Brockmann, Knut

[["dc.bibliographiccitation.firstpage","893"],["dc.bibliographiccitation.issue","12"],["dc.bibliographiccitation.journal","Neuroradiology"],["dc.bibliographiccitation.lastpage","898"],["dc.bibliographiccitation.volume","48"],["dc.contributor.author","Dreha-Kulaczewski, Steffi F."],["dc.contributor.author","Dechent, Peter"],["dc.contributor.author","Helms, Gunther"],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Gärtner, Jutta"],["dc.contributor.author","Brockmann, Knut"],["dc.date.accessioned","2017-09-07T11:49:54Z"],["dc.date.available","2017-09-07T11:49:54Z"],["dc.date.issued","2006"],["dc.description.abstract","Introduction Hereditary spastic paraplegia with thin corpus callosum (HSP-TCC) is a complicated form of autosomal-recessive hereditary spastic paraplegia. Characteristic clinical features comprise progressive spastic gait, cognitive impairment, and ataxia. Diagnostic MRI findings include thinning of the corpus callosum and non-progressive white matter (WM) alterations. Methods To study the extent of axonal involvement, we performed localized proton magnetic resonance spectroscopy (MRS) of the cerebral WM and cortical grey matter (GM) in a patient with HSP-TCC at 20 and 25 years of age. The second investigation included diffusion tensor imaging (DTI). Results While MRS of the GM was normal, affected WM was characterized by major metabolic alterations such as reduced concentrations of N-acetylaspartate and N-acetylaspartyl-glutamate, creatine and phosphocreatine, and choline-containing compounds as well as elevated levels of myo-inositol. These abnormalities showed progression over a period of 5 years. DTI revealed increased mean diffusivity as well as reduced fractional anisotropy in periventricular WM. The metabolic and structural findings are consistent with progressive neuroaxonal loss in the WM accompanied by astrocytic proliferation-histopathological changes known to occur in HSP-TCC. Conclusion Our results are in agreement with the hypothesis that the primary pathological process in HSP-TCC affects the axon, possibly due to impaired axonal trafficking."],["dc.identifier.doi","10.1007/s00234-006-0148-2"],["dc.identifier.gro","3143582"],["dc.identifier.isi","000242365500005"],["dc.identifier.pmid","17013586"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/1111"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.publisher","Springer"],["dc.relation.issn","0028-3940"],["dc.title","Cerebral metabolic and structural alterations in hereditary spastic paraplegia with thin corpus callosum assessed by MRS and DTI"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","701"],["dc.bibliographiccitation.issue","5"],["dc.bibliographiccitation.journal","Neurology"],["dc.bibliographiccitation.lastpage","706"],["dc.bibliographiccitation.volume","65"],["dc.contributor.author","Hanefeld, Folker"],["dc.contributor.author","Brockmann, Knut"],["dc.contributor.author","Pouwels, Petra J. W."],["dc.contributor.author","Wilken, Barbara"],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Dechent, Peter"],["dc.date.accessioned","2018-11-07T10:55:47Z"],["dc.date.available","2018-11-07T10:55:47Z"],["dc.date.issued","2005"],["dc.description.abstract","Background: Pelizaeus-Merzbacher disease (PMD) is a rare X-linked recessive neurologic disorder caused by a mutation in the proteolipid protein (PLP) gene on chromosome Xq22. The associated depletion of PLP and severe reduction of other major myelin proteins results in dysmyelination. MRI reveals loss of T1 contrast between gray and affected white matter and T2 hyperintensities of white matter due to elevated water content. Methods: In vivo proton magnetic resonance spectroscopy (MRS) was used to determine cerebral metabolite patterns in five patients with genetically proven PMD. Absolute metabolite concentrations were obtained in cortical gray matter, affected white matter, and basal ganglia and compared to age-matched control values. Results: In comparison to age-matched controls, MRS of affected white matter resembled the metabolite pattern of cortical gray matter, as indicated by increased concentrations of N-acetylaspartate and N-acetylaspartylglutamate (tNAA), glutamine (Gln), myo-inositol ( Ins), and creatine and phosphocreatine. Most remarkably, the concentration of choline-containing compounds was reduced. Parietal gray matter and basal ganglia appeared