High-dose Methylprednisolone Research Articles

Acute Disseminated Encephalomyelitis

Absrtract Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory demyelinating disorder of the central nervous system that predominantly affects the pediatric population, and it is characterized by an acute or subacute onset of multifocal neurological symptoms. ADEM is typically a monophasic illness, and the majority of cases are associated with either a preceding infection or vaccination. First-line therapy for the disease is intravenous administration of high-dose methylprednisolone, and the long-term prognosis of ADEM is generally favorable.

P035 Inflammatory myopathy and metabolic disorders causing myopathies

Abstract Background/Aims Myopathies due to inborn errors of metabolism can be difficult to differentiate from inflammatory myopathies. Careful history, examination and laboratory tests are required to establish the diagnosis. We present a case of Riboflavin Transport Deficiency (Brown-Vialetto-Van Laere syndrome) masquerading as an inflammatory myopathy. Methods A 37-year-old lady presented with severe proximal muscle weakness. She had background of sensory neuropathy and chronic anaemia. Notably, her sister had a history of similar symptoms. Creatine Kinase (CK) was 360 IU/L and lactate dehydrogenase (LDH) was 1700 IU/L. Inflammatory markers were normal. The Ro52 antibody was weakly positive. Electromyography showed evidence of a sensory neuropathy with myopathic features. There was symmetrical fatty infiltration and atrophy of the thigh muscles on magnetic resonance imaging (MRI). Positron emission tomography (PET-CT) scan showed widespread intense uptake in skeletal muscle groups. She was given 3 pulses of IV methyprednisolone followed by oral prednisolone which did not provide clinical benefit. Intravenous immune globulin was given when she developed bulbar weakness, with difficulty swallowing and breathing. She required non-invasive ventilation and nasogastric feed. There were necrotic and regenerative muscle fibres on the muscle biopsy, in keeping with rhabdomyolysis. Electron microscopy showed abundant lipid accumulation, suggestive of a metabolic disorder. Urinary organic acids were raised, triggering an acylcarnitines blood spot test, which were increased. This was compatible with riboflavin transport 'Brown-Vialetto-Van-Laere syndrome'. Riboflavin 500mg TDS was started resulting in significant clinical improvement. Prednisolone was weaned, genetic testing sent, and she was transferred for neurorehabilitation. Results Riboflavin Transport Deficiency (Brown-Vialetto-Van Laere syndrome) is an autosomal recessive neurodegenerative genetic disorder. It affects females and males equally. Symptoms can appear in infants as well as adults. These include hearing and visual loss, bulbar palsy leading to dysphagia and speech problems. Paralysis of diaphragm may cause breathing difficulty. Initially it affects the proximal muscles and then generalized muscle weakness. Molecular genetic testing is required to confirm diagnosis. Patients may have abnormal plasma levels of flavin or acylcarnitine. Acylcarnitines are biological intermediates, used in the diagnosis of fatty acid oxidation disorders. Treatment includes riboflavin supplementation and supportive measures. Response to treatment is variable. Conclusion This lady was initially managed as inflammatory myopathy but did not respond to high dose methylprednisolone. There were atypical features including normal inflammatory markers, MRI thighs showing predominantly fatty infiltration and muscle atrophy and the muscle biopsy with abundant lipid accumulation suggestive of a metabolic disorder. We are awaiting full results of genetic testing. This case is a reminder of the importance of tissue diagnosis and reassessing the initial diagnosis if the clinical picture changes or patients do not respond as expected to treatment. Disclosure M. Malik: None. A. Mason: None. B. Davidson: None. J. Furby: None.

High-Dose Intravenous Methylprednisolone May Have Long-Term Benefits on the Daytime Sleepiness of Narcolepsy: A Case Report.

High-Dose Intravenous Methylprednisolone May Have Long-Term Benefits on the Daytime Sleepiness of Narcolepsy: A Case Report.

