High-dose Intravenous Methylprednisolone Research Articles

MOG Antibody-Positive Myelopathy as a Paraneoplastic Manifestation of Lymphoma

Although myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune demyelinating disease, it may occur as a paraneoplastic syndrome in rare instances. A 80-year-old woman presented with paresthesia and weakness of four extremities for 10 days. Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis. Laboratory tests revealed positive MOG antibodies. Both high-dose intravenous methylprednisolone and plasma exchange were not effective and further evaluation showed lymphoma. Herein, we reported a case of MOG-positive paraneoplastic myelopathy with lymphoma.

Severe Ocular Hypertension in a Child with Pediatric Optic Neuritis

Purpose: To report a rare case of severe ocular hypertension following intravenous methylprednisolone treatment in a child with bilateral optic neuritis.Case summary: A 6-year-old girl presented with bilateral severe visual loss and disc swelling. Magnetic resonance imaging revealed optic nerve enhancement, and she received high-dose intravenous methylprednisolone for 3 days and oral prednisolone 30 mg/day for 2 weeks. Two weeks after initiating treatment, ophthalmologic examination revealed an intraocular pressure (IOP) of 46 mmHg in the right eye and 42 mmHg in the left eye. The best-corrected visual acuity was 1.0 in the right eye and 0.6 in the left eye. Relative afferent pupillary defect was noted in the left eye. Fundus examination showed normal cup-to-disc ratio (0.4) in both eyes. The iridocorneal angle was open in all directions. The treatment plan included the cessation of oral prednisolone and initiation of latanoprost and dorzolamide-timolol fixed combination agent. After 12 hours, the IOP decreased to 18 mmHg in the right eye and 14 mmHg in the left eye. Six months later, her visual acuity was 1.0 in both eyes, and IOP was maintained at 11 and 13 mmHg in the right and left eye, respectively. Optic disc examination showed no sign of glaucomatous change with normal cup-to disc ratio in both eyes.Conclusions: Since steroid medication may cause asymptomatic IOP elevation, regular intraocular pressure measurements are required during steroid treatment for pediatric optic neuritis. The administration of IOP-lowering eyedrops returned IOP to the normal range without damaging the optic nerve.

52 Use of corticosteroids in childhood systemic lupus erythematosus: experience from a pediatric rheumatology center

BackgroundChildhood-onset Systemic Lupus Erythematosus (cSLE) is a rare but severe multisystem autoimmune/inflammatory disease that can affect any organ system and cause significant damage, disability and/or death. Corticosteroids are one of the important therapeutic weapons for childhood Systemic Lupus Erythematosus (SLE). Prednisone doses are increased during flare-ups of lupus disease, and then progressively reduced to reach a threshold dose in order to avoid the harmful effects of this treatment in growing children. The oral route is by far the most used over prolonged periods, however corticosteroid boluses are recommended in the treatment of severe acute attacks of the disease.ObjectiveAssess the use of corticosteroids in childhood systemic lupus erythematosus (SLE) and the risk of steroid-related damage.MethodsPatients with childhood -onset SLE (n = 83) treated at paediatric rheumatology centre were followed for 36 months. Information on medication use, disease activity as measured by SLEDAI was collected.ResultsEighty-three (83) patients were studied. Female: male ratio was 1:49. Mean ages at lupus onset and diagnosis were respectively: 10, 12 (±3,88_ and 11, 3 (±3,62) years. Corticosteroids were used in 92% of cases in oral form and on 37% of cases as a bolus of high dose intravenous methylprednisolone. The indication of corticoids bolus was significantly related to severe forms (p = 0,001), high disease activity (p = 0, 00008), and macrophage activation Syndrome (p = 0, 0006). The doses varied between 0.5–1 g/1.73m2. The mean initial disease activity in these patients was 30 ± 10, while that found after taking the treatment was 12.9 ± 15. The decrease was statistically significant (p = 0, 000001). More than half of our patients were on high dose oral corticosteroids (2 mg/kg/d) and only 1/5 of the patients benefited from a dose of (1 mg/kg/day). The use of corticosteroids has been associated with significant side effects, including growth delay (22%), cushingoid obesity (58%), stretch marks (49%), osteoporosis (33%), femoral head osteonecrosis (1%), infections (54%). The association of certain side effects with a high dose of oral corticosteroids (2 mg/kg/day) was significant in this case cushingoid obesity (p = 0, 0003), stretch marks (p = 0.014).ConclusionCorticosteroids are the mainstay of therapy for prompt control of acute disease related inflammation in patients with SLE; they remain the first-line treatment for significant lupus manifestations, especially severe forms and life-threatening situations Higher dosage and longer duration of therapy, which are common in SLE, result in greater risk of adverse effects. Their use wisely is the guarantee of a better response and the reduction of deleterious effect.