normal but showed a tendency for elevated tNAA, Gln, and Ins. Conclusions: Magnetic resonance spectroscopy (MRS)-detected alterations are consistent with enhanced neuroaxonal density, astrogliosis, and reduction of oligodendroglia. These disturbances in cellular composition are in close agreement with the histopathologic features characteristic of dys- and hypomyelination. The proton MRS profile of Pelizaeus-Merzbacher disease (PMD) differs from the pattern commonly observed in demyelinating disorders and allows PMD to be distinguished from other leukodystrophies."],["dc.identifier.doi","10.1212/01.wnl.0000174642.32187.20"],["dc.identifier.isi","000231821300009"],["dc.identifier.pmid","16157902"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/49865"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Lippincott Williams & Wilkins"],["dc.relation.issn","0028-3878"],["dc.title","Quantitative proton MRS of Pelizaeus-Merzbacher disease - Evidence of dys- and hypomyelination"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","444"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Pediatric Research"],["dc.bibliographiccitation.lastpage","449"],["dc.bibliographiccitation.volume","63"],["dc.contributor.author","Dreha-Kulaczewski, Steffi F."],["dc.contributor.author","Dechent, Peter"],["dc.contributor.author","Finsterbusch, Jurgen"],["dc.contributor.author","Brockmann, Knut"],["dc.contributor.author","Gärtner, Jutta"],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Hanefeld, Folker A."],["dc.date.accessioned","2017-09-07T11:48:46Z"],["dc.date.available","2017-09-07T11:48:46Z"],["dc.date.issued","2008"],["dc.description.abstract","The neuropathology of vanishing white matter (VWM) disease is characterized by a loss of white matter (WM). Although recent histopathological studies suggest a primary glial dysfunction, the purpose of this work was to assess the extent of axonal involvement in VWM using long-term follow-up proton MR spectroscopy. White and gray matter of nine children with genetically proven VWM and late infancy/early childhood onset were investigated with short-echo time, single-voxel proton MR spectroscopy over up to 8 years starting as early as less than 2 years after the onset of symptoms (5 patients). Total N-acetyl-aspartate (-51% from normal control), creatine and phosphocreatine (-47%), and myo-inositol (-49%) were reduced in WM at early disease stages. Choline-containing compounds were less severely decreased (-31%). Follow-up investigations revealed progressive reduction of all metabolites in WM. In gray matter, no distinct changes were detected at early stages. Later total N-acetyl-aspartate decreased slightly (-22%). Assuming the metabolite alterations to primarily reflect changes in cellular composition, the observed pattern indicates early axonal involvement or loss as well as relatively enhanced turnover of myelin. These early stages are followed by a complete cellular loss in cerebral WM."],["dc.identifier.doi","10.1203/01.pdr.0000304934.90198.25"],["dc.identifier.gro","3143328"],["dc.identifier.isi","000254374300021"],["dc.identifier.pmid","18356755"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/830"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.relation.issn","0031-3998"],["dc.title","Early reduction of total N-acetyl-aspartate-compounds in patients with classical vanishing white matter disease. A long-term follow-up MRS study"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","1049"],["dc.bibliographiccitation.issue","7"],["dc.bibliographiccitation.journal","Journal of Neurology"],["dc.bibliographiccitation.lastpage","1058"],["dc.bibliographiccitation.volume","255"],["dc.contributor.author","Brockmann, Knut"],["dc.contributor.author","Dreha-Kulaczewski, Steffi F."],["dc.contributor.author","Dechent, Peter"],["dc.contributor.author","Boennemann, Carsten"],["dc.contributor.author","Helms, Gunther"],["dc.contributor.author","Kyllerman, Marten"],["dc.contributor.author","Brueck, Wolfgang"],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Huehne, Kathrin"],["dc.contributor.author","Gärtner, Jutta"],["dc.contributor.author","Rautenstrauss, Bernd"],["dc.date.accessioned","2017-09-07T11:48:16Z"],["dc.date.available","2017-09-07T11:48:16Z"],["dc.date.issued","2008"],["dc.description.abstract","Mutations in the mitofusin 2 (MFN2) gene are a major cause of primary axonal