Factors influencing intravenous methylprednisolone pulse therapy in Chinese patients with isolated optic neuritis associated with AQP4 antibody-seropositive neuromyelitis optica

This study investigated the factors influencing intravenous methylprednisolone pulse (IVMP) therapy for recovering visual acuity in Chinese patients with aquaporin-4 (AQP4) antibody-seropositive neuromyelitis optica-related optic neuritis (NMO-ON). This retrospective case series included 243 affected eyes of 182 patients (36 male, 146 female) diagnosed with NMO-ON in the Neuro-Ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to September 2020. All patients with AQP4-antibody seropositivity had clinical manifestations of acute ON, excluding other diagnoses and received IVMP treatment at 500 mg/day or 1000 mg/day for 3 days. Primary outcome was the extent of improvement in logMAR visual acuity after IVMP treatment. The therapeutic influences of sex, age, baseline visual acuity, therapeutic intervals, and IVMP dose on acute NMO-ON were analysed. Chi-square tests, Mann–Whitney U-tests, Kruskal–Wallis tests, Spearman’s correlation coefficients, and multiple linear regression were used for statistical analysis. Age ranged between 7 and 80 years (median age, 44; interquartile range [IQR], 29–52) years. Among the 243 eyes, the median improvement in logMAR visual acuity was 0.3 (IQR, 0–0.9). Therapeutic efficacy of IVMP was significantly higher in female than in male patients (Z = 2.117, P = 0.034). The treatment effect gradually decreased with increase in age at onset (Rs = 0.157, P = 0.015), and visual improvement was significantly lower in patients aged > 50 years than in those ≤ 50 years (Z = 2.571, P = 0.010). When patients had low visual acuity at onset, improvements were more obvious (rho = − 0.317, P < 0.001); however, final visual acuity was still low (rho = 0.688, P < 0.001). Therapeutic effect was negatively correlated with therapeutic intervals (rho = 0.228, P = 0.001). Dosage of methylprednisolone (1000 mg/day or 500 mg/day) did not significantly influence treatment efficacy (Z = 0.951 P = 0.342). Therefore, IVMP therapy can improve visual acuity in the affected eyes of patients with AQP4 antibody-seropositive NMO-ON with similar effect at 500 mg/day and 1000 mg/day doses. Sex, age at onset, and therapeutic intervals may influence the efficacy of IVMP in patients with NMO-ON.

Successful Treatment of IgA Vasculitis With Prolonged Cutaneous Manifestation With Colchicine in a 10-Year-Old Boy

We report a 10-year-old boy with IgA vasculitis (IgAV) with prolonged cutaneous manifestations who was successfully treated with colchicine. At the age of 9, he was diagnosed as having IgAV by typical purpura, abdominal pain, and hematochezia. Initially, his severe gastrointestinal manifestation subsided by prednisolone 60 mg/day and intravenous methylprednisolone pulse therapy. However, his gastrointestinal manifestation was glucocorticoid-dependent and refractory to factor XIII concentrate, intravenous immunoglobulin G, and mycophenolate mofetil. His abdominal pain and hematochezia responded to the combination therapy with dapsone and low dose of prednisolone 5 mg/day and did not relapse even after discontinuation of dapsone. On the other hands, the effect of dapsone on his cutaneous manifestation was dose-dependent. As well dapsone had no glucocorticoid-sparing effect. Approximately 12 months after onset, colchicine treatment was started, which resulted in remission of his chronic cutaneous manifestation. After prednisolone was tapered off, his cutaneous manifestation is currently well-controlled on colchicine 0.5 mg/day without adverse events. He had never complicated by kidney involvements. In conclusion, colchicine treatment exerts a beneficial effect in IgAV patients with prolonged cutaneous manifestation refractory to multiple drugs.

