Novel Genome-Wide Interactions Mediated via BOLL and EDNRA Polymorphisms in Intracranial Aneurysm

Objective@#: The association between boule (BOLL) and endothelin receptor type A (EDNRA) loci and intracranial aneurysm (IA) formation has been reported via genome-wide association studies. We sought to identify genome-wide interactions involving BOLL and EDNRA loci for IA in a Korean adult cohort. @*Methods@#: Genome-wide pairwise interaction analyses of BOLL and EDNRA involving 250 patients with IA and 296 controls were performed using the additive effect model after adjusting for confounding factors. @*Results@#: Among 512575 single-nucleotide polymorphisms (SNPs), 23 and 11 common SNPs suggested a genome-wide interaction threshold (p<1.25×10-8) involving rs700651 (BOLL) and rs6841581 (EDNRA). Rather than singe SNP effect of BOLL or EDNRA on IA development, they showed a synergistic effect on IA formation via multifactorial pair-wise interactions. The rs1105980 of PTCH1 gene showed the most significant interaction with rs700651 (natural log-transformed odds ratio [lnOR], 1.53; p=6.41×10-11). The rs74585958 of RYK gene interacted strongly with rs6841581 (lnOR, -19.91; p=1.64×10-9). Although, there was no direct interaction between BOLL and EDNRA variants, two EDNRA-interacting gene variants of TNIK (rs11925024 and rs1231) and FTO (rs9302654), and one BOLL-interacting METTL4 gene variant (rs549315) exhibited marginal interaction with BOLL gene. @*Conclusion@#: BOLL or EDNRA may have a synergistic effect on IA formation via multifactorial pair-wise interactions.

Updated Trans-Ethnic Meta-Analysis of Associations between Inflammation-Related Genes and Intracranial Aneurysm

Objective@#: We performed an expanded multi-ethnic meta-analysis to identify associations between inflammation-related loci with intracranial aneurysm (IA) susceptibility. This meta-analysis possesses increased statistical power as it is based on the most data ever evaluated. @*Methods@#: We searched and reviewed relevant literature through electronic search engines up to August 2022. Overall estimates were calculated under the fixed- or random-effect models using pooled odds ratio (OR) and 95% confidence intervals (CIs). Subgroup analyses were performed according to ethnicity. @*Results@#: Our meta-analysis enrolled 15 studies and involved 3070 patients and 5528 controls including European, Asian, Hispanic, and mixed ethnic populations. Of 17 inflammation-related variants, the rs1800796 locus (interleukin [IL]-6) showed the most significant genome-wide association with IA in East-Asian populations, including 1276 IA patients and 1322 controls (OR, 0.65; 95% CI, 0.56–0.75; p=3.24×10-9) under a fixed-effect model. However, this association was not observed in the European population (OR, 1.09; 95% CI, 0.80–1.47; p=0.5929). Three other variants, rs16944 (IL-1β), rs2195940 (IL-12B), and rs1800629 (tumor necrosis factor-α) showed a statistically nominal association with IA in both the overall, as well as East-Asian populations (0.01

Is the retroauricular region a suitable donor site for full-thickness skin grafting in the era of mask wearing?

Background@#During the coronavirus disease 2019 (COVID-19) pandemic, the everyday use of face masks massively increased to prevent the spread of infection. Many people complain of ear pain and erosion when wearing a mask for extended periods of time. If prolonged mask usage is uncomfortable for ordinary people, a question arises—how much more inconvenient must mask wearing be for patients with a full-thickness skin graft (FTSG) donor site in the retroauricular region? Herein, we evaluated complications related to face mask use in 27 patients with a retroauricular FTSG donor site, with the goal of clarifying whether the retroauricular region is still an appropriate FTSG donor site in the context of the COVID-19 pandemic. @*Methods@#Complications in 27 patients treated by FTSGs harvested from the retroauricular region from 2019 to 2021 were investigated. A questionnaire comparing the normal and the operated sides was administered. Pain, itching, soreness, deformity, and symmetry were further investigated. @*Results@#Pain and itching were the only observed complications. The operated side was slightly more painful than the normal side in four patients (14.8%), and four patients (14.8%) reported itching on the operated side. However, all patients who experienced complications stated that the pain or itching did not interfere with mask wearing. Most symptomatic patients were older than 60 years of age (23.8%, p=0.185) and wore masks for longer periods of time (28.5%, p=0.326). @*Conclusion@#Few retroauricular donor site-related complications occurred, and none of these complications caused inconvenience in daily life. Therefore, in the COVID-19 pandemic, the use of the retroauricular region as an FTSG donor site remains appropriate.