Secondary autoimmune hypothalamitis with severe memory impairment 7 years after the onset of diabetes insipidus due to lymphocytic hypophysitis: a case report

BackgroundAutoimmune hypothalamitis is a very rare neuroendocrine disorder that causes central diabetes insipidus, headache, visual impairment, and sometimes cognitive impairment. Autoimmune hypothalamitis may occur in association with autoimmune hypophysitis, including lymphocytic hypophysitis, or in isolation. It is not known whether autoimmune hypothalamitis and autoimmune hypophysitis are consecutive diseases.Case presentationA 52-year-old woman developed autoimmune hypothalamitis 7 years after developing central diabetes insipidus due to lymphocytic hypophysitis, resulting in severe memory impairment. High-dose intravenous methylprednisolone therapy improved her cognitive function and decreased the size of the lesion.ConclusionThis case presented a unique clinical course, with a long period of time between the onset of autoimmune hypopituitaritis and the development of autoimmune hypothalamitis.

A Rare Case of Pembrolizumab Associated Encephalopathy

We present a unique case of a 55-year-old man with confusion thought to be due to pembrolizumab which he was receiving for renal cell carcinoma. His workup for other possible etiologies for encephalopathy was negative. He was treated with high dose intravenous methylprednisolone followed by prednisone taper and intravenous immunoglobulin with gradual improvement in his mentation.

Encephalitis with Extensive Cortical Brain Magnetic Resonance Imaging Changes Secondary to Myelin Oligodendrocyte Glycoprotein Antibody Disease

Patient: Male, 17-month-old Final Diagnosis: Fluid attenuated inversion recovery (FLAIR) imaging showing cortical hyperintense lesions in myelin-oligodendrocyte-glycoprotein (MOG) associated encephalitis with seizures (FLAMES Symptoms: Encephalopathy • fever • left sided weakness • seizures Medication: — Clinical Procedure: Plasmapharesis Specialty: Neurology Objective: Rare disease Background:The relatively new autoimmune disorder, anti-myelin oligodendrocyte glycoprotein (MOG) disease is particularly interesting because of its broad range of presentations. This entity’s appearance on magnetic resonance imaging (MRI) of the brain often makes identifying this disease a challenging process. Younger patients tend to present with an acute disseminated encephalomyelitis picture, with encephalopathy and multifocal neurological signs, while older patients are more likely to present with optic neuritis. We, however, report an atypical case of a patient who presented with encephalopathy, seizures, and significant cortical and subcortical gray matter involvement and was found to have anti-MOG positivity in serum.Case Report:A 17-month-old previously healthy boy presented to Emergency Department with fever, lethargy, vomiting, and left-sided weakness. Eventually, he required intubation due to a prolonged seizure. Continuous electroencephalogram captured several focal seizures, and MRI of the brain showed cortical and subcortical T2 hyperintensities. After extensive laboratory evaluation, he tested positive for anti-MOG antibody. He was empirically started on high-dose intravenous pulse methylprednisolone, followed by plasma exchange, given the poor response to the intravenous steroids. At the 5-month follow-up, the results of the neurological examination had dramatically improved, and MRI findings had largely resolved.Conclusions:This case highlights the importance of suspecting anti-MOG antibody-mediated encephalitis, even while ruling out infectious etiologies, in children presenting with encephalopathy, seizures and MRI abnormalities. Prompt recognition would allow for less delay in treatment and hopefully improve prognosis.

Long-Term Efficacy and Low Adverse Events of Methylprednisolone Pulses Combined to Low-Dose Glucocorticoids for Systemic Sclerosis: A Retrospective Clinical Study of 10 Years' Follow-Up.