Therapeutic Response and Possible Biomarkers in Acute Attacks of Neuromyelitis Optica Spectrum Disorders: A Prospective Observational Study

ObjectiveTo explore the outcomes of NMOSD attacks and investigate serum biomarkers for prognosis and severity.MethodPatients with NMOSD attacks were prospectively and observationally enrolled from January 2019 to December 2020 at four hospitals in Guangzhou, southern China. Data were collected at attack, discharge and 1/3/6 months after acute treatment. Serum cytokine/chemokine and neurofilament light chain (NfL) levels were examined at the onset stage.ResultsOne hundred patients with NMOSD attacks were included. The treatment comprised intravenous methylprednisolone pulse therapy alone (IVMP, 71%), IVMP combined with apheresis (8%), IVMP combined with intravenous immunoglobulin (18%) and other therapies (3%). EDSS scores decreased significantly from a medium of 4 (interquartile range 3.0–5.5) at attack to 3.5 (3.0–4.5) at discharge, 3.5 (2.0–4.0) at the 1-month visit and 3.0 (2.0–4.0) at the 3-month visit (p&lt;0.01 in all comparisons). The remission rate was 38.0% at discharge and 63.3% at the 1-month visit. Notably, relapse occurred in 12.2% of 74 patients by the 6-month follow-up. Higher levels of T helper cell 2 (Th2)-related cytokines, including interleukin (IL)-4, IL-10, IL-13, and IL-1 receptor antagonist, predicted remission at the 1-month visit (OR=9.33, p=0.04). Serum NfL levels correlated positively with onset EDSS scores in acute-phase NMOSD (p&lt;0.001, R2 = 0.487).ConclusionsOutcomes of NMOSD attacks were generally moderate. A high level of serum Th2-related cytokines predicted remission at the 1-month visit, and serum NfL may serve as a biomarker of disease severity at attack.Clinical Trial Registrationhttps://clinicaltrials.gov/ct2/show/NCT04101058, identifier NCT04101058.

Effects of Glucocorticoid Pulse Therapy on Thyroid Function and Thyroid Antibodies in Children with Graves’ Disease

Objective The purpose of this study is to observe the effects of glucocorticoid pulse therapy on thyroid function and thyroid antibodies in children with Graves’ disease (GD).Methods Twenty children who were treated by intravenous methylprednisolone pulse therapy (MPT) followed by oral prednisolone administration and antithyroid drugs were included in the pulse group. Twenty children who were treated with antithyroid drugs alone were included in the control group. Serum concentrations of free triiodothyronine (FT3), free thyroxine (FT4), thyrotropin (TSH), thyroid peroxidase antibodies (TPOAb), thyroglobulin antibodies (TGAb), and thyrotropin receptor antibodies (TRAb) were recorded at baseline and 10 days, 30days, and 60 days after treatment. Results Significant differences in FT3, FT4, TSH, TPOAb, TGAb, and TRAb levels were found in both groups from baseline to follow-up time points (all P<0.05). On the 30th day, the TRAb level in the pulse group was significantly lower than that in the control group (P=0.023). However, the level of TRAb rose on the 60th day. For values of TRAb at baseline, 10 days, and 60 days after treatment, there were no significant differences respectively between the two groups (all P>0.05). No significant differences were observed in FT3, FT4, TSH, TPOAb, and TGAb levels between the two groups (all P>0.05). Conclusion Our results suggest that the effects of intravenous MPT followed by oral prednisolone on TRAb level are temporary in children with GD. It is not helpful to the sustained recovery of thyroid function.

Polyarteritis nodosa with perirenal hematoma due to the rupture of a renal artery aneurysm

We present the case of a 67-year-old man in good health with perirenal hematoma due to a ruptured arterial aneurysm in the kidney. The patient developed weight loss, muscle weakness, multiple mononeuropathy, hypertension, anemia, renal insufficiency, and multiple lacuna infarctions about a month ago. He was admitted to the hospital due to worsening of his symptom. After admission, severe right-flank pain suddenly occurred; he was then transferred to our hospital. Renal angiography revealed bilateral multiple microaneurysms, and the patient was diagnosed with polyarteritis nodosa based on the clinical, radiographic, and histological findings. We performed selective coil embolization to the ruptured aneurysm and administered oral prednisolone along with intravenous methylprednisolone pulse therapy. Cyclophosphamide pulse therapy was also given. The treatment improved clinical and laboratory findings and achieved clinical remission. Selective coil embolization to the bleeding aneurysm of polyarteritis nodosa was minimally invasive and promptly effective. Immunosuppressants proved useful in the regulation of disease activity and the aneurysm.