POS-026 INAUGURAL ACUTE AND SEVERE RENAL FAILURE OF THROMBOTIC THROMBOCYTOPENIC PURPURA

Thrombotic Thrombocytopenic Purpura (TTP) is a rare disease characterized by generalized microvascular thrombotic lesions. associating hemolytic anemia and thrombocytopenia associated. The most frequent other signs are neurological, cardiac and gastrointestinal damage. Associated Kidney damage is rare and poses the problem of differential diagnosis with hemolytic and uremic syndrome. We report a case of TTP with atypical presentation but a favorable outcome. Observational case report A 43-year-old patient was admitted for investigation of severe acute renal failure (creatinine 1642 μmol/l). The history revealed generalized tonicoclonic seizures in childhood. He has no hypertension nor neurological or gastrointestinal signs. Biologicals finding were: microscopic hematuria, proteinuria: 400mg/g, hemoglobin 6g/dl, schizocyte 2%, haptoglobin<0.1g/l, platelets 35. 109/l, direct coombs test was negative. Antinuclear antibody were positive (1/200) and speckled, low C3, normal C4. Factor H was normal without factorH antibodies. Ultrasound showed normal kidney size. The diagnosis of lupus nephritis was retained, renal biopsy was contraindicated because of deep thrombocytopenia. Several dialysis sessions were performed. The patient was treated by high-dose intravenous methylprednisolone (0.5 g/d for three days) then oral prednisone up to 1 mg/kg and by monthly intravenous pulses of cyclophosphamide dosed at 1g/m2 for 6 months (according to the NIH regimen) with improvement of renal function (creatinine 240 μmol/l) and hematological disturbances. Kidney biopsy was performed three months later, after correction of thrombocytopenia, renal histology showed eight normal glomeruli with interstitial fibrosis and negative immunofluorescence. Six months later, while the patient was on prednisolone 20 mg/day, he presented with hemiparesis and dysarthria revealing a stroke in the left middle cerebral arterial territory with hypertensive emergency (blood pressure: 200/100mmHg) and acute renal failure (creatinine 959μmol /l), hemolytic anemia and thrombocytopenia. The patient received high-dose intravenous corticosteroids and 3 sessions of plasmapheresis using fresh frozen plasma with clinico-biological improvement. ADAMTS-13 activity level was 1% without autoantibodies (<12U/ml). Two years after the inaugural episode, he maintains stable renal function (creatinine 200 μmol/l) using regularly fresh frozen plasma perfusion. This is a constitutional and severe ADAMTS-13 deficiency, with atypical renal involvement and absence of hypertension at presentation. The neurological manifestations, appeared nine months after the onset of the disease, rectified the diagnosis. Initial improvement with corticosteroid therapy and plasmapheresis suggest that autoantibodies may also be present. Regular supplementation of deficient protein maintained remission.

Wiskott-Aldrich syndrome

Two cases of successful cadaveric renal transplantation in patients with the Wiskott-Aldrich syndrome have previously been reported in the literature [1,2]. The first patient [1] had an almost uneventful outcome, while the second one [2] had to be treated with highdose methylprednisolone for biopsy-proven cellular rejection. The optimal immunosuppressive regimen in patients with Wiskott-Aldrich syndrome remains unknown. We report here the case of a Wiskott-Aldrich patient who underwent living-related renal transplantation. This patient however developed a number of infectious and non-infectious complications which led to his death 3 months after transplantation.

Subacute cerebellar ataxia following respiratory symptoms of COVID-19: a case report

BackgroundSevere acute respiratory syndrome virus 2 (SARS-CoV-2) is spreading globally and causes most frequently fever and respiratory symptoms, i.e. Coronavirus disease 2019 (COVID-19), however, distinct neurological syndromes associated with SARS-CoV-2 infection have been described. Among SARS-CoV-2-infections-associated neurological symptoms fatigue, headache, dizziness, impaired consciousness and anosmia/ageusia are most frequent, but less frequent neurological deficits such as seizures, Guillain-Barré syndrome or ataxia may also occur.Case presentationHerein we present a case of a 62-year-old man who developed a subacute cerebellar syndrome with limb-, truncal- and gait ataxia and scanning speech 1 day after clinical resolution of symptomatic SARS-CoV-2 infection of the upper airways. Apart from ataxia, there were no signs indicative of opsoclonus myoclonus ataxia syndrome or Miller Fisher syndrome. Cerebral magnetic resonance imaging showed mild cerebellar atrophy. SARS-CoV-2 infection of the cerebellum was excluded by normal cerebrospinal fluid cell counts and, most importantly, absence of SARS-CoV-2 RNA or intrathecal SARS-CoV-2-specific antibody production. Other causes of ataxia such as other viral infections, other autoimmune and/or paraneoplastic diseases or intoxication were ruled out. The neurological deficits improved rapidly after high-dose methylprednisolone therapy.ConclusionsThe laboratory and clinical findings as well as the marked improvement after high-dose methylprednisolone therapy suggest a post-infectious, immune-mediated cause of ataxia. This report should make clinicians aware to consider SARS-CoV-2 infection as a potential cause of post-infectious neurological deficits with an atypical clinical presentation and to consider high-dose corticosteroid treatment in case that a post-infectious immune-mediated mechanism is assumed.