BackgroundPatients with systemic sclerosis (SSc) have poor prognosis without cure methods. We began, 10 years ago, to relieve active SSc using short-term intravenous high-dose methylprednisolone pulse (MP-Pulse) and then maintain remission using long-term and low-dose oral glucocorticoids (LTLD-GC).MethodsTotal 46 of SSc patients with interstitial lung disease (ILD) and induration of skin during January 2006 to December 2019 were analyzed retrospectively, who were followed up for 10 years or more. The patients were treated with MP-Pulse (15 mg/kg/day, 4 days/week, for 2 weeks) with (n=21) or without (n=25) LTLD-GC (prednisone 5–10 mg/day or methylprednisolone 4–8 mg/day). The biographic and clinical data, including occurrence of infection or any adverse reactions, were collected at baseline, 6 months, 1 year, and annually through 10 years after treatment.ResultsFrom baseline to 10 years, compared with MP-Pulse alone, MP-Pulse/LTLD-GC significantly reduced skin and lung fibrosis and improved lung function: Rodnan skin score (mRSS: 22.1±12.4 to 8.16±2.5, P<0.001), forced vital capacity (FVC: 71.7% to 89.83%, P<0.001), forced expiratory volume in the first second (FEV1: 75.7% to 87.88%, P<0.001), diffusing capacity of the lung for carbon monoxide (DLCO: 63.4% to 87.73%, P<0.001), and high-resolution chest computerized tomography scan (HRCT score: 3.96±2.81 to 1.42±0.83, P<0.001). None of the 46 patients had femoral head necrosis, compression fracture, death, or life-threatening adverse events.ConclusionThese outcomes indicate that intravenous MP-Pulse combined with oral LTLD-GC could achieve significant remission and better long-term (10 years) efficacy without severe adverse effects in SSc patients with ILD and induration of skin.

Cytotoxic lesions of the corpus callosum after COVID-19 vaccination

A 23-year-old previously healthy man (Patient 1) and a 33-year-old woman with a past history of depression (Patient 2) developed neurological symptoms approximately 1 week after receipt of the first COVID-19 mRNA vaccination and deteriorated over the next week. Patient 1 reported nausea, headache, a high fever, and retrograde amnesia. Patient 2 reported visual disturbance, headache, dysarthria, a left forearm tremor, dysesthesia of the mouth and distal limbs, and visual agnosia. PCR test results for SARS-CoV-2 were negative. Complete blood cell count, biochemistry, and antibody test and cerebrospinal fluid test findings were unremarkable. Diffusion-weighted and fluid-attenuated inversion recovery MRI of the brain showed a high signal intensity lesion at the midline of the splenium of the corpus callosum compatible with cytotoxic lesions of the corpus callosum (CLOCCs). High-dose intravenous methylprednisolone improved their symptoms and imaging findings. CLOCCs should be considered in patients with neurological manifestation after COVID-19 vaccination.

Antibody-Positive Neuromyelitis Optica Spectrum Disorder After Second COVID-19 Vaccination: a Case Report

In the era of the COVID-19 pandemic declared in March 2020, widespread vaccination protocols were initiated to mitigate the severity and spread of COVID-19. Although COVID-19 vaccines have been generally considered safe, adverse events post-vaccination have been reported, including the development of demyelinating disease. We report a rare case of de novo aquaporin-4-positive neuromyelitis optica spectrum disorder (NMOSD) in an 80-year-old man following BNT162b SARS-CoV-2 vaccination to raise the awareness of this possible severe adverse event in an older adult. An 80-year-old South Asian man presented 2 days following his second dose of the Pfizer-BioNTech COVID-19 mRNA BNT162b2 vaccine with progressive left-sided leg weakness and numbness resulting in falls. MRI of the spine revealed a longitudinally extensive transverse myelitis from T3–T4 to T9–T10. Serum antibody testing revealed positive aquaporin-4 (AQP4) antibodies. He was diagnosed with AQP4-positive NMOSD and was treated with high-dose intravenous methylprednisolone and plasma exchange with some improvement. He was subsequently treated with mycophenolate mofetil and a slow steroid wean. This case report adds to the existing literature and suggests that COVID-19 vaccinations may trigger de novo NMOSD or NMOSD relapses in some individuals. Although rare, our patient presented with new-onset NMOSD in his 80 s following COVID-19 vaccination. As such, it is relevant to consider AQP4 testing in those presenting with a post-vaccination myelitis, regardless of age. Ongoing vaccine surveillance and research are needed to understand the risk of NMOSD post-COVID-19 vaccinations further.