A Case of Recurrent Ischemic Stroke Associated with Immunoglobulin G4-Related Disease

Immunoglobulin G4-related disease (IgG4 RD) is a systemic immune-mediated inflammatory disease that presents as multiple organ dysfunction or mass lesions with lympho-plasmacytic infiltration. However, there are few case reports presenting central nervous system involvements. Herein, we report a case of a 70-year-old man with recurrent ischemic stroke which induced by IgG4 RD. IgG4 RD should be considered and treated as one of etiologies manifesting small vessel disease infarctions, especially when it occurs multiply and recurrently.

The Efficacy of Intra-Articular Hyaluronic Acid Injections in Ankle Osteoarthritis / 대한족부족관절학회지

Purpose@#This study sought to investigate the appropriate indications for intra-articular injection of hyaluronic acid in ankle osteoarthritis, its efficacy at each stage of osteoarthritis and to compare the efficacy related differences at each stage. @*Materials and Methods@#A retrospective study was conducted from January 2016 to September 2019 on 43 patients (50 cases) diagnosed with ankle arthritis and given intra-articular injection of hyaluronic acid. Patients were classified according to the modified Takakura ankle osteoarthritis stages. Stage 2 comprised 24 cases; stage 3A, 15 cases; stage 3B, 8 cases; and stage 4, 3 cases. The clinical evaluation was carried out using the visual analogue scale (VAS), American Orthopaedic Foot and Ankle Society (AOFAS) score, and the Foot and Ankle Outcome Score (FAOS). The patients’ satisfaction with the injection was evaluated at 1 year after injection. A radiographic evaluation was performed to gauge the progression of osteoarthritis before and after the injection. @*Results@#The VAS, AOFAS score, and FAOS at 3 months and 1 year after injection showed statistically significant improvements compared to those before the injection. There was a significant difference in the VAS for each stage over time; however, this was not the case with the AOFAS score and the FAOS. A significant improvement of the VAS after injection was seen only in stage 3A when compared with the other stages. The overall satisfaction rate with the injection was 90%. There were no ankle osteoarthritic stage changes in the serial follow-up radiograph. @*Conclusion@#Intra-articular injection of hyaluronic acid resulted in a significant clinical improvement up to 1 year after the injection.Therefore, the intra-articular injection of hyaluronic acid could be a treatment option for ankle osteoarthritis.

Monitoring Radiation Doses during Diagnostic and Therapeutic Neurointerventional Procedures: Multicenter Study for Establishment of Reference Levels / 신경중재치료의학

