Intravenous Steroid Pulse Therapy Research Articles

Venous Thoracic Outlet Syndrome Combined with Brachial Neuritis Caused by Lymphadenopathy after Vaccination for Coronavirus Disease 2019: A Case Report

Thoracic outlet syndrome (TOS) occurs due to compression of the neurovascular bundle exiting the thoracic outlet, through which the brachial plexus and subclavian vessels pass. Here, we report a case of venous TOS combined with brachial neuritis, which was caused by axillary lymphadenopathy after the first dose of the BNT162b2 vaccine against coronavirus disease 2019 (COVID-19). A 17-year-old female patient presented with left upper extremity swelling and pain after inoculation with the BNT162b2 vaccine in the left deltoid muscle. Contrast-enhanced brachial plexus magnetic resonance imaging revealed severe swelling of the left axillary and subclavian lymph nodes, which lie immediately above the subclavian vein. An electrodiagnostic study revealed left brachial plexopathy, mainly involving the lower trunk with mixed demyelinating and axonal injury. The patient received intravenous steroid pulse therapy and oral steroid therapy. A follow-up examination showed complete recovery of muscle strength and function, pain, and swelling in the left upper extremity within 3 months after vaccination against COVID-19.

Optic Chiasmal Enhancement Revealed by Magnetic Resonance Imaging in a Patient with Ethambutol-induced Toxic Optic Neuropathy

Purpose: We report a bitemporal visual field defect accompanied by optic chiasmal enhancement in a patient with ethambutol (EMB)-induced toxic optic neuropathy.Case summary: A 53-year-old woman presented with bilateral visual disturbance that had gradually worsened over the prior 2 months. She had been prescribed 800 mg EMB daily to treat pulmonary tuberculosis for the past 10 months but had stopped EMB 1 month prior because of visual disturbance. She had been prescribed 600 mg rifampicin and 1,000 mg clarithromycin daily for 11 months. Her visual acuity was 0.2 in the right eye and 0.1 in the left. Color vision was reduced. Automated perimetry demonstrated a bitemporal visual field defect, which was more severe in the left eye. Magnetic resonance imaging (MRI) of the optic chiasm revealed contrast enhancement in the body of the chiasm. The optic nerve of the left eye evidenced a higher signal intensity than did that of the right, consistent with the visual field test results. One month after her initial visit, the visual symptoms deteriorated further. We delivered high-dose, intravenous steroid pulse therapy (methylprednisolone 1,000 mg daily for 3 days). At the 1-year follow-up, the visual acuity was 0.4 in the right eye and 0.1 in the left.Conclusions: Bitemporal hemianopia may develop in patients with EMB-induced toxic optic neuropathy. Enhancement of the optic chiasm, consistent with the visual field defect, could be observed in MRI.

Prognostic Factors for Visual Outcomes in Acute Anterior Optic Neuritis Using Optical Coherence Tomography

Purpose: To identify the clinical characteristics and predictors of visual outcomes in acute anterior optic neuritis patients treated with high-dose steroid pulse therapy using spectral domain optical coherence tomography (SD-OCT).Methods: In this study, the medical records of 34 acute anterior optic neuritis patients treated with intravenous high-dose steroid pulse therapy and followed-up for at least 3 months were retrospectively reviewed. The patients were divided into two groups based on the final best-corrected visual acuity (BCVA); group 1 (n = 23) had a final BCVA ≥20/32, while group 2 (n = 11) had a final BCVA <20/32. Clinical characteristics and optical parameters obtained using SD-OCT were evaluated to identify factors influencing the final visual outcome.Results: There were no statistically significant differences in clinical or demographic data between the two groups. Peripapillary retinal nerve fiber layer (pRNFL) swelling at initial presentation was less common (p = 0.015), and the time to pRNFL thickness stabilization was longer (p = 0.016), in group 1. Multivariate logistic regression demonstrated better final visual acuity for patients in whom pRNFL thickness stabilization took longer, as well as in those with a smaller reduction in macular ganglion cell-inner plexiform layer (mGCIPL) thickness, and those without recurrence (p = 0.002, 0.016, and 0.035, respectively).Conclusions: Intravenous high-dose steroid pulse therapy much improved long-term visual outcomes in acute anterior optic neuritis patients with delayed pRNFL thickness stabilization, a smaller mGCIPL thickness reduction, and no recurrence. This suggests that inner retinal layer thickness and recurrence after stabilization may serve as biomarkers for final visual acuity after intravenous steroid treatment in acute anterior optic neuritis.

