Now showing 1 - 4 of 4
  • 2002Journal Article
    [["dc.bibliographiccitation.firstpage","699"],["dc.bibliographiccitation.issue","6"],["dc.bibliographiccitation.journal","Journal of Neurology"],["dc.bibliographiccitation.lastpage","705"],["dc.bibliographiccitation.volume","249"],["dc.contributor.author","Henkel, K."],["dc.contributor.author","Zerr, I."],["dc.contributor.author","Hertel, A."],["dc.contributor.author","Gratz, K. F."],["dc.contributor.author","Schroter, A."],["dc.contributor.author","Tschampa, Henriette J."],["dc.contributor.author","Bihl, H."],["dc.contributor.author","Bull, U."],["dc.contributor.author","Grunwald, F."],["dc.contributor.author","Drzezga, A."],["dc.contributor.author","Spitz, J."],["dc.contributor.author","Poser, Sigrid"],["dc.date.accessioned","2018-11-07T10:29:01Z"],["dc.date.available","2018-11-07T10:29:01Z"],["dc.date.issued","2002"],["dc.description.abstract","The aim of this study was to explore the sites of metabolic changes with [F-18]2-fluoro-2-desoxy-D-glucose (FDG) and positron emission tomography (PET) in patients with Creutzfeldt-Jakob disease and to correlate the findings with clinical symptoms. Static [F-18]FDG-PET studies of eight patients with the diagnosis of confirmed or probable CJD were retrospectively analysed by two physicians from departments of nuclear medicine independently with a strong interrater agreement (kappa=0,98). The clinical data of the patients, based on a standardized evaluation by physicians from the German Creutzfeldt-Jakob disease surveillance study, was correlated with the PET findings. [F-18]FDG-PET shows widespread hypometabolism in CJD. All patients had a reduction of cerebral glucose metabolism in at least one temporal or parietal region. Additionally in 7 of our own 8 cases and 3 of 4 cases from the literature the occipital lobe, the cerebellum or the basal ganglia were involved. These findings differ from typical patterns of hypometabolism in Alzheimer's disease and other neurodegenerative disorders. In two thirds of the cases the distribution was markedly asymmetric. Myoclonus was present in five out of our eight own cases. Our data suggest that myoclonus might correlate with metabolic impairment of contralateral parietal and temporal lobes. In three of four patients with visual symptoms FDG uptake was reduced in the visual cortex bilaterally. Typical hyperintensities on MRI were only found in two of the eight cases at the time of PET-studies. Our results demonstrate that [F-18]FDG-PET appears to be a sensitive investigation in CJD and could be useful to differentiate CJD from other neuro degenerative disorders."],["dc.identifier.doi","10.1007/s00415-002-0695-3"],["dc.identifier.isi","000176407100008"],["dc.identifier.pmid","12111302"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/43553"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Dr Dietrich Steinkopff Verlag"],["dc.relation.issn","0340-5354"],["dc.title","Positron emission tomography with [F-18]FDG in the diagnosis of Creutzfeldt-Jakob disease (CJD)"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]
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  • 2000Journal Article
    [["dc.bibliographiccitation.firstpage","1751"],["dc.bibliographiccitation.issue","12"],["dc.bibliographiccitation.journal","ARCHIVES OF NEUROLOGY"],["dc.bibliographiccitation.lastpage","1757"],["dc.bibliographiccitation.volume","57"],["dc.contributor.author","Schroter, A."],["dc.contributor.author","Zerr, I."],["dc.contributor.author","Henkel, K."],["dc.contributor.author","Tschampa, Henriette J."],["dc.contributor.author","Finkenstaedt, M."],["dc.contributor.author","Poser, Sigrid"],["dc.date.accessioned","2018-11-07T09:23:41Z"],["dc.date.available","2018-11-07T09:23:41Z"],["dc.date.issued","2000"],["dc.description.abstract","Objective: To evaluate the diagnostic usefulness of magnetic resonance imaging (MRI) in the clinical diagnosis of Creutzfeldt-Jakob disease (CJD). Background: Creutzfeldt-Jakob disease is a rare neurodegenerative disease that belongs to the group of human spongiform encephalopathies and usually affects elderly people. It is clinically characterized by rapidly progressive dementia and development of neurological symptoms, such as myoclonus or ataxia. Until now, neuroradiologic investigations have only played a minor role in establishing the clinical diagnosis of CJD, and they are often performed to exclude differential diagnoses. Setting: A university hospital, base of the German National Creutzfeldt-Jakob Disease Surveillance Study. Methods and Patients: In this study, MRIs from suspected cases of CJD were examined by one investigator blinded to the diagnosis. Patients were classified according to the established clinical and neuropathological criteria. Results: Bilateral symmetric, high signal intensities on T2-weighted MRIs were present in the basal ganglia of 109 (67%) of 162 patients with CJD. In the control group, which consisted of non-CJD dementia patients, these abnormalities on T2-weighted MRIs were found in 4 (7%) of 58 patients. This corresponds to a high specificity in the differential diagnosis of CJD. Conclusion: These results indicate that MRI is a useful and valuable tool with reasonable sensitivity (67%) and high specificity (93%) and should be considered as an additional cornerstone in the clinical diagnosis of CJD."],["dc.identifier.doi","10.1001/archneur.57.12.1751"],["dc.identifier.isi","000165810400011"],["dc.identifier.pmid","11115241"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/29640"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Amer Medical Assoc"],["dc.relation.issn","0003-9942"],["dc.title","Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]
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  • 2001Journal Article