Purpose@#To assess patient radiation doses during diagnostic and therapeutic neurointerventional procedures from multiple centers and propose dose reference level (RL). @*Materials and Methods@#Consecutive neurointerventional procedures, performed in 22 hospitals from December 2020 to June 2021, were retrospectively studied. We collected data from a sample of 429 diagnostic and 731 therapeutic procedures. Parameters including dose-area product (DAP), cumulative air kerma (CAK), fluoroscopic time (FT), and total number of image frames (NI) were obtained. RL were calculated as the 3rd quartiles of the distribution. @*Results@#Analysis of 1160 procedures from 22 hospitals confirmed the large variability in patient dose for similar procedures. RLs in terms of DAP, CAK, FT, and NI were 101.6 Gy·cm2, 711.3 mGy, 13.3 minutes, and 637 frames for cerebral angiography, 199.9 Gy·cm2, 3,458.7 mGy, 57.3 minutes, and 1,000 frames for aneurysm coiling, 225.1 Gy·cm2, 1,590 mGy, 44.7 minutes, and 800 frames for stroke thrombolysis, 412.3 Gy·cm2, 4,447.8 mGy, 99.3 minutes, and 1,621.3 frames for arteriovenous malformation (AVM) embolization, respectively. For all procedures, the results were comparable to most of those already published. Statistical analysis showed male and presence of procedural complications were significant factors in aneurysmal coiling. Male, number of passages, and procedural combined technique were significant factors in stroke thrombolysis. In AVM embolization, a significantly higher radiation dose was found in the definitive endovascular cure group. @*Conclusion@#Various RLs introduced in this study promote the optimization of patient doses in diagnostic and therapeutic interventional neuroradiology procedures. Proposed 3rd quartile DAP (Gy·cm2) values were 101.6 for diagnostic cerebral angiography, 199.9 for aneurysm coiling, 225.1 for stroke thrombolysis, and 412.3 for AVM embolization. Continual evolution of practices and technologies requires regular updates of RLs.

A Preliminary Study of the Association between SOX17 Gene Variants and Intracranial Aneurysms Using Exome Sequencing

Objective@#Conflicting results regarding SOX17 genes and the risk of intracranial aneurysms (IA) exist in the Korean population, although significant positive correlations were noted in genome-wide association studies in European and Japanese populations. Therefore, we aimed to investigate an association between SOX17 gene variants and IA using exome sequencing data. @*Methods@#This study included 26 age-gender matched IA patients and 26 control subjects. The SOX17 gene variants identified from whole-exome sequencing data were examined. Genetic associations to estimate odds ratio (OR) and 95% confidence interval (CI) were performed using the software EPACTS. @*Results@#The mean age of the IA and control groups were 51.0±9.3 years and 49.4±14.3 years, respectively (p=0.623). Seven variants of SOX17, including six single nucleotide polymorphisms and one insertion and deletion, were observed. Among these variants, rs12544958 (A>G) showed the most association with IA, but the association was not statistically significant (OR, 1.97; 95% CI, 0.81-4.74; p=0.125). Minor allele frequencies of the IA patients and controls were 0.788 and 0.653, respectively. None of the remaining variants were significantly associated with IA formation. @*Conclusion@#No significant association between SOX17 gene variants and IA were noted in the Korean population. A large-scale exome sequencing study is necessary to investigate any Korean-specific genetic susceptibility to IA.

Development of a Korean Culture-Friendly Olfactory Function Test and Optimization of a Diagnostic Cutoff Value

Objectives@#. Cultural familiarity and safety must be considered when assessing olfactory ability. The YSK olfactory function (YOF) test is a new olfactory function test using culturally familiar odorants to Koreans. @*Methods@#. The YOF test comprises three subtests for threshold (T), discrimination (D), and identification (I). The identification test included eight universal and four Korean culture-friendly odorants, which were selected considering eight major functional groups. Data were obtained from 1,127 subjects over 19 years old. Subjects were classified as having normosmia (n=542), hyposmia (n=472), and anosmia (n=113) by self-reported olfactory function. The YOF test and the Korean version of the Sniffin’ stick test (KVSS-II) were performed on the same day in random order. Diagnostic cutoffs for anosmia and hyposmia were calculated using the Youden index (J). @*Results@#. The mean values for each T/D/I subtest and the total TDI score were as follows: normosmia (T, 4.6±2.3; D, 8.6±2.1; I, 11.1±1.7; TDI score, 24.2±4.5); hyposmia (T, 3.3±2.2; D, 7.1±2.5; I, 9.2±3.1; TDI score, 19.5±6.4); and anosmia (T, 1.7±1.2; D, 5.1±2.5; I, 5.0±3.2; TDI score, 11.8±5.6). The correlation coefficients between the YOF test and KVSS-II were 0.57, 0.65, 0.80, and 0.86 for T, D, I, and the TDI score, respectively (P<0.001). The diagnostic cutoffs were a TDI score ≤14.5 (J=0.67) for anosmia and 14.5(TDI score ≤21.0 (J=0.38) for hyposmia. The diagnostic efficacy of the YOF test (area under the curve [AUC], 0.88) was equivalent to that of the KVSS-II (AUC, 0.88; P=0.843; DeLong method). @*Conclusion@#. The YOF test is a new olfactory test using safe and Korean culture-friendly odorants. It showed equivalent validity with the conventional olfactory function test. Furthermore, the YOF test provides information on the major functional groups of odorants, potentially enabling a more comprehensive interpretation for patients with olfactory disorders.