Clinical Spectrum and Treatment Outcomes of Patients with Developmental and/or Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep

Purpose: Developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep (D/EE-SWAS) is a spectrum of conditions characterized by various phenotypes of cognitive, linguistic, and behavioral regression associated with spike-and-wave activation in sleep. We aimed to investigate the phenotypic spectrum and treatment outcomes of pediatric patients with D/EE-SWAS.Methods: We retrospectively analyzed the medical records of pediatric patients diagnosed with D/EE-SWAS and treated at Severance Children’s Hospital from 2006 to 2022. We extracted information from their medical records on electroencephalography before and after treatment, types of treatment, seizure frequency, and developmental profiles. The primary outcome was reduction of the spike-wave index on electroencephalography after treatment.Results: Twenty-one patients with a median age of 5.3 years (interquartile range, 4.1 to 6.6) at diagnosis were included. Ten patients had delayed development. The patients received various anti-seizure medications. Fourteen received long-term, high-dose steroid therapy, 10 were placed on a ketogenic diet, four received intravenous steroid pulse therapy, and one each was treated with intravenous immunoglobulin and cannabidiol. The most effective treatments were steroid therapy and a ketogenic diet, which were also effective in reducing seizures and improving cognition. Side effects during treatment were transient and treatable.Conclusion: We described the clinical spectrum of pediatric patients with D/EE-SWAS. Steroid therapy and a ketogenic diet can be considered effective therapeutic options for patients with D/EE SWAS.

Pneumocystis Jirovecii Pneumonia in Systemic Lupus Erythematosus: A Nationwide Cohort Study in Taiwan.

To evaluate Pneumocystis jirovecii pneumonia (PJP) infection risk in patients with systemic lupus erythematosus (SLE) in Taiwan. We identified 24,367 patients with SLE from the National Health Insurance research database between 1997 and 2012 and compared the PJP incidence rates (IRs) with those in 243,670 age- and sex-matched non-SLE controls. PJP risk in the patients was evaluated using a Cox multivariate proportional hazards model. The SLE patients exhibited a significantly higher PJP risk than the controls, with an IR of 2.63 per 10,000 person-years and IR ratio of 27.65 (95% confidence interval 17.2-45.3; P < 0.001). Male sex (hazard ratio [HR] 2.42, P < 0.01), end-stage renal disease (ESRD; HR 1.74, P=0.01), recent use of mycofenolate mofetil (MMF; HR 4.43, P < 0.001), intravenous steroid pulse therapy (HR 108.73, P < 0.001), and average oral dosage of >7.5 mg/day prednisolone or equivalent treatment (HR 4.83, P < 0.001) were associated with PJP in SLE, whereas hydroxychloroquine use reduced its risk (HR 0.51, P=0.01). Of note, cyclophosphamide was not associated with PJP infection in the multivariate Cox proportional hazard model. Patients with SLE have a considerably high PJP risk. Cyclophosphamide does not increase PJP risk. Male sex, ESRD, MMF use, intravenous steroid pulse therapy, and oral prednisolone or equivalent treatment (>7.5 mg/day) are risk factors for PJP, whereas hydroxychloroquine use reduces PJP risk.

