Krasnianski, Anna

[["dc.bibliographiccitation.firstpage","2288"],["dc.bibliographiccitation.journal","Brain"],["dc.bibliographiccitation.lastpage","2296"],["dc.bibliographiccitation.volume","129"],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Kallenberg, Kai"],["dc.contributor.author","Meissner, Bettina"],["dc.contributor.author","Collie, Donald A."],["dc.contributor.author","Roeber, Sigrun"],["dc.contributor.author","Bartl, Mario"],["dc.contributor.author","Heinemann, Uta"],["dc.contributor.author","Varges, Daniel. A."],["dc.contributor.author","Kretzschmar, Hans A."],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2018-11-07T09:17:31Z"],["dc.date.available","2018-11-07T09:17:31Z"],["dc.date.issued","2006"],["dc.description.abstract","A typical clinical course and low sensitivity of established diagnostic tests are the main diagnostic problems in the MV2 subtype of sporadic Creutzfeldt-Jakob disease (sCJD). Clinical symptoms and signs, MRI, EEG and biochemical CSF markers were studied in 26 patients. Histological findings were semiquantitatively evaluated. Compared with typical sCJD, the disease duration was prolonged (median 12 months). Dementia, ataxia and psychiatric symptoms were present in all patients. Extrapyramidal signs were observed in 88%. T2-weighted MRI showed basal ganglia hyperintensities in 90%. Increased thalamic signal intensity was detected in 88% on diffusion-weighted MRI. Increased CSF tau-protein was found in 83%, and the 14-3-3 test was positive in 76%. The EEG revealed periodic sharp wave complexes in only two patients. Kuru plaques, severe thalamic and basal ganglia gliosis and spongiform changes, and neuronal loss in the pulvinar were the prominent histological features. At least one of the three diagnostic tests (MRI, tau- and 14-3-3 protein) supported the clinical diagnosis in all patients. MRI was the most sensitive of the diagnostic tests applied. Thalamic hyperintensities were observed unusually frequently. Prolonged disease duration, early and prominent psychiatric symptoms, absence of typical EEG, thalamic hyperintensities on MRI and relatively low 14-3-3 protein sensitivity may be suspicious for variant CJD. However, distinct sensory symptoms and young age at onset, which are often found in the latter, are not common in the MV2 subtype, and the pulvinar sign was observed in only one case."],["dc.identifier.doi","10.1093/brain/awl123"],["dc.identifier.isi","000240679700006"],["dc.identifier.pmid","16720682"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/28191"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Oxford Univ Press"],["dc.relation.issn","0006-8950"],["dc.title","Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","1544"],["dc.bibliographiccitation.issue","10"],["dc.bibliographiccitation.journal","Neurology"],["dc.bibliographiccitation.lastpage","1550"],["dc.bibliographiccitation.volume","65"],["dc.contributor.author","Meissner, Bettina"],["dc.contributor.author","Westner, I. M."],["dc.contributor.author","Kallenberg, Kai"],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Bartl, Mario"],["dc.contributor.author","Varges, Daniel. A."],["dc.contributor.author","Bosenberg, C."],["dc.contributor.author","Kretzschmar, Hans A."],["dc.contributor.author","Knauth, Michael"],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Zerr, I."],["dc.date.accessioned","2018-11-07T10:54:10Z"],["dc.date.available","2018-11-07T10:54:10Z"],["dc.date.issued","2005"],["dc.description.abstract","Background: Recently, six molecular subtypes of sporadic CJD ( sCJD) have been identified showing differences regarding the disease course, neuropathologic lesion patterns, and sensitivity to diagnostic tools. Only isolated cases of the rare VV1 type have been reported so far. Objective: To describe the clinical characteristics and neuropathologic lesion profiles in nine cases. Methods: In the years 1993 until late 2003, 571 definite neuropathologically confirmed cases of sporadic CJD were identified in Germany. Of these, nine were homozygous for valine and displayed type 1 of the pathologic PrPSc in the brain ( VV1 type). Results: The authors describe eight men and one woman belonging to the VV1 type. All patients were relatively young at disease onset ( median 44 years vs 65 years in all sCJD) with prolonged disease duration ( median 21 months vs 6 months in all sCJD). During the initial stages, their main clinical signs were personality changes and slowly progressive dementia as well as focal neurologic deficits. None of the nine VV1 patients had periodic sharp- wave complexes ( PSWCs) in the EEG. Only two out of seven displayed the typical signal increase of the basal ganglia on MRI, whereas signal increase of the cortex was seen in all patients. The 14- 3- 3 protein levels were elevated in CSF in all cases tested. Conclusions: The clinical diagnosis of the VV1 type of sCJD can be best supported by the 14- 3- 3 test and cortical signal increase on MRI. Because of the young age at onset vCJD is sometimes suspected as a differential diagnosis. MRI plays an important role in differentiating these two disease types and should be performed early during the disease course."],["dc.identifier.doi","10.1212/01.wnl.0000184674.32924.c9"],["dc.identifier.isi","000233428100008"],["dc.identifier.pmid","16221949"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/49508"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Lippincott Williams & Wilkins"],["dc.relation.issn","0028-3878"],["dc.title","Sporadic Creutzfeldt-Jakob disease - Clinical and diagnostic characteristics of the rare VV1 type"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","863"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Journal of Alzheimer s Disease"],["dc.bibliographiccitation.lastpage","873"],["dc.bibliographiccitation.volume","17"],["dc.contributor.author","Meyne, Felix"],["dc.contributor.author","Gloeckner, Sara Friederike"],["dc.contributor.author","Ciesielczyk, Barbara"],["dc.contributor.author","Heinemann, Uta"],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Meissner, Bettina"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2018-11-07T08:34:47Z"],["dc.date.available","2018-11-07T08:34:47Z"],["dc.date.issued","2009"],["dc.description.abstract","We performed a study on levels of the total prion protein (PrP) in humans affected by different neurological diseases and assessed the influence of several factors such as age, gender, and disease severity on the cerebrospinal fluid PrP levels. PrP-ELISA technique was used to analyze cerebrospinal fluid (CSF) samples. 