    [["dc.bibliographiccitation.firstpage","33"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Journal of Neurology Neurosurgery & Psychiatry"],["dc.bibliographiccitation.lastpage","39"],["dc.bibliographiccitation.volume","71"],["dc.contributor.author","Tschampa, Henriette J."],["dc.contributor.author","Neumann, M."],["dc.contributor.author","Zerr, I."],["dc.contributor.author","Henkel, K."],["dc.contributor.author","Schroter, A."],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Steinhoff, B. J."],["dc.contributor.author","Kretzschmar, Hans A."],["dc.contributor.author","Poser, Sigrid"],["dc.date.accessioned","2018-11-07T08:56:13Z"],["dc.date.available","2018-11-07T08:56:13Z"],["dc.date.issued","2001"],["dc.description.abstract","Objectives-To describe the clinical presentation of patients with Alzheimer's disease (AD) or dementia with Lewy bodies (DLB) who were suspected of having Creutzfeldt-Jakob disease (CJD) and to investigate whether current clinical diagnostic criteria cover these atypical forms of AD and DLB. Methods-Brains from necropsy were examined for the diagnosis of CJD at the German reference centre for spongiform encephalopathies. Symptoms and signs in patients with suspected CJD in whom necropsy showed AD (n=19) or DLB (n=12) were analysed. Their data were compared with a group of patients with CJD (n=25) to determine overlapping and discriminating clinical features. All patients were classified according to clinical diagnostic criteria for CJD, AD, and DLB. Results-Demented patients were suspected of having CJD if disease was rapidly progressing and/or focal neurological signs appeared and/or an EEG showed sharp wave complexes. Myoclonus and limb rigidity were the most common neurological signs in all three dementias. DLB was not suspected in any patient, although patients with DLB showed parkinsonism (58%) and fluctuations (58%). Periodic sharp wave complexes (PSWCs) in EEG typical of CJD were found in five patients with AD and one patient with DLB. 14-3-3 Protein in CSF was detected in 20 patients with CJD, in two patients with AD, but not in any patient with DLB. Although most patients with DLB or AD met the clinical criteria for their respective diagnosis (74% and 90%), they also fulfilled criteria for CJD (42% and 58%). Conclusions-in patients with rapidly progressive dementia and focal neurological signs, CJD should be the first line diagnosis. Facing the triad dementia, myoclonus, and rigidity, AD should be considered if the disease course is longer and DLB is the differential diagnosis if parkinsonism or fluctuations are present. Findings on EEG or CSF typical of CJD do not exclude AD or DLB."],["dc.identifier.doi","10.1136/jnnp.71.1.33"],["dc.identifier.isi","000169436500009"],["dc.identifier.pmid","11413259"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/23085"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","British Med Journal Publ Group"],["dc.relation.issn","0022-3050"],["dc.title","Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]
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  • 2000Journal Article
    [["dc.bibliographiccitation.firstpage","323"],["dc.bibliographiccitation.issue","3"],["dc.bibliographiccitation.journal","Annals of Neurology"],["dc.bibliographiccitation.lastpage","329"],["dc.bibliographiccitation.volume","48"],["dc.contributor.author","Zerr, I."],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Giese, A."],["dc.contributor.author","Bodemer, Monika"],["dc.contributor.author","Schroter, A."],["dc.contributor.author","Henkel, K."],["dc.contributor.author","Tschampa, Henriette J."],["dc.contributor.author","Windl, Otto"],["dc.contributor.author","Pfahlberg, Annette"],["dc.contributor.author","Steinhoff, B. J."],["dc.contributor.author","Gefeller, Olaf"],["dc.contributor.author","Kretzschmar, Hans A."],["dc.contributor.author","Poser, Sigrid"],["dc.date.accessioned","2018-11-07T10:23:19Z"],["dc.date.available","2018-11-07T10:23:19Z"],["dc.date.issued","2000"],["dc.description.abstract","According to the recently established molecular basis for phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD), six different phenotypes are characterized by the size of the protease-resistant fragment of the pathological prion protein (types 1 and 2) and homozygosity or heterozygosity for methionine or valine at codon 129 of the prion protein gene (designated by MM1, MM2, MV1, MV2, VV1, and VV2). In the present investigation, we analyzed the value of commonly used clinical tests (electroencephalogram [EEG], detection of 14-3-3 protein in cerebrospinal fluid [CSF], and hyperintensity of the basal ganglia in magnetic resonance imaging) for the clinical diagnosis in each CJD phenotype. The detection of periodic sharp and slow wave complexes in the EEG is reliable in the clinical diagnosis of MM1 and MV1 patients only. The CSF analysis for 14-3-3 protein showed high sensitivity in all analyzed subgroups with the exception of MV2 patients. Valine-homozygous patients had a negative EEG, but most had detectable levels of neuronal proteins in the CSF. The sensitivity of the magnetic resonance imaging was 70%, irrespective of the subgroup, but was particularly reliable in the clinical diagnosis of MV2 patients. The widening spectrum of diagnostic techniques in CJD is not only useful in the increased accuracy of the clinical diagnosis but should also lead to the identification of more atypical cases of sporadic CJD."],["dc.identifier.doi","10.1002/1531-8249(200009)48:3<323::AID-ANA6>3.0.CO;2-5"],["dc.identifier.isi","000089024600006"],["dc.identifier.pmid","10976638"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/42437"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Lippincott Williams & Wilkins"],["dc.relation.issn","0364-5134"],["dc.title","Current clinical diagnosis in Creutzfeldt-Jakob disease: Identification of uncommon variants"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]
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