Optic neuritis as the ocular manifestation of dengue infection: a case report

Optic neuritis is characterized by decreased vision, impaired colour perception, relative afferent pupillary defects, and scotoma. Optic neuritis following dengue infection is rare and might be underdiagnosed. The pathophysiology of optic neuritis after dengue infection is still unclear and there are only a few reports. We report a case of bilateral simultaneous optic neuritis in a young female adult following dengue haemorrhagic fever. On presentation, she complained of blurred vision, pain around the eyes, central scotoma, and progressively worsening visual acuity to no perception of light in both eyes. The next day, laboratory examination showed leucopoenia (6.74 [4.5–11.5 103/μL]) and lymphocytosis (52.7 [18–42%]), suggesting viral infection with positive anti-dengue IgM and IgG. The patient received intravenous pulse steroid therapy according to the Optic Neuritis Treatment Trial. At 3 months follow-up, best corrected visual acuity improved to 6/6, with pale optic discs but normal perimetry. Although complications of dengue fever in the eye are rare, early recognition must be established to prevent permanent vision loss.

Application of Magnetic Resonance Imaging in the Evaluation of Disease Activity in Graves’ Ophthalmopathy

ObjectiveTo evaluate magnetic resonance imaging parameters, T2 signal intensity ratios (SIRs), and normalized apparent diffusion coefficients (n-ADC) of the extraocular muscles (EOMs) in the identification of different stages of Graves’ ophthalmopathy (GO) and to find out the correlation of T2-SIRs and n-ADC values with disease changes after anti-inflammatory treatment. MethodsAltogether, 43 patients (86 orbits) were enrolled and classified into “active” or “inactive” stages by clinical activity score (CAS). Twenty-three (53.5%) patients received anti-inflammatory treatment and underwent a follow-up evaluation. Fifteen age- and gender-matched control participants (30 orbits) were included. T2-SIRs and n-ADC values of EOMs were calculated among GO and healthy controls and were correlated with CAS. Changes in these parameters were also evaluated before and after anti-inflammatory treatment. ResultsMean T2-SIRs and n-ADC values were both significantly higher in GO patients than in controls and higher in active GO than in inactive GO. In the inactive stage, n-ADC values of inferior rectus muscles were still higher than those in healthy controls. Both T2-SIRs and n-ADC values decreased after intravenous steroid pulse therapy. The cutoff value of pretreatment n-ADC was 1.780 to detect stages with specificity of 93.7% and sensitivity of 48.3% (P = .035). ConclusionT2-SIRs and n-ADC values are valuable magnetic resonance imaging indicators of the inflammatory activity in GO by detecting involvement of EOMs. They are also ideal tools to monitor the efficacy of anti-inflammatory treatment in patients with active stage GO. n-ADC values, when combined with CAS, can be promising predictive factors in the detection of stages of diseases.

Fulminant myocarditis triggered by OC43 subtype coronavirus: a disease deserving evidence-based care bundles

Fulminant myocarditis triggered by OC43 subtype coronavirus: a disease deserving evidence-based care bundles

Successful treatment of a 12-year-old boy with Guillain-Barré syndrome requiring tracheostomy due to respiratory muscle paralysis: A case report.

Childhood Guillain-Barré syndrome (GBS) occasionally leads to respiratory failure early after onset, requiring long-term ventilation management after tracheal intubation. However, patients requiring tracheostomy management are rare. In the present study, a case of a 12-year-old boy with GBS who required artificial respiration management due to rapid progression of respiratory muscle paralysis is reported. Intravenous immunoglobulin (IVIg) and pulse steroid therapy were provided; however, both were ineffective and tracheostomy was necessary 26 days after onset. A second course of IVIg and pulse steroid therapy was administered on day 34. With continued rehabilitation, the patient was able to walk long distances on day 74 and was subsequently discharged on day 89. In cases of severe GBS, when IVIg and pulse steroid therapy do not improve the respiratory muscle strength of the patient, early tracheostomy may improve the patient's quality of life during artificial respiration management.

