 | Konomi ShimodaDepartment of Pediatrics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan | Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, ... |
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Konomi Shimoda:Expert Impact
Concepts for whichKonomi Shimodahas direct influence:Vici syndrome,Opitz syndrome,Static encephalopathy,Video report,Acute stage,Murinederived antigens,Receptor encephalitis,Aspartate receptor.
Konomi Shimoda:KOL impact
Concepts related to the work of other authors for whichfor which Konomi Shimoda has influence:Vici syndrome,Nmdar encephalitis,Aspartate receptor,Streptococcal infections,Tic disorders,Finca disease,Teratoma removal.
KOL Resume for Konomi Shimoda
| Year | |
|---|---|
| 2017 | Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan |
| 2013 | Department of Neuropediatrics, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo 183-0042, Japan |
| 2012 | Department of Neuropediatrics, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan |
| Concept | World rank |
|---|---|
| vici syndrome vicis | #44 |
| syndrome vicis | #44 |
| static encephalopathy childhood | #50 |
| vicis | #54 |
| video report | #59 |
| neurodegeneration adulthood | #66 |
| gabhs infection | #69 |
| mutations epg5 | #77 |
| childhood neurodegeneration | #80 |
| shortlived plasmablasts | #82 |
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Prominent publications by Konomi Shimoda
Rituximab ameliorates anti-N-methyl-d-aspartate receptor encephalitis by removal of short-lived plasmablasts
[ PUBLICATION ]
We measured anti-N-methyl-D-aspartate receptor (NMDAR) autoantibody levels and assessed B cell subsets using multicolor flow cytometry of peripheral blood mononuclear cells (PBMCs) from a recurrent anti-NMDAR encephalitis case to evaluate the effectiveness of rituximab treatment. Rituximab depleted CD20(+) fractions of naïve and memory B cell subsets and reduced the number of CD20(-) plasmablasts. This study suggests that short-lived plasmablasts are removed by rituximab-induced ...
| Known for Receptor Encephalitis | Murinederived Antigens | Shortlived Plasmablasts | Methyl Aspartate | Rituximab Treatment |
Defects in autophagosome-lysosome fusion underlie Vici syndrome, a neurodevelopmental disorder with multisystem involvement
[ PUBLICATION ]
Vici syndrome (VICIS) is a rare, autosomal recessive neurodevelopmental disorder with multisystem involvement characterized by agenesis of the corpus callosum, cataracts, cardiomyopathy, combined immunodeficiency, developmental delay, and hypopigmentation. Mutations in EPG5, a gene that encodes a key autophagy regulator, have been shown to cause VICIS, however, the precise pathomechanism underlying VICIS is yet to be clarified. Here, we describe detailed clinical (including brain MRI and ...
| Known for Vici Syndrome | Multisystem Involvement | Neurodevelopmental Disorder | Corpus Callosum | Epg5 Gene |
Novel DHCR7 mutation in a case of Smith–Lemli–Opitz syndrome showing 46,XY disorder of sex development
[ PUBLICATION ]
Smith–Lemli–Opitz syndrome is an autosomal recessive disease caused by mutations in 7-dehydrocholesterol reductase (DHCR7), which is rarely observed in Japan. We report a Japanese case with 46,XY disorder of sex development and Y-shaped 2–3 toe syndactyly. DHCR7 gene analysis revealed compound heterozygous mutations including the novel mutation H442R. Early diagnosis led to starting cholesterol treatment at an early age.
| Known for Opitz Syndrome | Sex Development | Smith Lemli | Early Diagnosis | 7dehydrocholesterol Reductase |
Autoimmune neurological disorders associated with group-A beta-hemolytic streptococcal infection
[ PUBLICATION ]
Although central nervous system (CNS) disorders associated with group-A beta-hemolytic streptococcal (GABHS) infection occur only rarely, Sydenham's chorea is a well-recognized disease that can arise following infection. Children may develop a tic, obsessive compulsive disorder (OCD), and extrapyramidal movement subsequent to GABHS infection. These disorders have been termed pediatric autoimmune neuropsychiatric disorders associated with streptococci (PANDAS). Herein we report one case ...
| Known for Gabhs Infection | Autoimmune Diseases | Streptococcus Pyogenes | Nervous Cns | Beta Hemolytic |
First video report of static encephalopathy of childhood with neurodegeneration in adulthood
[ PUBLICATION ]
| Known for Static Encephalopathy | Video Report | Childhood Neurodegeneration | Neurodegenerative Diseases |
Ataxia-telangiectasia is a chronic progressive disorder affecting the nervous and immune systems, caused by a genetic defect in the ATM protein. Clasmatodendrosis, a distinct form of astroglial death, has rarely been reported in ataxia-telangiectasia. Neuropathology of our patient disclosed diffuse edema of the cerebral and cerebellar white matter with prominent clasmatodendrosis, implicating ATM in the regulation of astroglial cell death.
| Known for Ataxia Telangiectasia | White Matter |