Presentation of SARS-CoV-2 in a Pediatric Heart Transplant Recipient with Multiple Underlying Comorbidities

A six-year-old heart transplant recipient with additional significant co-morbidities, including severe hypoxic-ischemic injury, gastrostomy, tracheostomy, and mechanical ventilation dependency, encountered SARS-CoV-2 infection. The patient received tacrolimus and mycophenolate to prevent graft rejection, presented initially with SARS-CoV-2 positive and presumed pseudomonas aeruginosa pneumonia. Twenty-three days later, the patient presented with fever recurrence with evidence for systemic inflammation, which resolved rapidly with high-dose methylprednisolone. Interestingly, while IgM to SARS-CoV-2 was present, IgG was not detected even three months after his first positive test for SARS-CoV-2. The author discusses potential immune mechanisms that might have affected the course of multi-system inflammatory syndrome children (MIS-C) in this patient.

Recovery from COVID-19 Pneumonia in a Heart Transplant Recipient

IntroductionSolid-organ transplant patients have a high risk of severe infection related to Severe Acute Respiratory Syndrome Coronavirus-2. There are limited data on COVID-19 presentation and clinical outcome in a cardiac transplant recipient.Case ReportA 54-year old woman a heart transplant recipient presented with symptoms of fatigue, excessive sleepiness and cough with phlegm for one week. She did not report any fever or shortness of breath. She had a heart transplant six months prior complicated by antibody-mediated rejection. She was treated with plasmapheresis, intravenous immune globulin, and high dose methylprednisolone. She could not have further scheduled RV biopsies due to the lockdown. She remained on a high dose of immune suppressive medication till her current presentation. Her medication included Prednisolone 20mg daily, Mycophenolate Mofetil (MMF) 1g bid, Tacrolimus 7 mg bid for a target FK level 10-15. On her current presentation to the hospital, she was found to be hypoxic, tachypnic, tachycardic with a BP 130/70. Her chest x-ray showed bilateral infiltrates. She had leukopenia 3.5 and lymphopenia 0.2, CRP 25, ferritin 1106, LDH 632, and IL6 87. She was started empirically on oseltamivir, vancomycin and piperacillin/tazobactam. Her COVID-19 PCR result was positive. Subsequently, she was started on Favipiravir loading of 1600 mg for two doses and a maintenance dose of 600 mg twice daily for 7 days. The MMF and tacrolimus were discontinued. The prednisone was switched to hydrocortisone 50mg IV q6h. Despite treatment, she had reduced level of consciousness and progressive bilateral lung infiltrates requiering mechanical ventilation. The multidisciplinary team discussed enrolling patients in the convalescent plasma study. The patient's family was informed and they agreed and consented to proceed with plasma therapy. Two units of compatible ABO plasma therapy was given for two consecutive days. Intravenous dexamethasone was started. She was extubated successfully after ten days. Given her marked clinical improvement, she was started on MMF 1g bid, and tacrolimus adjusted to the target FK level of 5. The patient was discharged home after three weeks of admission.SummaryThis case represents a recent heart transplant recipient who presented with COVID-19 pneumonia. Her treatment involved convalescent plasma transfusion, Favipiravir, dexamethasone, and reduction of immune suppression.

Re-examining the effects of high-dose intravenous methylprednisolone for secondary progressive multiple sclerosis.