Charcot-Marie-Tooth (CMT) neuropathy. This study aims at further characterization of cerebral white matter alterations observed in patients with MFN2 mutations. Molecular genetic, magnetic resonance imaging (MRI), magnetic resonance spectroscopy (MRS), and diffusion tensor imaging (DTI) investigations were performed in four unrelated patients aged 7 to 38 years with early onset axonal CMT neuropathy. Three distinct and so far undescribed MFN2 mutations were detected. Two patients had secondary macrocephaly and mild diffuse predominantly periventricular white matter alterations on MRI. In addition, one boy had symmetrical T2-hyperintensities in both thalami. Two patients had optic atrophy, one of them with normal MRI. In three patients proton MRS revealed elevated concentrations of total N-acetyl compounds (neuronal marker), total creatine (found in all cells) and myo-inositol (astrocytic marker) in cerebral white and gray matter though with regional variation. These alterations were most pronounced in the two patients with abnormal MRI. DTI of these patients revealed mild reductions of fractional anisotropy and mild increase of mean diffusivity in white matter. The present findings indicate an enhanced cellular density in cerebral white matter of MFN2 neuropathy which is primarily due to a reactive gliosis without axonal damage and possibly accompanied by mild demyelination."],["dc.identifier.doi","10.1007/s00415-008-0847-1"],["dc.identifier.gro","3143273"],["dc.identifier.isi","000258025000016"],["dc.identifier.pmid","18425620"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/769"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.publisher","Springer"],["dc.publisher.place","Heidelberg"],["dc.relation.issn","0340-5354"],["dc.title","Cerebral involvement in axonal Charcot-Marie-Tooth neuropathy caused by mitofusin2 mutations"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","38"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Annals of Neurology"],["dc.bibliographiccitation.lastpage","46"],["dc.bibliographiccitation.volume","52"],["dc.contributor.author","Brockmann, Knut"],["dc.contributor.author","Bjornstad, A."],["dc.contributor.author","Dechent, Peter"],["dc.contributor.author","Korenke, C. G."],["dc.contributor.author","Smeitink, J."],["dc.contributor.author","Trijbels, JMF"],["dc.contributor.author","Athanassopoulos, S."],["dc.contributor.author","Villagran, R."],["dc.contributor.author","Skjeldal, O. H."],["dc.contributor.author","Wilichowski, E."],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Hanefeld, Folker"],["dc.date.accessioned","2018-11-07T10:19:26Z"],["dc.date.available","2018-11-07T10:19:26Z"],["dc.date.issued","2002"],["dc.description.abstract","A deficiency of succinate dehydrogenase is a rare cause of mitochondrial encephalomyopathy. Three patients, 2 sisters and I boy from an unrelated family, presented with symptoms and magnetic resonance imaging signs of leukoencephalopathy. Localized proton magnetic resonance spectroscopy indicated a prominent singlet at 2.40ppm in cerebral and cerebellar white matter not present in gray matter or basal ganglia. The signal was also elevated in cerebrospinal fluid and could be identified as originating from the two equivalent methylene groups of succinate. Subsequently, an isolated deficiency of complex II (succinate:ubiquinone oxidoreductase) was demonstrated in 2 patients in muscle and fibroblasts. One of the sisters died at the age of 18 months. Postmortem examination showed the neuropathological characteristics of Leigh syndrome. Her younger sister, now 12 months old, is also severely affected; the boy, now 6 years old, follows a Milder, fluctuating clinical course. Magnetic resonance spectroscopy provides a characteristic pattern in succinate dehydrogenase deficiency."],["dc.identifier.doi","10.1002/ana.10232"],["dc.identifier.isi","000176451800007"],["dc.identifier.pmid","12112045"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/41656"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Wiley-liss"],["dc.relation.issn","0364-5134"],["dc.title","Succinate in dystrophic white matter: A proton magnetic resonance spectroscopy finding characteristic for complex II deficiency"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","45"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Brain and