Safety of Intravenous Methylprednisolone in Refractory and Severe Pediatric Uveitis.

PurposeTo evaluate the safety of intravenous high-dose pulse methylprednisolone succinate (IVHDM) in the management of severe or refractory non-infectious pediatric uveitis.MethodsWe reviewed all uveitis patients who were ≤16 years of age and who received IVHDM with a dose of ≥500 mg per day (1–3 days a month) for at least 3 months during their management at a tertiary care eye hospital.ResultsTwenty pediatric patients with severe or refractory uveitis who received IVHDM were identified. Six patients received IVHDM either once, as a preoperative medication, or at a lower dose than 500 mg, and were excluded. The remaining 14 patients received IVHDM for at least 4 months. Age (mean±SD) was 11.9±2.4 years and 50% were female. Duration of treatment was 14.2±7.5 months. Thirteen patients received IVHDM in combination with other immunomodulatory therapy (IMT). Except for two outliers, IVHDM was given at a dose of 8–25 mg/kg per infusion. Three major adverse events (AEs) occurred in two patients: a single episode of bradycardia, compression fracture following minor trauma and adrenal insufficiency. The number of AEs (major and minor) strongly correlated with duration of treatment (p=0.004) and moderately correlated with the cumulative dose/weight (p=0.051). Weight gain was associated with the use of concomitant oral steroids and not with duration of treatment or cumulative dose.ConclusionIVHDM may be a valid therapeutic option for aggressive/refractory pediatric uveitis. The reported AEs in this series can also be attributed to the concurrent IMT or the underlying disease itself.

ABCB1 gene polymorphisms impact the effect of high-dose intravenous methylprednisolone therapy on optic neuritis associated with AQP4-IgG-positive neuromyelitis optica spectrum disorder.

Patients with optic neuritis (ON) have significant individual differences in their response to high-dose intravenous methylprednisolone (HIMP) therapy. This study aims to evaluate the association between gene polymorphisms and the efficacy of HIMP therapy in Chinese Han patients with ON mediated by aquaporin-4 immunoglobulin G antibody (AQP4-IgG) -positive neuromyelitis optica spectrum disorder (NMOSD) or multiple sclerosis (MS). Chinese Han patients with AQP4-IgG+ NMOSD-ON or MS-ON were genotyped for four candidate genes: ABCB1 (rs1045642, rs1128503, rs2032582), NR3C1 (rs41423247), TBX21 (rs9910408, rs16947078) and VDR (rs731236, rs1544410, rs7975232, rs2228570). Patients were divided into glucocorticoid resistance (GR) and glucocorticoid sensitivity (GS) groups based on vision acuity (VA) improvement after HIMP treatment. Intergroup comparisons were performed on clinical characteristics, allele and genotype frequencies and haplotype distributions. A total of 267 patients completed the follow-up, including 120 patients with AQP4-IgG+ NMOSD-ON and 147 patients with MS-ON. We observed a significant association between the ABCB1 G2677T/A (rs2032582) polymorphism and glucocorticoid response in AQP4-IgG+ NMOSD-ON patients. Changes in VA scores in patients with the GG genotype were significantly lower than those in patients with the T/A T/A genotype (1.07 ± 1.20 vs. 1.77 ± 1.31, p=0.026). In the GS group, the G allele had a lower frequency than the T/A allele (32.03% vs. 60.16%, p=0.001). Logistic regression analysis showed that the G2677T/A GG and G T/A genotypes could increase the GR risk 3.53 and 2.67 times compared with the T/A T/A genotype, respectively (OR=3.534, 95% CI: 1.186-10.527, p=0.023; OR=2.675, 95% CI: 1.005-7.123, p=0.049). In addition, haplotype analysis showed that AQP4-IgG+ NMOSD-ON patients with the TAT/TTT haplotype (ABCB1 C3435T-G2677T/A-C1236T) were only 0.54 times more likely to develop GR than those with other haplotypes (OR=0.542, 95% CI: 0.315-0.932, p=0.026). However, we did not observe intergroup differences in the MS-ON population. Our findings suggest that the G > T/A polymorphism of ABCB1 G2677T/A and the TAT/TTT haplotype played a protective role in HIMP treatment of AQP4-IgG+ NMOSD-ON but not MS-ON.