Impact of treatment on prognosis of inflammatory myopathy-associated cardiomyopathy

Objective To observe the treatment of inflammatory myopathy-associatied cardiomyopathy and its impact on prognosis of disease. Methods In this single-center retrospective study, 29 cases of inflammatory myopathy-associated cardiomyopathy were collected in Peking Union Medical College Hospital from 1999 to 2016. The clinical data and adverse events during follow up were documented. Among 29 patients there were 11 cases of polymyositis, 8 cases of dermatomyositis, 8 cases of overlap syndrome and 2 cases of nonspecific myositis.All the patients started with sufficiene prednisone (1-2 mg·kg-1·d-1). 7 cases received intravenous immune globulin, while 12 cases were prescribed with steroid pulse therapy, 16 cases with methotrexate, 15 cases with cyclophosphamide, 6 cases with cyclosporine A, while 11 cases with combination of immune suppressors (methotrexate plus cyclophosphamide or cyclosporine A). After a median follow up of 4.8 years (2 month to 15 years), 14 cases died including 9 of cardiac death. Patients with cardiac deaths had lower usage percentages of intravenous gamma globulin (0 vs. 7/20, P=0.05) and steroid pulse therapy (1/9 vs. 11/20, P=0.043) than controls. Comparing with controls, patients in the group of adverse events were more prone to choose methotrexate alone rather than combination of immune suppressors (all-cause death: 1/14 vs. 8/15, P=0.014; cardiac death: 0 vs. 9/20, P=0.027). Kaplan-Meier survival analysis showed significant difference of survival rates between patients with combination of immune suppressors and controls (Log Rank χ2=6.001, HR=7.58, P=0.014), as well as between patients with β receptor blockers and controls (Log Rank χ2=4.589, HR=2.95, P=0.032). Conclusions We recommend a management strategy that emphasize both primary disease controlling and cardiac remodeling improvement in patients with inflammatory myopathy-associated cardiomyopathy. On the basis of sufficient glucocorticoids, intravenous gamma globulin, steroid pulse therapy, combination of methotrexate plus other immune suppressants, and β receptor blockers were worthy to be considered. Key words: Inflammatory myopathy; Cardiomyopathy; Heart failure; Treatment; Prognosis

Risk of severe herpes simplex virus infection in systemic lupus erythematosus: analysis of epidemiology and risk factors analysis in Taiwan

ObjectivePatients with systemic lupus erythematosus (SLE) are susceptible to herpes simplex virus (HSV) infection, which occasionally leads to severe complications including meningoencephalitis and keratitis. However, few attempts to analyse the...

Interstitial pneumonia suspected during regorafenib administration and exacerbated by subsequent therapy with lenvatinib for unresectable hepatocellular carcinoma

Recently, three tyrosine kinase inhibitors (TKIs) have become available for treatment of unresectable hepatocellular carcinoma (HCC). We herein report a case of a 59-year-old man with interstitial pneumonia that was suspected during regorafenib administration and was exacerbated by subsequent lenvatinib treatment for advanced HCC. After sorafenib was discontinued due to progressive HCC, regorafenib treatment was started. Progressive HCC was again noted and reticular shadows were suspected in both lower lung fields at 2months after starting regorafenib administration. Subsequent treatment with lenvatinib obtained a partial response for HCC, but the reticular shadows became marked and dyspnea on effort emerged, followed by hypoxemia and an increased Krebs von den Lungen-6 (KL-6) value. Because we suspected acute interstitial pneumonia, due to these TKIs, intravenous pulse steroid therapy was started immediately after discontinuing lenvatinib. Within 1 week after starting steroid therapy, the patient's respiratory condition and hypoxemia gradually began improving. No previous case of pulmonary interstitial changes that appeared in association with regorafenib administration for HCC and that were exacerbated by subsequent treatment with lenvatinib has been reported. This case emphasizes that it is necessary to observe the patient's respiratory condition and to perform imaging examinations to monitor for adverse events during TKI treatment.

Drug-induced acute interstitial nephritis: Prospective randomized trial comparing oral steroids and high-dose intravenous pulse steroid therapy in guiding the treatment of this condition.