Background/objective: Intravenous methylprednisolone (IVMP) is previously given to secondary progressive multiple sclerosis (SPMS) patients. This study aimed to re-examine the effects of IVMP in SPMS. Materials & methods: Major electronic databases were searched for randomized controlled trials. Results: Four randomized controlled trials were included. IVMP may be inferior to mitoxantrone (MTX) in terms of expanded disability status scale (EDSS) improvement. There was no significant difference in terms of EDSS reduction and magnetic resonance imaging (MRI) plaque reduction when IVMP + MTX were compared with MTX. There is no significant difference between IVMP and cyclophosphamidebased on EDSS progression and relapse reduction. Conclusion: IVMP should not be routinely used as treatmentfor SPMS and is not recommended as an alternative treatment for SPMS.

Acute Disseminated Encephalomyelitis (ADEM) in Children: A Case report

Acute Disseminated Encephalomyelitis (ADEM) is an inflammatory disease that causes demyelination and affects the white matter of the brain and spinal cord through an immune response. The patient, an eight-year-old Iranian Kurdish boy with a height of 125 cm and a weight of 24 kg, complained from fever for three days which didn’t respond to acetaminophen medication. After three days, the symptoms developed into headache, vomiting, and decreased consciousness. The patient was admitted to the emergency department of Imam Hasan hospital in Bojnourd, Iran, in July 2019. LP diagnostic testing, Wright Agglutination test, Brain CT, and MRI examination were performed. Finally, a diagnosis of acute disseminated encephalomyelitis (ADEM) was confirmed. Following the ADEM diagnosis, methylprednisolone 500 mg was administered immediately and continued for five days. This case study suggests that MRI is the most effective method of diagnosis for ADEM, and high-dose methylprednisolone is the treatment of choice for this syndrome.

Systemic methylprednisolone for hearing preservation during cochlear implant surgery: A double blinded placebo-controlled trial

AimTo assess whether a single, peri-operative, high dose of methylprednisolone can improve the preservation of residual acoustic hearing following cochlear implantation (CI). MethodsThis was a double blinded placebo-controlled trial, performed in a tertiary academic centre. The hypothesis was that methylprednisolone would improve the preservation of hearing, and lower electrode impedances. Adult patients (18–85 years) with hearing at 85 dB or better at 500 Hz in the ear to be implanted were randomly allocated to either treatment (methylprednisolone, 1g administered intravenously upon induction of anaesthesia) or control (normal saline infusion). As per standard clinical practice, all patients received a routine dose of dexamethasone (8 mg intravenously) on induction of anaesthesia. Implantation was undertaken with a slim and flexible lateral wall electrode via the round window. Surgical technique was routine, with adherence to soft surgical principles. The primary outcome was hearing preservation within 20 dB at 500 Hz, 12 months following cochlear implantation. Secondary outcomes included hearing preservation at 6 weeks and 3 months, monopolar electrode impedance, and Consonant-Vowel-Consonant (CVC) Phoneme scores at 3 and 12 months after surgery. ResultsForty-five patients were enrolled into the control group and 48 patients received the steroid. The number of patients achieving hearing preservation at 12 months did not differ significantly between those receiving methylprednisolone treatment and the controls. There were no differences in hearing preservation at any frequency at either 6 weeks or 3 months after implantation. Neither CVC phoneme scores nor electrode impedances differed between the groups. ConclusionsThis paper demonstrates that high-dose local steroid injection at surgery was not effective in preventing a loss of residual hearing, improving speech perception, or lowering electrode impedances. The findings were contrary to the experimental literature, and emerging clinical evidence that steroid elution from implant electrodes influences cochlear biology in humans. We found no evidence to support the widely-held practice of administering intravenous steroids in the perioperative period, in an attempt to preserve residual hearing.