Efficacy of Plasma Exchange Treatment for Demyelinating Optic Neuritis Associated with Various Serum Antibodies: A Prospective Cohort Study.

Introduction To evaluate the value of plasma exchange (PE) for patients with three subtypes of demyelinating optic neuritis (ON): aquaporin-4 (AQP4) antibody-positive ON (AQP4-ON), myelin oligodendrocyte glycoprotein (MOG) antibody-positive ON (MOG-ON), and AQP4 and MOG double-antibody-seronegative ON (D-ON).MethodsA single-center prospective study compared the logarithm of the minimum angle of resolution (logMAR) best-corrected visual acuity (BCVA) at most severe onset, 1 day before intravenous high-dose methylprednisolone (IVMP) treatment, 1 day before PE treatment, after five-cycles of PE therapy, and at 1-, 3-, and 6-month follow-up visits. The proportions of eyes in each visual outcome category were also compared. Logistic regression and a receiver operating characteristic curve were used to analyze predicted factors for VA improvement.ResultsA total of 124 ON attacks of 122 patients were included. No significant differences were found in BCVA (P = 0.659) before and after PE therapy for 22 D-ON attacks, but VA improved in two of six MOG-ON patients. In 95 AQP4-ON patients suffering 96 attacks, the mean logMAR BCVA markedly improved and was steadily maintained after five-cycles of PE treatments (adjusted P < 0.001), with VA exhibiting a significantly increasing trend (adjusted P = 0.001) after PE treatment. The combination of the number of previous ON episodes and the time window to PE treatment showed accuracy of 74.7% for predicting an improvement in BCVA score ≥ 2 levels. In addition, a combination of logMAR VA before PE and the time window to PE treatment resulted in 83.4% accuracy in predicting whether VA would regain 1.0 logMAR.ConclusionPE therapy effectively improves visual outcomes for AQP4-ON patients, but offers limited value for D-ON patients. Early initiation greatly increases likelihood of achieving VA improvement.Supplementary InformationThe online version contains supplementary material available at 10.1007/s40120-022-00344-w.

Acute transverse myelitis in an adult-patient with underlying ulcerative colitis: a case report

BackgroundUlcerative colitis (UC) is an idiopathic inflammatory bowel disease that limits to colon mucosa, which characterised by relapsing and remitting abdominal pain and diarrhea. Neurological complications in UC patients are usually underestimated. The most frequently reported neurological disorders associated with UC are peripheral neuropathy, cerebrovascular disease and demyelinating disease. However, acute transverse myelitis (TM) is rarely reported in UC patients.Case presentationWe report a case of a 39-year-old man presented with fatigue, muscle weakness, numbness in the lower limbs and fingers with underlying UC. Laboratory results revealed elevated neutrophil count, high-sensitivity C-reactive protein and erythrocyte sedimentation rate. Strip-shaped high signal intensity was identified in the cervical and thoracic spinal cord on T2-weighted magnetic resonance imaging. Acute TM was diagnosed. Significant improvements after intravenous high-dose methylprednisolone were observed.ConclusionWe speculate that acute TM may be the extraintestinal manifestation of UC, which may be related to the abnormalities of cell-mediated and humoral immunity rather than the side effect of mesalazine.

Long-Term Visual Prognosis in Patients With Aquaporin-4-Immunoglobulin G–Positive Neuromyelitis Optica Spectrum Disorder

To identify the factors associated with visual prognosis for functional and structural outcomes of optic neuritis (ON) in patients with aquaporin-4-immunoglobulin (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD). We included the eyes that experienced at least 1 episode of ON and were followed for at least 2 years after the first attack of ON in patients with AQP4-IgG-positive NMOSD. We performed a retrospective review of clinical data, including ophthalmological examination and orbital MRI, of 34 eyes of 22 patients. Functional outcomes were measured as final visual acuity, visual field index, and mean deviation and structural outcomes as final retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GCIPL) thickness. The mean age at onset of the first ON was 42.7 ± 13.7, and all patients were female. The poor visual acuity was significantly associated with the worse final visual acuity and thinner RNFL and GCIPL. Older age also showed a negative correlation with RNFL thickness. The number of attacks was not statistically significant for functional and structural outcomes. The lesion involving the intracanalicular optic nerve to the chiasm on orbital MRI showed worse visual acuity and a thinner GCIPL. Rapid high-dose intravenous methylprednisolone pulse therapy within 3 days was statistically significant, with better visual acuity and more preserved GCIPL thickness. Our results indicate that the severity of ON rather than the number of recurrences might be critical for the visual prognosis of patients with AQP4-IgG-positive NMOSD. Rapid treatment within 3 days may improve visual outcomes, and a younger age at onset may have better visual outcomes.

