed for the treatment of RA. Together, this comprehensive genetic study sheds light on fundamental genes, pathways and cell types that contribute to RA pathogenesis, and provides empirical evidence that the genetics of RA can provide important information for drug discovery.

[A case of suspected dry tap during spinal anesthesia for caesarean section].

Spinal anesthesia was attempted in a 21-year-old woman for acute cesarean section with lumbar puncture at L3-4 and L4-5 in another hospital, but it was abandoned after more than 10 attempts because no cerebrospinal fluid (CSF) was seen flowing. She was transferred to our hospital, and we attempted spinal anesthesia at L2-3 and CSF was seen flowing. Although 0.5% hyperbaric bupivacaine 2.0ml was injected, the anesthetic effect was insufficient. At this point we injected 0.5% hyperbaric bupivacaine 1.5 ml in the same space and she developed sensory block up to T3. Surgery proceeded uneventfully. There were no postoperative neurological complications related to spinal anesthesia.