Congestive Myelopathy Due to Spinal Epidural Arteriovenous Fistula

No abstract available.

Dramatic unilateral decrease in uptake via the dopamine transporter: Imaging in a patient with hemiparkinsonism following the lacunar stroke in substantia nigra

@#Vascular parkinsonism (VaP) is typically defined as having predominant lower body involvement, postural instability, less prominent rest tremor and little or no response to treatment with levodopa. In this study, we report a patient with VaP with clear demonstration of a dramatic unilateral decrease of radiotracer uptake in a 18F-FP-CIT-PET study. A 62-year-old right-handed woman was referred to the neurology department due to rest tremor and rigidity in the right hand, which began after undergoing resection surgery for a left acoustic neuroma 7 years prior. Brain MRI, taken at 1 year after surgery showed an ischemic stroke lesion in the left medial pons and the left substantia nigra. 18F-FP-CIT-PET revealed a marked reduction of radiotracer uptake in left striatum compared to that of the right. We treated the patient with 100 mg of levodopa, 200 mg of entacarpone and 25 mg of carbidopa. There was an improvement in bradykinesia and tremor, but the symptoms persisted, and there was no deterioration during 6 months of observation. After acoustic neuroma surgery, ischemic complications are uncommon, and even a small lesion in the nigrostriatal pathway can cause a hemiparkinsonism. If a patient experience sudden onset hemiparkinsonism, they should be carefully examined for lesions in the nigrostriatal pathways. Under these conditions, the 18F-FP-CIT-PET scan can enable visualization of a unilateral decrease and is a useful tool for diagnosis and differentiation from idiopathic Parkinson’s disease

Prediction of Prolonged Hemodynamic Instability During Carotid Angioplasty and Stenting / 신경중재치료의학

PURPOSE: The aim of this study was to assess the risk factors of prolonged hemodynamic instability (HDI) after carotid angioplasty and stenting (CAS). Herein, a simplified predictive scoring system for prolonged HDI is proposed. MATERIALS AND METHODS: Sixty-six patients who had CAS from 2011 to 2016 at a single institution were evaluated. Prolonged HDI was defined as systolic blood pressure >160 mm Hg or <90 mm Hg or heart rate <50 beats/min, lasting over 30 minutes despite medical treatments. For the study, clinical data and radiologic data, including plaque morphology and stenosis were analyzed. RESULTS: Prolonged HDI was observed in 21 patients (31.8%). Multivariable analysis revealed that calcification (OR, 6.726; p=0.006), eccentric stenosis (OR, 3.645; p=0.047) and extensive plaque distribution (OR, 7.169; p=0.006) were related to prolonged HDI. According to these results, a simplified scoring scale was proposed based on the summation of points: 2 points for calcified plaque, 2 points for extensive plaque distribution, and 1 point for eccentric stenosis. The percentages of prolonged HDI according to the total score were as follows: score 0, 8.7%; score 1, 20.0%; score 2, 38.5%; score 3, 72.7%; score 4, 66.7%; score 5, 100%. From the analysis, the total score in patients with prolonged HDI was significantly higher than those without prolonged HDI (p<0.001). CONCLUSION: Prolonged HDI can be associated with calcification of plaque, eccentric stenosis and extensive plaque distribution, and a simplified scoring system enables prediction of prolonged HDI according to our cohort.