Development"],["dc.bibliographiccitation.lastpage","50"],["dc.bibliographiccitation.volume","25"],["dc.contributor.author","Brockmann, Knut"],["dc.contributor.author","Pouwels, Petra J.W."],["dc.contributor.author","Dechent, Peter"],["dc.contributor.author","Flanigan, Kevin M."],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Hanefeld, Folker"],["dc.date.accessioned","2021-06-01T10:50:17Z"],["dc.date.available","2021-06-01T10:50:17Z"],["dc.date.issued","2003"],["dc.description.abstract","Magnetic resonance imaging of a girl with giant axonal neuropathy revealed a progressive white matter disease. In close agreement with histopathological features reported previously, localized proton magnetic resonance spectroscopy at 9 and 12 years of age indicated a specific damage or loss of axons (reduced N-acetylaspartate and N-acetylaspartylglutamate) accompanied by acute demyelination (elevated choline-containing compounds, myo-inositol, and lactate) in white matter as well as a generalized proliferation of glial cells (elevated choline-containing compounds and myo-inositol) in both gray and white matter. (C) 2002 Elsevier Science B.V. All rights reserved."],["dc.identifier.doi","10.1016/s0387-7604(02)00154-7"],["dc.identifier.isi","000180767900008"],["dc.identifier.pmid","12536033"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/86597"],["dc.language.iso","en"],["dc.notes.intern","DOI-Import GROB-425"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.relation.issn","0387-7604"],["dc.title","Cerebral proton magnetic resonance spectroscopy of a patient with giant axonal neuropathy"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","39"],["dc.bibliographiccitation.journal","European Journal of Paediatric Neurology"],["dc.bibliographiccitation.lastpage","39"],["dc.bibliographiccitation.volume","11"],["dc.contributor.author","Dreha-Kulaczewski, S."],["dc.contributor.author","Dechent, P."],["dc.contributor.author","Helms, G."],["dc.contributor.author","Frahm, J."],["dc.contributor.author","Gaertner, J."],["dc.contributor.author","Brockmann, K."],["dc.date.accessioned","2018-04-23T11:47:36Z"],["dc.date.available","2018-04-23T11:47:36Z"],["dc.date.issued","2007"],["dc.identifier.doi","10.1016/s1090-3798(08)70391-x"],["dc.identifier.gro","3142236"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/13360"],["dc.language.iso","en"],["dc.notes.intern","lifescience updates Crossref Import"],["dc.notes.status","final"],["dc.relation.issn","1090-3798"],["dc.title","DO06 Complete recovery of NAA reduction in white matter disorders demonstrated by serial proton MRS"],["dc.type","journal_article"],["dc.type.internalPublication","unknown"],["dc.type.peerReviewed","no"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","819"],["dc.bibliographiccitation.issue","5"],["dc.bibliographiccitation.journal","Neurology"],["dc.bibliographiccitation.lastpage","825"],["dc.bibliographiccitation.volume","60"],["dc.contributor.author","Brockmann, K."],["dc.contributor.author","Dechent, P."],["dc.contributor.author","Wilken, B."],["dc.contributor.author","Rusch, O."],["dc.contributor.author","Frahm, J."],["dc.contributor.author","Hanefeld, F."],["dc.date.accessioned","2021-06-01T10:48:09Z"],["dc.date.available","2021-06-01T10:48:09Z"],["dc.date.issued","2003"],["dc.description.abstract","Background: Krabbe disease (globoid cell leukodystrophy [GLD]) is an autosomal recessive lysosomal disorder affecting the central and peripheral nervous system. The authors performed MRS to characterize metabolic alterations and their regional variation in brain tissue in GLD in vivo. Methods: Abnormalities of cerebral metabolite concentrations were assessed in seven patients with biochemically proven GLD-four with infantile, two with juvenile, and one with adult subtype-using quantitative localized proton MRS of standardized brain regions. Results: In infantile GLD, pronounced elevation of both myo-inositol and choline-containing compounds in affected white matter reflected demyelination and glial proliferation. The accompanying decrease of N-acetylaspartate pointed to neuroaxonal loss. Gray matter showed similar, albeit much milder alterations. In juvenile GLD, MRS indicated astrocytosis with minor neuroaxonal damage in white matter. In a patient with adult GLD, results of MRS of affected white matter were close to normal. MRS data are in agreement with histopathologic features of GLD. Conclusions: Proton MRS