The most important aspect of treating drug-induced acute interstitial nephritis (AIN) is timely discontinuation of the offending drug. Steroids, oral as well as intravenous (IV), are used in the treatment of drug-induced AIN. The present study was undertaken to compare the efficacy of oral prednisolone versus IV suprapharmacological doses of corticosteroids in the treatment of drug-induced AIN. This prospective randomized controlled study included drug-induced AIN diagnosed on histopathology over a period of two years. Patients were randomized to oral prednisolone (Group A) 1 mg/kg for two weeks or pulse methylprednisolone (Group B) 30 mg/kg for three days (maximum 1 g) followed by oral prednisolone 1 mg/kg for two weeks, tapered over two weeks. Response was reported as complete remission (CR) [improvement in estimated glomerular filtration rate (eGFR) to ≥60 mL/min/1.73 m2], partial remission (PR) (improvement but eGFR <60 mL/min/1.73 m2), or nonresponders to steroids (no CR/PR). Steroid therapy was instituted to 31 biopsy-proven AIN cases (Group A - 16 and Group B - 15). Drugs implicated in the causation of AIN included pantoprazole, diclofenac, rifampicin, naproxen, aspirin, imipenem, piroxicam, cefixime, lornoxicam, Chinese herbs, etoricoxib, ciprofloxacin, and phenytoin. There was no difference in the baseline parameters between the two groups. At the end of follow-up, 58.06% achieved CR and 41.93% achieved PR. In Group A, nine (56.2%) achieved CR and seven (43.7%) achieved PR. In Group B, nine (60%) achieved CR and six (40%) achieved PR. There was no significant difference between the two groups. Pulses of high doses of corticosteroids have a significant but transient anti-inflammatory effect. Both oral and IV suprapharmacological doses of corticosteroids are equally effective in the treatment of drug-induced AIN, if used early.

Clinical profile of patients with posterior scleritis: A report from Eastern India.

Purpose:This study aimed to report the clinical profile of patients with posterior scleritis at a tertiary eye center in Eastern India.Methods:This was a single-center retrospective case series of patients who were diagnosed as posterior scleritis between January 2010 and December 2014, with a follow-up period of at least 6 months.Results:The study included 18 patients of posterior scleritis with a mean age of 41.2 ± 10.6 years (range: 26–63 years). With female preponderance (55.6%), majority of the posterior scleritis cases were unilateral (88.9%). Sixteen patients reported with diminution of vision, eleven patients (61.1%) had ocular pain on presentation, and five patients complained of headache. Concurrent anterior scleritis was found in three eyes (15%) with posterior scleritis. Choroidal folds and subretinal fluid at the posterior pole were the most common fundus findings and were seen in seven eyes (35%) each. No systemic association was detected in any patient even after extensive laboratory workup and multidisciplinary consultation. All patients received oral steroid, and 11 (61.1%) of them required intravenous pulse steroid therapy. Immunosuppressive was used in 6 (33.3%) patients, and oral azathioprine was the most common immunosuppressive used in the study. Recurrence was noted in eight eyes (40%). The mean best-corrected visual acuity improved to logarithm of the minimal angle of resolution (logMAR) 0.06 ± 0.051 at the final follow-up from 0.47 ± 0.45 logMAR at presentation (P = 0.00608).Conclusion:Posterior scleritis is relatively rare but can occur without systemic involvement. Aggressive immunomodulatory therapy is required to treat vision-threatening condition.

Use of Single- or Two-dose Pulse Methylprednisolone in the Treatment of Acute Immune Thrombocytopenic Purpura.