Stent-Assisted Coil Embolization of Vertebrobasilar Dissecting Aneurysms: Procedural Outcomes and Factors for Recanalization

OBJECTIVE: Outcomes of stent-assisted coil embolization (SACE) have not been well established in the setting of vertebrobasilar dissecting aneurysms (VBDAs) due to the low percentage of cases that need treatment and the array of available therapeutic options. Herein, we presented clinical and radiographic results of SACE in patients with VBDAs. MATERIALS AND METHODS: A total of 47 patients (M:F, 30:17; mean age ± SD, 53.7 ± 12.6 years), with a VBDA who underwent SACE between 2008 and 2014 at two institutions were evaluated retrospectively. Medical records and radiologic data were analyzed to assess the outcome of SACE procedures. Cox proportional hazards regression analysis was conducted to determine the factors that were associated with aneurysmal recanalization after SACE. RESULTS: Stent-assisted coil embolization technically succeeded in all patients. Three cerebellar infarctions occurred on postembolization day 1, week 2, and month 2, but no other procedure-related complications developed. Immediately following SACE, 25 aneurysms (53.2%) showed no contrast filling into the aneurysmal sac. During a mean follow-up of 20.2 months, 37 lesions (78.7%) appeared completely occluded, whereas 10 lesions showed recanalization, 5 of which required additional embolization. Overall recanalization rate was 12.64% per lesion-year, and mean postoperative time to recanalization was 18 months (range, 3-36 months). In multivariable analysis, major branch involvement (hazard ratio [HR]: 7.28; p = 0.013) and the presence of residual sac filling (HR: 8.49, p = 0.044) were identified as statistically significant independent predictors of recanalization. No bleeding was encountered in follow-up monitoring. CONCLUSION: Stent-assisted coil embolization appears feasible and safe for treatment of VBDAs. Long-term results were acceptable in a majority of patients studied, despite a relatively high rate of incomplete occlusion immediately after SACE. Major branch involvement and coiled aneurysms with residual sac filling may predispose to recanalization.

A Case of Recurred Cholesteatoma Removal via Middle Cranial Fossa Approach / 대한이비인후과학회지

Cholesteatoma is a benign disease but it has an aggressive feature that may lead to serious conditions. Many strategies have been introduced for the treatment cholesteatoma, yet no definite single method has been established: it should be treated respectively regarding the completion of cholesteatoma, conservation of tympanic mucosa, proper ventilation of middle ear and preservation of hearing. A 25-year old patient visited our clinic for right-sided facial paralysis of 5 days. The patient underwent canal wall down mastoidectomy 10 years ago due to cholesteatoma and a few revision surgeries under local anesthesia for recurrence. Pre-operative temporal bone CT showed suspected recurred cholesteatoma in internal auditory canal and labyrinthine segment of facial nerve nearby. The patient underwent a removal of cholesteatoma via middle cranial fossa approach. This case report is a successful completion of recurred cholesteatoma, for which no recurrence is shown. Facial nerve function is improved at postoperative 1 year.

Use of Triple Microcatheters for Endovascular Treatment of Wide-Necked Intracranial Aneurysms: A Single Center Experience