COVID-19 Infection or Buttock Injections? The Dangers of Aesthetics and Socializing During a Pandemic

IntroductionSilicone (polydimethylsiloxane) injections are used for cosmetic augmentation. Their use is associated with life-threatening complications such as acute pneumonitis, alveolar hemorrhage, and acute respiratory distress among others [1,2]. We report a case of a Hispanic woman who developed severe respiratory distress syndrome after gluteal silicone injections.Case PresentationA 44-year-old Hispanic female presented to the Emergency Department complaining of progressive dyspnea on exertion for two weeks. Chest imaging revealed patchy bibasilar airspace opacities of peripheral distribution. Labs were significant for leukocytosis, elevated PT, D-dimer, lactate dehydrogenase, and fibrinogen, concerning for COVID-19, however SARS-CoV-2 testing was negative multiple times. The patient later became encephalopathic, hypoxemic, and eventually required intubation. Further history uncovered that the patient had received illicit gluteal silicone injections a few days prior to her onset of symptoms. The patient was diagnosed with silicone embolism syndrome (SES) and initiated on high dose intravenous methylprednisolone [1].Case DiscussionPatients from lower socioeconomic backgrounds utilize illicit services to receive silicone injections at minimal costs. This leads to dangerous outcomes. The serology and imaging findings observed in our case have similarities to the typical presentation of COVID-19 pneumonia making the initial diagnosis difficult. This case serves as a cautionary tale of the importance of thorough history taking in patients with concern for COVID-19.

Bilateral Optic Neuritis Caused by Meningococcal Meningoencephalitis

AbstractIn this article, we described a case of pathogen-induced bilateral optic neuritis accompanying meningococcal meningoencephalitis in an adolescent male. A 15-year-old boy presented to our emergency room due to progressive severe headache, stiff neck, diffuse extremity pain, fever, and nausea concerning meningoencephalitis. Intravenous ceftriaxone, metamizole, and ondansetron were started immediately. Due to acute autonomic dysregulation and development of petechiae, he was transferred to the pediatric intensive care unit. Next morning, the patient developed severe visual impairment. Investigations revealed elevated C-reactive protein, procalcitonin and leucocyte count, decreased platelet count, and clotting activation. Cerebrospinal fluid (CSF) analysis revealed increased leucocyte count, protein, and decreased glucose concentration with pathological CSF cytology. Brain magnetic resonance imaging revealed an increased contrast enhancement in the optic nerve sheath, consistent with acute bilateral optic neuritis. He was started on high-dose intravenous pulse methylprednisolone therapy. After treatment with ceftriaxone und pulse steroids, the patient was discharged in good condition without any visual sequel.

Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature.

Myelin oligodendrocyte glycoprotein antibody-associated disease is an immune-mediated demyelinating disease of the central nervous system that is present in both adults and children. The most common clinical manifestations are optic neuritis, myelitis, acute disseminated encephalomyelitis, and brainstem syndrome. Cerebral cortical encephalitis (CCE) is a rare clinical phenotype of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), which usually begins with seizures, headaches, and fever, and may be misdiagnosed as viral encephalitis in the early stages. Herein, we report two typical MOG antibody (MOG-Ab)-positive patients presenting with CCE, both of whom presented with headache, fever, seizures, and who recovered completely after immunotherapy. In addition, we performed a systematic review of the present literature from the perspectives of population characteristics, clinical symptoms, MRI abnormalities, treatments, and prognosis. Among the patients reported in 25 articles, 33 met our inclusion criteria, with the age of onset ranging from 4 to 52 years. Most of the patients had seizures, headache, fever, and unilateral cortical lesions on brain MRI. For acute CCE, 30 patients were treated with high-dose intravenous methylprednisolone, and the symptoms of most patients were completely relieved after immunotherapy. This study reported our experience and lessons learned in the diagnosis and treatment of MOG-Ab-positive CCE and provides a systematic review of the literature to analyse this rare clinical phenotype.

