Pulse Methylprednisolone with Oral Prednisolone versus Adrenocorticotropic Hormone in Children with West Syndrome: a Randomized Controlled Trial

Background and Purpose: West syndrome is an epileptic encephalopathy of infancy. According to guidelines, adrenocorticotrophic hormone (ACTH) is probably effective for the short-term management of infantile spasm, but there is little uniformity in treatment due to variable response. This study has been done to evaluate the efficacy of pulse methylprednisolone as compared to ACTH in children with West syndrome.Methods: Children between 3 months to 24 months with the diagnosis of West syndrome were included and ACTH and pulse methyl prednisolone followed by oral prednisolone were given after randomization. Total duration of treatment was 6 weeks in both groups.Results: Total 87 children were enrolled; 12 patients lost in follow up. Finally, 43 received ACTH and 32 received pulse methylprednisolone. In pulse methylprednisolone group, 28.13% showed 50-80% response, 28.13% showed 80-99% response and 21.87% patients showed 100% response. In ACTH group, 41.86% showed 50-80% response, 25.58% showed 80-99% response and only 3 (6.97%) patients showed 100% response. Methylprednisolone treatment regimen did not cause significant or persistent adverse effects.Conclusions: Pulse methylprednisolone followed by oral prednisolone for 6 weeks is as effective as ACTH. Thus, methylprednisolone therapy can be an important alternative to ACTH.

A Case of Influenza Virus-Induced Acute Cerebellitis Treated with Steroid Pulse Therapy

Acute cerebellitis (AC) is characterized by acute onset cerebellar ataxia brain magnetic resonance imaging (MRI) abnormalities of the cerebellum. The most common cause of AC is viral infection, and some patients with AC experience neurological sequelae. AC associated with influenza virus is extremely rare, and its prognosis and treatment are unknown. We present the case of a 2-year-old boy with influenza virus-induced AC who was treated with pulse steroid therapy. The patient presented with fever, anorexia, vomiting, malaise, altered consciousness, truncal ataxia, dysmetria, and dysarthria. He was diagnosed with influenza using a nasopharyngeal antigen test. Brain MRI showed hyperintense T2 and diffusion-weighted signal abnormalities in the cerebellar white matter and dentate nuclei bilaterally. The patient was treated with two courses of pulse methylprednisolone therapy and recovered completely in 2 months after the onset. The prognosis of AC is poorer than that of acute cerebellar ataxia, which shows similar symptoms to AC with normal brain MRI. The type of virus might also be associated with the prognosis of AC. Literature review showed that one of the five cases (including the present case, 20%) reported with influenza-associated AC was noted to have neurological sequelae, which might be more severe than those of varicella-zoster-related AC. Given that the pathogenesis of AC is assumed to be immune-mediated, pulse methylprednisolone therapy might be a good option for the treatment of influenza virus-induced AC.

Severe Adult Still’s Disease Complicated by Purtscher-Like Retinopathy Treated with Intravenous Pulse Methylprednisolone and Tocilizumab

Adult Still’s disease (ASD) is a rare systemic inflammatory disorder in which ocular manifestations have rarely been described. We report a 29-year-old Japanese woman with a rare case of refractory ASD complicated by Purtscher-like retinopathy. She was diagnosed with ASD and started on a high dose of oral prednisolone. Two days after the initiation of the treatment, she presented with blurred vision in the left eye, and the funduscopic examination revealed bilateral Purtscher-like retinopathy. Despite treatment with high-dose oral prednisolone for 2 weeks, she developed macrophage activation syndrome. Considering the severity of ASD, intravenous pulse methylprednisolone therapy and tocilizumab injection were administered. Although all the laboratory data and Purtscher-like retinopathy gradually improved, nerve fiber layer defect (NFLD) in both eyes appeared and visual field defect remained corresponding to the NFLD. In conclusion, Purtscher-like retinopathy might be useful as a poor prognostic factor of ASD, which needs appropriate systemic immunosuppressive treatment. Early detection and long-term follow-up of Purtscher-like retinopathy is important because it has the possibility of developing permanent visual field defect.

AIRE Gene Mutation Presenting at Age 2 Years With Autoimmune Retinopathy and Steroid-Responsive Acute Liver Failure: A Case Report and Literature Review

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare monogenic autosomal recessive disorder caused by mutation in the autoimmune regulator (AIRE) gene. Patients usually are diagnosed at ages between 5 and 15 years when they show 3 or more manifestations, most typically mucocutaneous candidiasis, Addison’s disease, and hypoparathyroidism. APECED-associated hepatitis (APAH) develops in only 10% to 40% of patients, with severity varying from subclinical chronic active hepatitis to potentially fatal acute liver failure (ALF). Ocular abnormalities are fairly common, most often keratopathy but sometimes retinopathy. Here we report a 2-year-old Japanese girl with an AIRE gene mutation who developed APAH with ALF, preceded by autoimmune retinopathy associated with anti-recoverin antibody before major symptoms suggested a diagnosis of APECED. Intravenous pulse methylprednisolone therapy followed by a corticosteroid combined with azathioprine treatment resolved ALF and achieved control of APAH. To our knowledge, our patient is the youngest reported to have ALF resulting from an AIRE gene mutation. Pulse methylprednisolone induction therapy followed by treatment with corticosteroid plus azathioprine may well be effective in other children with APAH and AIRE gene mutations.

Anti-MOG antibody associated long segment myelitis presenting as anterior cord syndrome

A patient having clinical features reminiscent of anterior cord syndrome (ACS) was found to have long segment myelitis on MRI. Investigations revealed serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody positivity. He was treated with pulse methylprednisolone followed by immunosuppressant therapy with mycophenolate mofetil, which led to clinical recovery. Anterior cord syndrome has so far not been reported in the context of anti-MOG antibody associated disease.

Case Report: Catheter-Directed Endovascular Thrombolysis for Refractory Cerebral Venous Sinus Thrombosis in a Patient With Behçet Disease

Background: Behçet disease (BD) is an autoimmune and vasculitic disorder. BD affects the veins more frequently than the arteries. The cerebral venous sinus thrombosis (CVST) was reported to occur in about 20% of neuro-BD. Huge dilemma exists in the treatment of CVST with BD, some of which were refractory to the standard therapy.Case Presentation: Here, we report a BD case with refractory intracranial hypertension caused by CVST which is successfully treated with catheter-directed endovascular thrombolysis. Before endovascular thrombolysis, intravenous pulse methylprednisolone combined with anticoagulant therapy was used, and resulted in limited effects.Conclusion: We conclude that catheter-directed thrombolysis may be considered for refractory CVST with BD, provided that coexistent pulmonary and cerebral artery aneurysms are ruled out.