Objectives:In immune thrombocytopenic purpura (ITP) treatment, the main goal is achieving the platelet level most rapidly for hemostasis. Pulse steroid therapy is common due to the rapid increase in the platelet count within the first 48 hours. Intravenous (IV) pulse steroid therapy is usually administered as a single methylprednisolone dose in the morning. Oral methylprednisolone is generally used as two divided doses due to its half-life, but there is no efficacy study for the use of pulse methylprednisolone therapy in two doses. In this study, we aimed to investigate whether the administration of single or double doses of pulse steroid treatment, which is the cheapest and most economical way to treat patients, differ in terms of platelet count increase rate.Methods:The diagnosis of acute ITP was made based on the appropriate clinical, laboratory, and bone marrow findings and platelet count <100.000/mm3. All the patients were diagnosed with bone marrow aspiration, and they were admitted to the hospital. All patients with platelet counts below 20000/mm3 and those who had wet purpura or active bleeding were treated. Patients in need of treatment were randomly divided into two treatment groups with closed envelope method. The first group was given IV pulse methylprednisolone (30 mg/kg/day for three days and 20 mg/kg/day for four days) in the early morning hours. The second group received the same daily dosages in two divided doses. Hemoglobin, white blood cell, and platelet counts were evaluated before and on the first, second, third, fifth, and seventh days of treatment. To evaluate the rate of treatment response, platelet counts over 20.000/mm3, 50.000/mm3, and 100.000/mm3 obtained on the first, second, third, and seventh days of treatment were compared.Results:Sixty patients with acute ITP diagnosis receiving pulse steroid therapy were included in the study. Platelet counts of the patients in group 2, who received pulse steroids in two doses, reached ≥20.000/mm³ on the second day [median, (2–3) days], ≥50.000/mm³ on the third day [median, (2.7–3.5) days], ≥100.000/mm³ on the fifth day [median, (3–5) days], which were significantly lower than the platelet counts of the patients in the first group on the third day [median, (2–5) days], fifth day [median, (4–7) days], and seventh day [median, (4–7) days], respectively (p<0.001, p<0.001, p=0.004).Conclusion:This study shows that administration of IV pulse steroid therapy in two doses is more effective in increasing the platelet count in early period in patients with acute ITP, especially whose platelet count is less than 20.000/mm³, and when we prefer to increase the platelet counts rapidly due to risk of intracranial hemorrhage.

High-dose intravenous steroid pulse therapy in ocular involvement of Behcet's disease: a pilot double-blind controlled study.

To evaluate the efficacy of intravenous high-dose pulses of methylprednisolone (IVPM) for treatment of ocular involvement in Behcet's disease (BD). In a double-blind control study, we randomized BD patients with posterior uveitis (PU) and/or retinal vasculitis (RV) into two groups. They received either IVPM (1000 mg methylprednisolone) or placebo for 3 consecutive days. Both groups received combination therapy with IV cyclophosphamide, azathioprine and prednisolone for 6 months. Visual acuity (VA), Disease Activity Index (DAI) based on the inflammatory state of each section of each eye, total inflammatory (TIAI) and adjusted DAI (TADAI) for each patient were calculated. The comparisons were done by paired t- and Mann-Whitney U-test. Seventeen patients in each group completed the treatment. The mean VA improved from 0.5 to 0.8 (P < 0.000001) for the study and from 0.6 to 0.7 (P < 0.02) for the placebo group. The difference was significant (P = 0.01). The comparison showed no significant difference regarding DAI improvement in other items (P > 0.2): PU, 1.9 to 0.5 (P < 0.0006) versus 2.3 to 0.8 (P < 0.0002); RV: 4.0 to 1.1 (P < 0.0004) versus 3.1 to 1.1 (P < 0.0005); TIAI: 23 to 5.7 (P < 0.0002) versus 24.8 to 8.4 (P < 0.003); TADAI: 24.1 to 7.3 (P < 0.0002) versus 25.9 to 7.9 (P < 0.004). We had one flare in the study versus seven in the placebo group (P < 0.005). Adding high-dose intravenous steroid pulse therapy to conventional combination therapy for severe ocular lesions of BD may cause better improvement on VA and fewer flares during the first 6 months of treatment.

Efficacy of mizoribine and prednisolone combination therapy in adult patients with IgA vasculitis.