Diagnostic Approach and Successful Treatment of Ocular Tuberculosis

Introduction: The prevalence of ocular tuberculosis (TB) are 1% in patients with pulmonary TB and 20% in patients with extrapulmonary TB. The definitive diagnosis and its management are still challenging. This case highlights the diagnostic approach and successful management of ocular tuberculosis Purpose: To report the diagnostic approach and successful management of ocular tuberculosis.Case Presentation: A-15-Years-old-Girl, presented to outpatient clinic due to blurred vision on both eyes since 6 month ago. She was suffered from fever and solitaire mass along the neck lymph node 2 weeks before. The best corrected visual acuity (BCVA) of the right eye was 0.2 and the left eye 0.3. The ophthalmology examination of both eyes revealed mutton fat, 2+ flare, and 1+ cell, posterior synechia, koeppe and bussaca nodule, 4+ vitreous cells. Funduscopy were unremarkable due to vitritis. There was an elevation of erythrocyte sedimentation rate, positive mantoux test and Quantiferon-TB Gold. Chest radiograph showed fibrosis of the right lung. Fine needle aspiration biopsy of neck mass showed fibrotic tissue. Patient was diagnosed with tuberculous granulomatous panuveitis. She received anti-tuberculous therapy (ATT) along with high dose methylprednisolone 0.5 mg/BW/days on tapered dose. At 3 weeks follow up, vitritis was subsided and we revealed snowball, snowbanking, and optic disc swelling of both eyes that showed improvement at 11 weeks follow up. The BCVA become 1.0 at week-17 of therapy and remains stable until 8 months follow up. Conclusion: Diagnosis of ocular tuberculosis diagnose was made based on symptoms and signs of granulomatous panuveitis and supporting evidence of pulmonary TB ancillary tests. Significant clinical improvement was achieved after administration of ATT along with high dose oral steroid.

How I treat immune thrombocytopenia – a global view

How I treat immune thrombocytopenia – a global view

Clinical characteristics and outcomes of critically ill COVID-19 patients in Tokyo: a single-center observational study from the first wave

BackgroundMany studies have been published about critically ill coronavirus disease 2019 (COVID-19) during the early phases of the pandemic but the characteristic or survival of critically ill Japanese patients have not yet been investigated. We sought to investigate the characteristics, inflammatory laboratory finding trends, and outcomes among critically ill Japanese patients who were admitted to the intensive care unit (ICU) with the first wave of COVID-19.MethodsA retrospective observational study was performed in a single institution in the center of Tokyo. Laboratory-confirmed COVID-19 patients admitted to the ICU from March 19 to April 30, 2020 were included. Trends for significant inflammatory laboratory findings were analyzed. In-hospital death, days of mechanical ventilation or oxygen supplementation, days of ICU or hospital stay were followed until May 26, 2020.ResultsTwenty-four patients were included. Median age was 57.5 years, and 79% were male. The neutrophil-to-lymphocyte ratio was elevated to a median of 10.1 on admission and peaked on Day 10 of illness. Seventeen patients were intubated on Day 11 of illness and received mechanical ventilation. One patient underwent extracorporeal membrane oxygenation. The majority (88%) received systemic steroids, including 16 patients who received high dose methylprednisolone (500–1000 mg). Favipiravir was used in 38% of patients. Two patients, including 1 who refused intensive care, died. Eighteen patients were discharged. Median length of ICU and hospital stay for all patients was 6 and 22 days, respectively. Median length of ventilator dependency was 7 days. Four patients underwent a tracheostomy and received prolonged ventilation for more than 21 days. One patient receiving mechanical ventilation died. All survivors discontinued ventilator use.ConclusionsMortality was remarkably low in our single institutional study. Three survivors received mechanical ventilation for more than 3 weeks. Trends of clinically significant laboratory markers reflected the clinical course of COVID-19.

Susac’s syndrome: characteristic imaging

A 54-year-old man had a 12 month history of weakness of both lower limbs with urge urinary incontinence, 6 month history of visual loss and hearing impairment and a 1...

Clinicopathological features and outcomes of SLE patients with renal injury characterised by thrombotic microangiopathy.