OBJECTIVE: The dual microcatheter technique is common practice for coil embolization of a wide-necked aneurysm, due to safety and efficacy. However, technical limitations of some complex configurations may necessitate additional microcatheters to bolster coil stability, compact the coil, or for protection. Described herein is a triple microcatheter technique for endovascular management of wide-necked intracranial aneurysms. MATERIALS AND METHODS: Data accruing prospectively between January 2006 and October 2014 on simultaneously executed triple microcatheter coil embolization procedures done in 38 saccular aneurysms were reviewed. Clinical and morphological outcomes were assessed, with emphasis on technical aspects of treatment. RESULTS: The triple microcatheter technique was successfully applied to all 38 saccular aneurysms, involving the posterior communicating artery (n = 13), the middle cerebral artery (n = 10), the basilar tip (n = 7), the anterior cerebral artery (n = 5), and the internal carotid artery (n = 3). Stent protection was added in four patients and balloon remodeling in one. Dual microcatheters (n = 24) were usually deployed to deliver the coil within sacs of aneurysms, with the additional microcatheter used for protection. Otherwise, triple microcatheters were deployed for coil delivery (n = 11) or coils were delivered via a single microcatheter, with dual microcatheters deployed for protection (n = 3). Successful occlusion of aneurysms was achieved in 89.5% of cases, with no procedure-related morbidity or mortality. Stable occlusion was maintained in 72.2% (26/36) of the aneurysms at the final follow-up (mean interval, 30.2 +/- 22.7 months). CONCLUSION: The outcomes of this limited study suggest that the triple microcatheter technique may be an effective and safe therapeutic option for wide-necked aneurysms, using technical strategies tailored to complex angio-anatomic configurations.

Microcatheter Looping to Facilitate Aneurysm Selection in Coil Embolization of Paraclinoid Aneurysms

OBJECTIVE: Described herein is a microcatheter looping technique to facilitate aneurysm selection in paraclinoid aneurysms, which remains to be technically challenging due to the inherent complexity of regional anatomy. MATERIALS AND METHODS: This retrospective study was approved by our Institutional Review Board, and informed consent was waived. Microcatheter looping method was employed in 59 patients with paraclinoid aneurysms between January 2012 and December 2013. In the described technique, construction of a microcatheter loop, which is steam-shaped or pre-shaped, based on the direction of aneurysms, is mandatory. The looped tip of microcatheter was advanced into distal internal carotid artery and positioned atop the target aneurysm. By steering the loop (via inner microguidewire) into the dome of aneurysm and easing tension on the microcatheter, the aneurysm was selected. Clinical and morphologic outcomes were assessed with emphasis on technical aspects of the treatment. RESULTS: Through this looping technique, a total of 59 paraclinoid aneurysms were successfully treated. After aneurysm selection as described, single microcatheter technique (n = 25) was most commonly used to facilitate coiling, followed by balloon protection (n = 21), stent protection (n = 7), multiple microcatheters (n = 3), and stent/balloon combination (n = 3). Satisfactory aneurysmal occlusion was achieved through coil embolization in 44 lesions (74.6%). During follow-up of 53 patients (mean interval, 10.9 +/- 5.9 months), only one instance (1.9%) of major recanalization was observed. There were no complications related to microcatheter looping. CONCLUSION: This microcatheter looping method facilitates safe and effective positioning of microcatheter into domes of paraclinoid aneurysms during coil embolization when other traditional microcatheter selection methods otherwise fail.

A Case of Sudden Deafness with Intralabyrinthine Hemorrhage Intralabyrinthine Hemorrhage and Sudden Deafness

Sudden hearing deterioration may occur in our population, but it is difficult to explain the exact pathophysiology and the cause. Magnetic resonance imaging (MRI) in sudden sensorineural hearing loss (SSNHL) is usually useful to evaluate neural lesions such as acoustic schwannoma and hemorrhage in labyrinth. Recently some cases of SSNHL caused by intralabyrintine hemorrhage were reported by the advance of MRI. In the case of intralabyrintine hemorrhage, MRI showed a hyperintense signal in the labyrinth on the pre-contrast and contrast enhanced T1-weighted image and relatively weak intensity on T2-weighted image. The prognosis SSNHL by intralabyrintine hemorrhage is generally known to be poor. We report a case of sudden deafness with intralabyrintine hemorrhage who has a history of anticoagulant administration, with a review of literature.