A refractory anti-NMDA receptor encephalitis successfully treated by bilateral salpingo-oophorectomy and intrathecal injection of methotrexate and dexamethasone: a case report

Introduction Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an autoimmune-mediated disease that is common in young female patients with ovarian teratomas. With appropriate immunotherapy, most patients achieve a good prognosis. Nevertheless, some patients may be refractory to first- and second-line immunotherapy, thus alternative treatments are required for these patients. Case presentation: We present a case of anti-NMDA receptor encephalitis with ovarian teratoma. After the prompt removal of the teratoma and intense immunotherapy was administered, including an intravenous methylprednisolone pulse, intravenous immunoglobin, plasmapheresis, immunoadsorption, intravenous cyclophosphamide, and rituximab, the patient’s neurologic status did not improve. Bilateral salpingo-oophorectomy was then conducted, and intrathecal injection of methotrexate (MTX) and dexamethasone (DXM) was performed. The patient’s neurological symptoms improved dramatically, and she achieved a good prognosis after 23 months. Conclusions Intrathecal injection of MTX and DXM may be beneficial for treatment of refractory cases of anti-NMDA receptor encephalitis. Additional research is required to elucidate the mechanisms of intrathecal treatment with this therapy.

A refractory anti-NMDA receptor encephalitis successfully treated by bilateral ovariectomy and intrathecal injection of methotrexate and dexamethasone: A case report

Background: Anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis is an autoimmune-mediated disease, which is predominant in young female patients with ovarian teratomas. With proper immunotherapy, most of the patients achieve good prognosis. Nevertheless, some patients may be refractory to first and second-line immunotherapy, thus new treatments are required to help these patients. Case presentation: We present a case of anti-NMDA receptor encephalitis with ovary teratoma. After the prompt removal of the teratoma and strong immunotherapy including intravenous methylprednisolone pulse (IVMP), intravenous immunoglobin (IVIG), plasmapheresis, immunoadsorption, intravenous cyclophosphamide, and rituximab, the patient’s neurologic status did not improve. Bilateral ovariectomy was then conducted and intrathecal injection of methotrexate (MTX) and dexamethasone (DXM) was given. The patient’s neurological symptoms improved dramatically， and she achieved a good prognosis after 23 months. Conclusions: For refractory cases of anti-NMDA receptor encephalitis, intrathecal injection of MTX and DXM may be beneficial. More research is required to elucidate the mechanisms of intrathecal treatment.

Efficacy of methylprednisolone therapy in atypical Rolandic epilepsy with heterozygous RELN mutation

Background: Atypical Rolandic epilepsy, also known as benign childhood epilepsy with centrotemporal spikes (BECTS) variant is defined by the appearance of severe neuropsychological impairments and refractory epilepsy. The etiology of the disease remains unclear, and recent studies indicated that it is related to several gene mutations. The suitable treatment is also need to be further explored. Here, we present the case of a 9-year-old boy with BECTS variant found to have heterozygous RELN mutation, responded well to the corticosteroid therapy. Case presentation: A 9-year-old male patient with atypical benign Rolandic epilepsy was successfully treated with high-dose intravenous methylprednisolone pulse therapy (15 mg/kg daily for 3 consecutive days, and the infusion was repeated three times with a 4-day interval between each course). The treatment improved his electroencephalogram (EEG) and cognitive performance, reduced seizure frequency, and the effect maintained for one year of follow-up. Whole-exome sequencing (WES) revealed a meaningful heterozygous missense mutation in the RELN gene. Conclusion: We conclude that corticosteroid therapy should be considered as a therapeutic option in patients with BECTS variant who are also found to harbour RELN mutations.