Huppke, Brenda

[["dc.bibliographiccitation.firstpage","232"],["dc.bibliographiccitation.issue","3"],["dc.bibliographiccitation.journal","Pediatric Neurology"],["dc.bibliographiccitation.lastpage","234"],["dc.bibliographiccitation.volume","41"],["dc.contributor.author","Huppke, Peter"],["dc.contributor.author","Heise, Alexander"],["dc.contributor.author","Rostasy, Kevin"],["dc.contributor.author","Huppke, Brenda"],["dc.contributor.author","Gärtner, Jutta"],["dc.date.accessioned","2017-09-07T11:46:52Z"],["dc.date.available","2017-09-07T11:46:52Z"],["dc.date.issued","2009"],["dc.description.abstract","Idiopathic hypothalamic dysfunction is a rare disorder presenting at age 3-7 years. Severe hypothalamic and brainstem dysfunction leads to death in 25% of patients. The disease is presumed to be autoimmune, or in some cases paraneoplastic. No successful treatment has been reported. Patient V. developed hyperphagia, hypersomnia, and extreme aggression at age 7 years, accompanied by episodes of hyperthermia, hypothermia, sinus bradycardia, hypernatremia, hyponatremia, persistent hyperprolactinemia, hypothyroidism, and growth-hormone deficiency. At age 9 years, a diagnosis of idiopathic hypothalamic dysfunction was rendered, and immunoglobulin therapy was commenced. Nine courses of immunoglobulins, at a dose of 2 g/kg every 4 weeks, were administered. Reproducible improvements in behavior and no further episodes of hyponatremia or hypernatremia and sinus bradycardia were evident. The endocrinologic abnormalities and poor thermoregulation remained. Administration of immunoglobulins during late stages of idiopathic hypothalamic dysfunction led to improvement in some but not all signs. Assuming an autoimmune basis for this disorder, treatment during early stages of disease should be more effective. To facilitate such early treatment, increased awareness of this disorder is necessary, to allow for early diagnosis. (C) 2009 by Elsevier Inc. All rights reserved."],["dc.identifier.doi","10.1016/j.pediatrneurol.2009.03.017"],["dc.identifier.gro","3143067"],["dc.identifier.isi","000268866200018"],["dc.identifier.pmid","19664546"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/540"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.publisher","Elsevier Science Inc"],["dc.relation.issn","0887-8994"],["dc.title","Immunoglobulin Therapy in Idiopathic Hypothalamic Dysfunction"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.artnumber","135245851773284"],["dc.bibliographiccitation.firstpage","72"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Multiple Sclerosis Journal"],["dc.bibliographiccitation.lastpage","80"],["dc.bibliographiccitation.volume","25"],["dc.contributor.author","Huppke, Peter"],["dc.contributor.author","Huppke, Brenda"],["dc.contributor.author","Ellenberger, David"],["dc.contributor.author","Rostasy, Kevin"],["dc.contributor.author","Hummel, Hannah"],["dc.contributor.author","Stark, Wiebke"],["dc.contributor.author","Brück, Wolfgang"],["dc.contributor.author","Gärtner, Jutta"],["dc.date.accessioned","2018-04-23T11:47:25Z"],["dc.date.available","2018-04-23T11:47:25Z"],["dc.date.issued","2019-01"],["dc.description.abstract","Objective: Study aims were to determine the frequency of highly active disease in pediatric multiple sclerosis (MS), the response to natalizumab (NTZ) and fingolimod (FTY) treatment, and the impact of current treatment modalities on the clinical course. Methods: Retrospective single-center study in the German Center for MS in Childhood and Adolescence. Results: Of 144 patients with first MS manifestation between 2011 and 2015, 41.6% fulfilled the criteria for highly active MS. In total, 55 patients treated with NTZ and 23 with FTY demonstrated a significant reduction in relapse rate (NTZ: 95.2%, FTY: 75%), new T2 lesions (NTZ: 97%, FTY: 81%), and contrast-enhancing lesions (NTZ: 97%, FTY: 93%). However, seven patients switched from NTZ to FTY experienced an increase in disease activity. Comparing pediatric MS patients treated in 2005 with those treated in 2015 showed a 46% reduction in relapse rate and a 44% reduction in mean Expanded Disability Status Scale (EDSS). Conclusion: The rate of highly active disease among pediatric MS patients is high; more than 40% in our cohort. Response to NTZ and FTY treatment is similar if not better than observed in adults. Current treatment modalities including earlier treatment initiation and the introduction of NTZ and FTY have significantly improved the clinical course of pediatric MS."],["dc.identifier.doi","10.1177/1352458517732843"],["dc.identifier.gro","3142216"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/13338"],["dc.language.iso","en"],["dc.notes.intern","lifescience updates Crossref Import"],["dc.notes.status","final"],["dc.publisher","SAGE Publications"],["dc.relation.eissn","1477-0970"],["dc.relation.issn","1352-4585"],["dc.title","Therapy of highly active pediatric multiple sclerosis"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","no"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","941"],["dc.bibliographiccitation.issue","7"],["dc.bibliographiccitation.journal","Multiple Sclerosis"],["dc.bibliographiccitation.lastpage","946"],["dc.bibliographiccitation.volume","19"],["dc.contributor.author","Huppke, Peter"],["dc.contributor.author","Rostasy, Kevin"],["dc.contributor.author","Karenfort, Michael"],["dc.contributor.author","Huppke, Brenda"],["dc.contributor.author","Seidl, Rainer"],["dc.contributor.author","Leiz, Steffen"],["dc.contributor.author","Reindl, Markus"],["dc.contributor.author","Gärtner, Jutta"],["dc.date.accessioned","2017-09-07T11:47:41Z"],["dc.date.available","2017-09-07T11:47:41Z"],["dc.date.issued","2013"],["dc.description.abstract","Background: Some pediatric patients with inflammatory demyelinating central nervous system disorders cannot be classified under any of the established disease entities, making their treatment and prognosis difficult. Objective: The objective of this study is to characterize a subgroup of pediatric patients with recurrent demyelinating central nervous system disorders. Methods: This study includes a case series of pediatric patients with monophasic or recurrent acute disseminated encephalomyelitis (ADEM) who later presented with either monophasic or recurrent optic neuritis (ON). Results: We describe seven patients with a median follow-up of six years (five females, two males) who presented at a median age of 6 years (range 4-8 years) with monophasic (n = 4) or recurrent ADEM (two to four attacks) followed by monophasic (n = 3) or recurrent ON (two to nine attacks). Cranial magnetic resonance imaging (MRI) was typical for ADEM (n = 6) with complete or almost complete resolution of lesions on follow-up. Cerebrospinal (CSF) studies at the time of ADEM showed a pleocytosis in six patients and were negative for oligoclonal bands (OCBs) in all. In all patients high titers for serum anti-MOG antibodies were detected. Conclusion: ADEM followed by ON is a rare but distinct clinical phenotype among pediatric patients. Further studies are needed to allow recommendations on treatment or prognosis."],["dc.identifier.doi","10.1177/1352458512466317"],["dc.identifier.gro","3142346"],["dc.identifier.isi","000319567900018"],["dc.identifier.pmid","23128668"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/13095"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/7264"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.intern","Merged from goescholar"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.publisher","Sage Publications Ltd"],["dc.relation.issn","1352-4585"],["dc.rights","Goescholar"],["dc.rights.uri","https://goescholar.uni-goettingen.de/licenses"],["dc.title","Acute disseminated encephalomyelitis followed by recurrent or monophasic optic neuritis in pediatric patients"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]