Non-immune complex (IC)-mediated renal thrombotic microangiopathy (TMA) has been reported in patients with systemic lupus erythematosus (SLE), but most studies included patients with both renal TMA and IC-mediated lupus nephritis (LN). In this study, the clinicopathological features and outcomes of renal injury characterised by only renal TMA were retrospectively analyzed. Patients with glomerular and/or vascular TMA in the absence of subendothelial or epithelial immune deposits were screened from 2,332 biopsied of SLE patients. The TMA lesions were divided into glomerular, vascular or both. Acute tubular-interstitial injury was semi-quantitatively analyzed. The podocyte foot process effacement (FPE) was measured by electronic microscopy. Two hundred fifty-seven (11.0%) renal biopsies revealed TMA, among which 237 biopsies showed TMA coexisting with LN, and 20 (0.9%) biopsies had only renal TMA without or with only mesangial immune deposits. All patients manifested with acute kidney injury and haematological disorders. Among them, 11 (55%) required renal replacement therapy, 12 (60%) had nephrotic syndrome and 13 (65.0%) showed microvascular haemolytic anaemia with thrombocytopenia. Seventeen (85%) biopsies revealed both glomerular TMA and vascular TMA, two had only glomerular TMA and one had vascular TMA. Eight (40%) had no glomerular immune deposits and 12 (60%) showed only mesangial immune deposits. The acute tubulointerstitial injury in patients requiring dialysis was more severe than those not needing dialysis ((43.6 ± 24.9) % vs. (21.7 ± 20.1) %, p = 0.047). FPE of podocytes was positively correlated with proteinuria (r2 = 0.347, p = 0.006). All patients received high-dose methylprednisolone pulse therapy. Four patients received plasma exchange. The renal function of 11 patients requiring dialysis initially recovered after 16.0 (interquartile range [IQR] 9.0, 30.0) days of treatment. During the follow-up of 58.0 (IQR 36.0, 92.3) months, remission was achieved in 19 (95%) patients; only one patient had no response. No patient died or progressed to end-stage renal disease; six patients (30%) relapsed. Renal TMA, usually accompanying severe renal injury, was not uncommon in SLE patients with renal disease and should be distinguished from immune complex-mediated severe classes of LN. Early intensive immunosuppressive treatment may be associated with a good long-term renal outcome. Key Points • Most previous reports of renal TMA in SLE patients were associated with severe types of immune complex-mediated lupus nephritis; • Renal TMA with glomerular pauci-immune or only mesangial immune deposits was found in SLE patients and clinically presented with severe acute renal injury but good renal outcome; • Renal TMA should be considered as a unique type of SLE-associated renal injury.

Immunoglobulin G4-related disease presenting with peripheral neuropathy: a case report

BackgroundImmunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition characterized by high serum IgG4 concentrations and tissue infiltration by IgG4-positive plasma cells. Reports have demonstrated that IgG4-RD affects various organs, including the pancreas, kidney, lung, thyroid, and lacrimal and salivary glands. In the nervous system, hypertrophic pachymeningitis and hypophysitis are mainly related to IgG4-RD; however, the peripheral neuropathy involvement is unusual.Case presentationWe report on a 69-year-old woman with multiple mononeuropathy, weight loss and kidney mass in the setting of IgG4-RD. Biopsies of the kidney mass showed lymphoplasmacytic sclerosing inflammation with numerous IgG4-positive plasma cells. IgG4 and IgG4/IgG ratios in the blood were elevated. The patient was treated with high dose methylprednisolone with improvement in her neuropathy.ConclusionsIgG4-RD is a relatively recently reported systemic fibrous inflammatory disease caused by the infiltration of IgG4-positive plasma cells in various organs. In the nervous system, symptomatic peripheral nerve invasion is very rare. However, as demonstrated in our case, IgG4-RD may present with primarily peripheral nerve disease.

Risk Factor and Long-Term Outcome Analyses for Acute Limbic Encephalitis and Calcineurin Inhibitor-Induced Encephalopathy in Adults following Allogeneic Hematopoietic Cell Transplantation

