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指定難病 — No.151

ラスムッセン脳炎

検索語 Rasmussen Encephalitis ・ 最終更新 2026-07-23 00:16 ・ 最新に更新

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指定 No.151
Src PubMed · CT.gov · jRCT

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( 01 )EVIDENCE / PUBMED · 5件

世界の論文

直近の研究を、やさしい日本語で

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ランダム化比較試験(RCT)
MK-01 · PMID 42485251

Fluoroquinolones versus doxycycline in the treatment of rickettsial infection: A Tunisian multicenter prospective study

Abstract / 原文

INTRODUCTION: Doxycycline is considered as the standard treatment of rickettsiosis.Fluoroquinolones used as an alternative therapy, until recent studies have shown that Fluoroquinolones are associated with a deleterious outcome Objectives : To compare therapeutic efficacy and safety of fluoroquinolones and doxycycline in the treatment of Rickettsiosis Methods: we conducted an open-label randomized controlled multicenter study. This study aim to compare the efficacy and safety of oral ciprofloxacin (1000 mg/day) or ofloxacin (400mp/day) in 2 doses with those of oral doxycycline (200mg/day) once daily for 3 to 10 days, according to the severity of illness, in the treatment of Rickettsioses, We included patients more than 15 year-old who presented the characteristic clinical triad (eschar + rash + fever) and/or microbiological confirmation (positive serology and/or positive qPCR). RESULTS: Among 112 enrolled patients, 82 retained. Thirty-four patients received fluoroquinolones and 48 received doxycycline. Patients in both groups were comparable with respect to age. Two severe cases were noted with fluoroquinolones (meningitis = one, encephalitis= 1) and three with doxycycline (myocarditis=one, severe sepsis = l and encephalitis =1) .|The defervescence period was 2.6 + 1.04 days in the doxycycline group and2.31.09 days in the fluoroquinolones group (p20.05). All patients recovered. One patient treated by doxycycline had a digital necrosis, generating amputation. CONCLUSIONS: Both fluoroquinolones and doxycycline were effective for treating patients with rickettsia infections. Even that doxycycline remains the standard of treatment, with the advantage of a single daily dose; fluoroquinolones are an interesting alternative with no deleterious outcome.

Journal
La Tunisie medicale(2026 Apr)
Authors
8名
Type
Journal Article, Multicenter Study, Randomized Controlled Trial, Comparative Study
PubMedで原文を見る
不明
MK-02 · PMID 42484852

Efgartigimod as rescue therapy in refractory anti-GABAB receptor encephalitis: a case report

Journal
Journal of neurology(2026 Jul)
Authors
6名
Type
Letter
PubMedで原文を見る
基礎研究(細胞・動物など)
MK-03 · PMID 42484489

Integrating Cerebrospinal Fluid Metagenomic Next-Generation Sequencing and Immune Profiling in Recurrent HSV-1 Encephalitis: A Case Report and Narrative Review

Abstract / 原文

BackgroundRecurrent herpes simplex virus type 1 (HSV-1) encephalitis in children is rare, and its pathophysiology remains incompletely understood. Both viral reactivation and host immune dysregulation have been implicated. Advances in metagenomic next-generation sequencing (mNGS) and immune profiling provide new opportunities to elucidate disease mechanisms.Case Presentation: We detail a 13-year-old boy of Qiang ethnicity who experienced 3 neurologic episodes, including 2 virologically confirmed HSV-1 encephalitis events over 7 years. The third recurrence involved fever, seizures, and progressive bilateral temporal lobe lesions visible on magnetic resonance imaging. Cerebrospinal fluid (CSF) mNGS confirmed HSV-1 reactivation, and viral genomic sequencing demonstrated a highly conserved viral genome without high-confidence nonsynonymous mutations. Immune profiling showed compartmentalized central nervous system inflammation with elevated CSF cytokines (interleukin [IL]-6, IL-8, IL-10, interferon [IFN]-α, IFN-γ) and altered lymphocyte subsets, despite normal serum results. The patient was treated with acyclovir, intravenous immunoglobulin, and low-dose corticosteroids, which controlled seizures but left persistent neurocognitive deficits. Multidisciplinary follow-up is crucial to mitigate long-term neurocognitive sequelae.Literature Review: We reviewed 10 previously published pediatric cases of recurrent HSV-1 encephalitis, which demonstrated heterogeneous recurrence intervals, contralateral or novel lesion involvement, and frequent cognitive sequelae. Few studies integrated viral genomics or immune profiling.ConclusionsThe findings suggest that recurrent pediatric HSV-1 encephalitis may be driven by viral reactivation in the context of CNS-restricted immune dysregulation, rather than reinfection or viral evolution.