Behavioral and Psychological Symptoms in Patients with Parkinson's Disease According to Cognitive Function

BACKGROUND: Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by motor and nonmotor symptoms such as cognitive impairment and neuropsychiatric symptoms. The aim of this study was to investigate the spectrum of neuropsychiatric symptoms in PD according to cognitive function. METHODS: One hundred twenty seven patients with PD were consecutively recruited. They had undergone an intensive interview with a neurologist and the Seoul Neuropsychological Screening Battery-dementia version, and were divided into three groups: 27 patients in PD with normal cognition (PDNC), 57 in PD with mild cognitive impairment (PDMCI), and 43 in PD with Dementia (PDD). Forty five healthy controls without memory complaints were also recruited. The caregivers of all participants administered the Caregiver-Administered Neuropsychiatric Inventory (CGA-NPI). RESULTS: There were significant differences in the CGA-NPI score (22.8+/-20.9 vs. 6.4+/-10.1 vs. 1.7+/-3.9 vs. 1.0+/-1.6, p<0.001), and the prevalence of neuropsychiatric symptoms (100% vs. 64.9% vs. 37.0% vs. 44.4%, p<0.001) among PDD, PDMCI, PDNC and control groups. Depression was the most common symptom (43.3%), followed by anxiety (31.5%), apathy (26.8%), and night-time behavior (26.8%) in all PD patients. Delusion, hallucination, and aberrant motor behavior were observed frequently in PDD, but were rare in PDMCI. CONCLUSIONS: Depression was the most common neuropsychiatric symptom in PD. The presence of delusion, hallucination, or aberrant motor behavior may suggest PDD. The neuropsychiatric symptoms were not prevalent in PD with normal cognition.

Cerebral Venous Thrombosis in the Inferior Sagittal Sinus

No abstract available.

Clinical Features of Polyarteritis Nodosa in Korea

Polyarteritis nodosa (PAN) is a systemic vasculitis characterized by multi-organ involvement with protean manifestations. We evaluated the clinical features of PAN in Korea. Twenty-seven patients were diagnosed as PAN at Seoul National University Hospital between January 1990 and July 2003. The male-to-female ratio was 1.7:1 and mean age at onset (+/-SD) was 47.4+/-20 yr. Their presenting features at diagnosis were similar to those reported previously, i.e., myalgia, muscle weakness or leg tenderness (70%), fever (52%), weight loss >4 kg (44%), skin rash (44%), peripheral edema (33%), abdominal pain (33%), and arthralgia/arthritis (30%). However, the prevalence of testicular pain or tenderness was higher (24%) than reported previously and only three (11.5%) had HBsAg positivity without liver enzyme elevation. Nine patients (33%) had a five-factor score (FFS) of 2. Fourteen patients (52%) responded to treatment, 2 patients relapsed and 4 died within 1 yr of diagnosis. During a median follow-up of 55.5 months, three of the four PAN-related deaths had an initial FFS of 2. The clinical features of PAN were not significantly different from those reported previously. However, testicular pain or tenderness was more frequent and patients with a high FFS tended to have a poorer prognosis.

Successful Treatment of Necrotizing Fasciitis in a Patient with Rheumatoid Arthritis / 대한류마티스학회지

Necrotizing fasciitis is a life-threatening soft tissue infection involving skin, subcutaneous tissue, and superficial fascia. We report a case of necrotizing fasciitis that developed in a 76- year-old female patient taking low-dose methotrexate and prednisolone for rheumatoid arthritis (RA). A computed tomography scan of the neck during the initial work-up showed soft tissue swelling, loss of fat planes, and mild heterogeneous enhancement in the right lateral neck, suggesting cellulitis. The lesions were associated with skin necrosis and multiple bullae rapidly expanded to the right anterior chest in spite of empirical antibiotic therapy. Surgical debridement was immediately performed, and soft tissue biopsy from the lesions showed the pathologic finding consistent with necrotizing fasciitis. The skin wound defect was reconstructed by a flap operation with split-thickness skin graft. This case shows the development of necrotizing fasciitis in a elderly patient taking methotrexate and low-dose steroid for RA and highlights early recognition and prompt surgical debridement for successful management.