Post-transplantation acute limbic encephalitis (PALE) is a rare, severe inflammatory disorder in the bilateral limbic system, including the hippocampus. To date, only a few studies have reported details, including risk factors for PALE; however, further clinical evidence of PALE, especially in cerebrospinal fluid human herpesvirus 6-negative cases, is warranted. In addition, data are sparse regarding the risk factors for calcineurin inhibitor (CNI)-induced encephalopathy (CNIE) following allogeneic hematopoietic cell transplantation (allo-HCT) in adults. Therefore, we examined the risk factors for and clinical details of PALE and CNIE. We retrospectively analyzed consecutive patients who underwent allo-HCT between January 2005 and November 2017. A total of 485 patients age 46 years (median) were eligible. In total, 14 PALE cases and 11 CNIE cases were identified. Multivariable analyses identified older age, use of an HLA-mismatched unrelated donor (URD), graft-versus-host disease (GVHD) prophylaxis with CNI and mycophenolate mofetil, and grade II-IV acute GVHD as significantly associated with an increased risk of PALE. In 13 patients who received high-dose methylprednisolone (mPSL) therapy, 6 (46%) responded to mPSL therapy, and 3 (23%) achieved complete remission at day 90 after mPSL administration. Furthermore, myelodysplastic syndrome (MDS), HLA-mismatched URD, and grade II-IV acute GVHD were significantly associated with an increased risk of CNIE. The 5-year nonrelapse mortality rate was 50% in PALE and 63% in CNIE, suggesting a very poor prognosis. In conclusion, this study provides evidence that HLA-mismatched URD and acute GVHD may independently contribute to the development of PALE, possibly in part through HLA-mismatch-derived alloimmune responses. Other than acute GVHD, we have identified MDS and HLA-mismatched URD as novel predictors of CNIE after allo-HCT.

Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access.

BackgroundNeuromyelitis optica spectrum disorder (NMOSD) is an auto-immune disease of the central nervous system (CNS) associated with the IgG-antibody against aquaporin-4 (AQP4-IgG). There is little published epidemiology of NMOSD from sub-Saharan Africa (SSA).MethodsWe retrospectively collated NMOSD cases admitted to our tertiary regional neurology centre.ResultsWe identified 11 cases (10 female, average age 30 years). 64% (7/11) were seropositive for AQP4-IgG, measured using indirect immunofluorescence. The remaining cases could either not afford tests, or had pathognomonic radiological features. 57% (4/7) of seropositive cases had concurrent/recent CNS infection. All patients were treated with high-dose intravenous methylprednisolone (IVMP), and 36% (4/11) also had plasma exchange. Only 55% (6/11) of the patients were seen by a neurologist at presentation: they had less relapses (1.3 vs 2.4), less diagnostic delay (2.3 vs 7.4 months), and were less disabled at the end of our review period. 10 cases were immunosuppressed long-term: 60% on mycophenolate, 30% azathioprine, and one on rituximab.ConclusionOur study is the largest case series of NMOSD from the East Africa region. Patients faced challenges of access to appropriate and affordable testing, and timely availability of a neurologist at onset, which had impacts on their functional outcomes. The majority of the seropositive cases had recent/concurrent CNS infections, suggesting triggered auto-immunity.

Clinical and Paraclinical Measures Associated with Outcome in Cerebral Amyloid Angiopathy with Related Inflammation.

Cerebral amyloid angiopathy with related inflammation (CAA-ri) is a rare age-associated disorder characterized by an inflammatory response to amyloid in cerebral blood vessels. CAA-ri is often treated with corticosteroids, but response to treatment is variable. To assess the relationship between clinical and paraclinical measures and outcomes in patients with CAA-ri treated with high doses of methylprednisolone. Longitudinal clinical course, and results from serum and cerebrospinal fluid (CSF) testing, electroencephalography, and neuroimaging were reviewed from 11 prospectively-accrued CAA-ri patients diagnosed, treated, and followed at Barnes Jewish Hospital (St. Louis, MO, USA). Magnetic resonance imaging (MRI) changes were quantified using a scoring system validated in cases of amyloid related imaging abnormality (ARIA-E). Clinical outcomes were assessed as change in modified Rankin Scale (ΔmRS) from baseline to final assessment (median 175 days from treatment with high doses of methylprednisolone; range, 31-513). Worse outcomes following methylprednisolone treatment were associated with requirement for intensive care unit admission (median ΔmRS, 5 versus 1.5; p = 0.048), CSF pleocytosis (median ΔmRS 4.5 versus 1; p = 0.04), or lower CSF Aβ40 at presentation (rho = -0.83; p = 0.02), and diffusion restriction (median ΔmRS 4 versus 1.5; p = 0.03) or higher late ARIA-E scores (rho = 0.70; p = 0.02) on MRI, but not preexisting cognitive decline (median ΔmRS 2 versus 2; p = 0.66). Clinical and paraclinical measures associated with outcomes may inform clinical counseling and treatment decisions in patients with CAA-ri. Baseline cognitive status was not associated with treatment responsiveness.