provides a powerful tool for assessing metabolic disturbances and the extent of brain damage noninvasively in GLD."],["dc.identifier.doi","10.1212/01.WNL.0000049469.29011.E9"],["dc.identifier.isi","000182948300016"],["dc.identifier.pmid","12629240"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/85843"],["dc.language.iso","en"],["dc.notes.intern","DOI-Import GROB-425"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Lippincott Williams & Wilkins"],["dc.relation.eissn","1526-632X"],["dc.relation.issn","0028-3878"],["dc.title","Proton MRS profile of cerebral metabolic abnormalities in Krabbe disease"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","267"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Neuroradiology"],["dc.bibliographiccitation.lastpage","271"],["dc.bibliographiccitation.volume","46"],["dc.contributor.author","Brockmann, Knut"],["dc.contributor.author","Finsterbusch, Jürgen"],["dc.contributor.author","Schara, Ulrike"],["dc.contributor.author","Wilichowski, Ekkehard"],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Hanefeld, Folker"],["dc.date.accessioned","2018-11-07T10:50:03Z"],["dc.date.available","2018-11-07T10:50:03Z"],["dc.date.issued","2004"],["dc.description.abstract","In a 13-month-old boy with recurrent motor deterioration provoked by fever MRI and proton MRS detected a leukoencephalopathy with reduced cerebral metabolites and elevated lactate. At follow-up 6 and 16 months later these abnormalities improved gradually. Serial diffusion tensor imaging revealed a stroke-like pattern with an initial strong reduction of the apparent diffusion coefficient followed by elevated values 6 months later. The relative diffusion anisotropy remained reduced. Muscle biopsy confirmed a mitochondrial encephalomyopathy."],["dc.identifier.doi","10.1007/s00234-004-1181-7"],["dc.identifier.isi","000220861300003"],["dc.identifier.pmid","15034698"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/48573"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.relation.issn","0028-3940"],["dc.title","Stroke-like pattern in DTI and MRS of childhood mitochondrial leukoencephalopathy"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","35"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Pediatric Neurology"],["dc.bibliographiccitation.lastpage","40"],["dc.bibliographiccitation.volume","34"],["dc.contributor.author","Groeschel, S."],["dc.contributor.author","Brockmann, Knut"],["dc.contributor.author","Dechent, Peter"],["dc.contributor.author","Wilichowski, E."],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Hanefeld, Folker"],["dc.date.accessioned","2018-11-07T10:40:45Z"],["dc.date.available","2018-11-07T10:40:45Z"],["dc.date.issued","2006"],["dc.description.abstract","Megalencephaly with dilated Virchow-Robin spaces has been suggested to represent a new clinical entity. This report describes two males and a female who have been monitored from pregnancy. The patients manifest a relatively normal psychomotor development with some minor neurologic symptoms such as mild muscle hypotonia and clumsy motor performance. Biochemical and electrophysiologic tests were normal. In the white matter of the brain, a prominent dilatation of the Virchow-Robin spaces with some adjacent signal alterations could be demonstrated by magnetic resonance imaging. Magnetic resonance spectroscopy revealed normal metabolite concentrations in the cortical and deep gray matter and normal-appearing white matter. Affected white matter was characterized by mildly reduced to normal levels of myo-inositol and a decrease of all other metabolites including total N-acetyl moieties, choline-containing compounds, and total creatine. These data indicate that the dilatation of Virchow-Robin spaces reflects an underlying brain pathology causing neuroaxonal damage. Possible differential diagnoses are discussed. (c) 2006 by Elsevier Inc. All rights reserved."],["dc.identifier.doi","10.1016/j.pediatrneurol.2005.05.012"],["dc.identifier.isi","000234660600007"],["dc.identifier.pmid","16376276"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/46380"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Elsevier Science Inc"],["dc.relation.issn","0887-8994"],["dc.title","Magnetic resonance imaging and proton magnetic resonance spectroscopy of megalencephaly and dilated Virchow-Robin spaces"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]