Immunoglobulin (Ig)A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is one of the most common vasculitis caused by an IgA-mediated immune complex. It occurs most frequently in childhood and less commonly in adulthood. As for the treatment of IgAV in adults, there are few studies dealing with the administration and efficacy of intravenous pulse steroid therapy or combination therapy using prednisolone (PSL) and immunosuppressive drugs. Mizoribine (MZB) is a newly developed immunosuppressive drug with few adverse effects; however, there are currently few studies using MZB in adult patients with IgAV. In this study, we evaluated the efficacy of MZB combined with a course of PSL in adult patients with IgAV. Five patients with adult onset IgAV were enrolled in the study. All patients received oral PSL (initial dose 30-50mg/day), and MZB was administered orally at a single morning dose of 150mg. We investigated the clinical manifestations and prognosis of these patients receiving the combination therapy of MZB and PSL retrospectively. All patients showed complete or partial remission of proteinuria and microscopic hematuria with the combination therapy of MZB and PSL. Furthermore, no significant adverse effects were observed. Although this study had an uncontrolled small group, our results indicate that the combination of MZB with PSL could be a possible new treatment for adult patients with IgAV.

Leptospirosis in a child with acute respiratory distress syndrome

Yeşilbaş O, Şevketoğlu E, Kıhtır HS, Hatipoğlu N, Yıldırım HM, Akyol MB, Aktay-Ayaz N, Gökçe İ. Leptospirosis in a child with acute respiratory distress syndrome. Turk J Pediatr 2017; 59: 688-692. Leptospirosis is an infectious vasculitis, which can occur with different clinical features. While it is generally a subclinical and self-limited infection; kidney and liver dysfunction, pulmonary hemorrhage, thrombocytopenia, and cardiovascular collapse may occurr. A six-year-old boy presented with acute respiratory distress syndrome, shock, thrombocytopenia-associated multiple organ failure, and persistent high fever secondary to leptospirosis. Persistent high fever was resistant to intravenous immunoglobulin and pulse steroid therapy. He was successfully treated with plasmapheresis and hemofiltration with endotoxin-cytokine cleaning filter. In conclusion; leptospirosis may cause thrombocytopenia-associated multiple organ failure, persistent high fever, and acute respiratory distress syndrome. Plasmapheresis and hemofiltration should be considered in cases of severe leptospirosis with multiorgan failure.

Steroid pulse therapy prevents restenosis following balloon dilatation for esophageal stricture.

This study aimed to evaluate the effectiveness of intravenous steroid pulse therapy following balloon dilatation for esophageal stenosis and stricture in children. The study enrolled six children, including three with congenital esophageal stenosis and three with anastomotic strictures after surgery for esophageal atresia, all of whom were treated by balloon dilatation combined with high-dose intravenous methylprednisolone pulse therapy. Methylprednisolone was injected intravenously at a dose of 20mg/kg/day for 2days, starting from the day of dilatation, followed by 10mg/kg/day for 2days, for a total of 4days. Esophageal stricture recurred in all three patients with congenital esophageal stenosis despite repeated balloon dilatation without methylprednisolone. However, the symptoms of dysphagia improved and did not recur after systemic steroid pulse therapy following balloon dilatation. Symptoms also resolved in all three patients with anastomotic strictures following balloon dilatation with systemic steroid pulse therapy. All six patients remained asymptomatic after 6-21months follow-up, with no complications. Intravenous methylprednisolone pulse therapy following balloon dilatation is safe and effective for the treatment of esophageal stenosis and strictures in children.

Steroid-Responsive Reversible Cerebral Vasoconstriction Syndrome

A 50-year-old woman with thunderclap headache and transient neurologic symptoms was diagnosed with reversible cerebral vasoconstriction syndrome. Serial angiography showed multifocal arterial vasoconstrictions, and MRI revealed multiple scattered cerebral infa rctions. New neurologic symptoms and vasoconstriction in other arteries occurred repeatedly despite the on-going administration of traditional treatments. However, the symptoms reduced dramatically after administering intravenous steroid pulse therapy. This case suggests that steroid therapy can be considered as an alternative treatment when reversible cerebral vasoconstriction syndrome is intractable to traditional treatment. J Korean Neurol Assoc 34(1):33-36, 2016