Journal
Journal of child neurology(2026 Jul)
Authors
6名
Type
Journal Article
PubMedで原文を見る
観察研究
MK-04 · PMID 42483856

Chronic Kidney Disease Frequently Observed in Adults With Spina Bifida

Abstract / 原文

OBJECTIVES: We evaluated the association between chronic kidney disease and the current and background conditions of adults with spina bifida. METHODS: Patients with spina bifida (group A, n = 34) and patients with a history of pediatric encephalitis or spinal cord injury (group B, n = 23) who were older than 18 years between January 2013 and August 2022 were retrospectively analyzed. The chronic kidney disease-free survival was compared between groups. In each group, the congenital or pediatric background conditions (gender, disease type, history of febrile urinary tract infection, bladder augmentation, vesico-ureteral reflux, or detrusor overactivity) and current background conditions (age at last follow-up, ambulatory status, hypertension, and albuminuria) were compared between patients with or without chronic kidney disease. RESULTS: The median age was 37 years (range 20-67 years). The median chronic kidney disease-free survival was younger in group A than group B (48.0 vs. 65.0 years, p = 0.0027). In group A, chronic kidney disease was associated with pediatric febrile urinary tract infection (p = 0.0018) and vesico-ureteral reflux (p = 0.0090), current hypertension (p < 0.001), and albuminuria (p = 0.0053). Multivariate analysis showed only hypertension was significantly associated with chronic kidney disease. CONCLUSION: Young to middle-aged adults with spina bifida with a history of pediatric febrile urinary tract infection or vesico-ureteral reflux and those with current hypertension or albuminuria have an increased risk of chronic kidney disease, suggesting the need for renal function monitoring.

Journal
International journal of urology : official journal of the Japanese Urological Association(2026 Jul)
Authors
3名
Type
Journal Article
PubMedで原文を見る
観察研究
MK-05 · PMID 42483187

Clinical implications of free triiodothyronine levels and diagnostic revisions in antibody-negative autoimmune encephalitis

Abstract / 原文

BACKGROUND AND OBJECTIVE: Low triiodothyronine (T3) syndrome has been associated with initial clinical severity and poor long-term functional outcomes in autoimmune encephalitis (AE). However, its prognostic implications in antibody-negative AE remain unclear. The objective of this study was to explore the clinical significance of low T3 syndrome in patients with antibody-negative AE, and to assess the clinical characteristics of patients reclassified with alternative diagnoses during follow-up. METHODS: We conducted a retrospective cohort study of patients initially diagnosed with antibody-negative AE between January 2016 and June 2024. Patients were divided into two groups based on the presence or absence of low T3 syndrome. Demographics, clinical features, and ancillary test results were compared between the subgroups. Modified Rankin Scale (mRS) scores were used to evaluate neurological function during hospitalization and at the 12-month follow-up. In addition, we analyzed reclassified patients and their clinical outcomes to improve clinical management of antibody-negative AE. RESULTS: Of the 84 patients initially diagnosed with antibody-negative AE at discharge, eight were reclassified during their disease course (non-autoimmune encephalitis, n=4; paraneoplastic encephalitis, n=3; neuropsychiatric systemic lupus erythematosus, n=1; these eight patients were defined as reclassified group) and were excluded from the antibody-negative AE group. A total of 76 patients were ultimately enrolled. Of these, 23.68% presented with low T3 syndrome during the acute phase. Subgroup analysis further showed that patients with low T3 syndrome had a higher incidence of consciousness disturbances (p = 0.048), more frequent motor impairments, and higher scores on the modified Rankin Scale (mRS) throughout hospitalization. Overall, 61.84% (47/76) of patients achieved a favorable prognosis, whereas 38.16% (29/76) had an unfavorable outcome. Notably, low T3 syndrome was associated with poor prognosis in univariable analysis but not after multivariable adjustment. Discharge mRS was an independent predictor of unfavorable outcome (OR 0.293, 95% CI 0.103-0.834, p = 0.021). Furthermore, the reclassified group demonstrated a higher recurrence rate compared with the antibody-negative AE group. CONCLUSION: Acute-phase low T3 syndrome is common in antibody-negative AE but appears to reflect disease severity rather than serving as an independent prognostic biomarker; discharge mRS is a more reliable predictor. Larger prospective studies are needed to clarify the prognostic role of thyroid hormone alterations in this population. Furthermore, a subset of patients (8/84, 9.5%) initially diagnosed with antibody-negative AE received alternative diagnoses after 12 months of follow-up, highlighting diagnostic uncertainty and the urgent need to identify clinical red flags for re-evaluation.

Journal
Frontiers in immunology(2026)
Authors
9名
Type
Journal Article
PubMedで原文を見る
( 02 )TRIALS / JAPAN · 0件

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( 03 )REGISTRY / jRCT

治験をもっと探す

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