Kardiotoksičnost visoke doze metilprednizolona kod bolesnika sa hroničnom limfocitnom leukemijom - prikaz slučaja

Introduction: Several classes of drugs can cause atrial fibrillation (AF) in patients without heart disease, as well as the occurrence of AF in pre-existing heart disease. Drug-induced AF is most often clinically manifested in the form of paroxysms. High doses of intravenous methylprednisolone (HDMP) in the treatment of chronic lymphocytic leukemia (CLL) have been associated with the occurrence of various adverse effects, including AF. The exact mechanism of HDMP-induced AF is unknown. Case presentation: We present the case of a patient with CLL in whom AF occurred after the administration of a high dose of intravenous methylprednisolone. Conclusion: Physicians should be aware of the special circumstances in the treatment of CLL, because drug-induced AF can be a side effect that limits further therapy.

Therapeutic Plasma Exchange using Continuous Flow Cell Separator Machine in a Neuromyelitis Optica Patient Outcome and Analysis

Neuromyelitis optica (NMO) is an inflammatory central nervous system syndrome, associated with serum aquaporin-4 immunoglobulin G antibodies. Autoantibodies against aquaporin-4 (AQP4-IgG), the principal water channel on astrocyte foot processes at the blood brain barrier, are pathogenic in NMO. Acute attacks are managed by high-dose intravenous methylprednisolone, if symptoms fail to resolve, plasma exchange is considered. Here, we present a case of NMO spectrum disorder in a 20 year old lady, failed to pharmacotherapy, was managed by plasma exchange using Spectra Optia Cell Separator machine. Detailed plasma exchange protocol, disease progression and outcome was analysed in this study.

Purpura fulminans, presenting feature of multisystem inflammatory syndrome in children (MIS-C)

Introduction. Coronavirus disease 2019 (COVID-19) has been a worldwide health issue since early 2020, caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Even though children do not usually present with se­vere COVID-19 unless the patient has an underlying di­sease, post­in­fectious complications, such as the multisystem in­flam­ma­to­ry syndrome in children (MIS-C), have been a sig­ni­fi­cant cause of child morbidity and mortality during the pan­de­mic. Even more challenging is that MIS-C has a very broad differential diagnosis due to its clinical heterogeneity. Case report. We report the case of a 6-month-old infant with­out another medical history who was brought to the ge­ne­ral practitioner’s office for persistent fever and cough. The symp­toms did not ameliorate under antipyretic and mucolytic treat­ment, so the patient later presented in the emergency room with associated dyspnea, diarrhea, vomiting and rapidly ge­ne­ra­li­zing purpuric skin lesions suggestive of purpura ful­mi­nans. He was admitted into our hospital and received em­pi­ri­cal antibiotic treatment. He also developed neck stif­fness. Meningococcemia was thus suspected; however, all cultures came out negative. Laboratory studies showed an important inflammatory syndrome, thrombocytopenia, se­vere anemia, coagulopathy, low protein C, protein S and an­ti­throm­bin levels, low fibrinogen, high D-dimer levels and hypo­al­bu­mi­ne­mia. Cerebral magnetic resonance imaging (MRI) showed no evidence of brain lesions. SARS-CoV-2 IgG-type antibodies were positive, therefore MIS-C was sus­pec­ted. The laboratory studies were extended and revealed high NT-proBNP, IL-6 and IL-2 soluble antigen levels. Other pos­si­ble diagnoses were excluded, which permitted our team to establish the diagnosis of MIS-C. The specific treatment was initiated with intravenous immunoglobulins (IVIG) and high-dose methylprednisolone associated with enoxaparin, broad-spectrum antibiotics and with symptomatic treatment. The patient rapidly evolved well, with the remission of fe­ver, inflammatory syndrome, coagulopathy and all other clinical and laboratory alterations. The skin lesions ame­lio­ra­ted as well, however, at a slower pace. Conclusions. MIS-C is a potentially life-threatening complication of COVID-19 in children. Therefore, clinicians must maintain a high degree of suspicion in the setting of the ongoing SARS-CoV-2 pandemic. However, the diagnostic criteria should be strictly followed due to the clinical similarities this pathology might have with other known diseases.