293 CSF samples of patients with Creutzfeldt-Jakob disease (CJD), Alzheimer's disease, dementia with Lewy-bodies, Parkinson's disease, multiple sclerosis, cerebral ischemia, generalized epileptic seizures, and meningitis and encephalitis in comparison to controls were analyzed. We found a significant reduction of CSF PrP levels in patients suffering from all neurodegenerative disorders analyzed. This group exhibited mean PrP values of 164 ng/ml while non-neurodegenerative disorder patients and healthy controls showed PrP levels of 208 ng/ml and 226 ng/ml, respectively. CSF levels correlated with disease severity in CJD, Alzheimer's disease, and dementia with Lewy- bodies. The finding of decreased PrP levels in the CSF of patients not only with CJD but also in other neurodegenerative disorders is intriguing. Age-, gender-, and genetic-specific factors might be involved in the PrPc regulation."],["dc.identifier.doi","10.3233/JAD-2009-1110"],["dc.identifier.isi","000269629400014"],["dc.identifier.pmid","19542614"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/17899"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Ios Press"],["dc.relation.issn","1387-2877"],["dc.title","Total Prion Protein Levels in the Cerebrospinal Fluid are Reduced in Patients with Various Neurological Disorders"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","1126"],["dc.bibliographiccitation.issue","6"],["dc.bibliographiccitation.journal","European Journal of Neurology"],["dc.bibliographiccitation.lastpage","1133"],["dc.bibliographiccitation.volume","23"],["dc.contributor.author","Gmitterova, Karin"],["dc.contributor.author","Heinemann, U."],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Gawinecka, Joanna"],["dc.contributor.author","Zerr, I."],["dc.date.accessioned","2018-11-07T10:13:56Z"],["dc.date.available","2018-11-07T10:13:56Z"],["dc.date.issued","2016"],["dc.description.abstract","Background and purpose: Cerebrospinal fluid (CSF) analysis supports the clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) when applied within an adequate clinical context. A diagnostic potential has been attributed to CSF proteins such as 14-3-3, but also tau protein, phosphorylated tau (181P) (p-tau) protein, amyloid beta(1-42), S100B and neuron-specific enolase (NSE). There has been only limited information available about the contribution of CSF analysis in the differentiation of various molecular sCJD subtypes. Methods: The CSF levels of the aforementioned proteins from 73 sCJD patients with distinct molecular subtypes were determined. Results: Differences in tau values were significant amongst the homozygous patients (MM and VV genotype) compared to the heterozygous group (P = 0.07 and P = 0.02 respectively). Significantly higher CSF tau levels (P = 0.003) and NSE (P = 0.02) but lower p-tau/tau ratio (P = 0.01) were observed in MM1 compared to MM2 patients. The p-tau/tau ratio enabled the differentiation of MV genotype with higher levels in PrPsc type 2 (P = 0.04). Elevation of S100B (P < 0.001) and NSE (P = 0.03) was observed in VV2 compared to VV1 subtype. PRNP codon 129 genotype, PrPsc isotype, disease duration and clinical stage influenced the test sensitivity in all proteins. Conclusions: Cerebrospinal fluid protein levels might be useful in the pre-mortem differentiation of molecular sCJD subtypes when the codon 129 genotype is known."],["dc.identifier.doi","10.1111/ene.12991"],["dc.identifier.isi","000375765000021"],["dc.identifier.pmid","27029507"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/40525"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Wiley-blackwell"],["dc.relation.issn","1468-1331"],["dc.relation.issn","1351-5101"],["dc.title","Cerebrospinal fluid markers in the differentiation of molecular subtypes of sporadic Creutzfeldt-Jakob disease"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","150"],["dc.bibliographiccitation.issue","2"],["dc.bibliographiccitation.journal","The Lancet Neurology"],["dc.bibliographiccitation.lastpage","158"],["dc.bibliographiccitation.volume","13"],["dc.contributor.author","Haik, Stephane"],["dc.contributor.author","Marcon, Gabriella"],["dc.contributor.author","Mallet, Alain"],["dc.contributor.author","Tettamanti, Mauro"],["dc.contributor.author","Welaratne, Arlette"],["dc.contributor.author","Giaccone, Giorgio"],["dc.contributor.author","Azimi, Shohreh"],["dc.contributor.author","Pietrini, Vladimir"],["dc.contributor.author","Fabreguettes, Jean-Roch"],["dc.contributor.author","Imperiale, Daniele"],["dc.contributor.author","Cesaro, Pierre"],["dc.contributor.author","Buffa, Carlo"],["dc.contributor.author","Aucan, Christophe"],["dc.contributor.author","Lucca, Ugo"],["dc.contributor.author","Peckeu, Laurene"],["dc.contributor.author","Suardi, Silvia"],["dc.contributor.author","Tranchant, Christine"],["dc.contributor.author","Zerr, Ingo"],["dc.contributor.author","Houillier, Caroline"],["dc.contributor.author","Redaelli, Veronica"],["dc.contributor.author","Vespignani, Herve"],["dc.contributor.author","Campanella, Angela"],["dc.contributor.author","Sellal, Francois"],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Seilhean, Danielle"],["dc.contributor.author","Heinemann, Uta"],["dc.contributor.author","Sedel, Frederic"],["dc.contributor.author","Canovi, Mara"],["dc.contributor.author","Gobbi, Marco"],["dc.contributor.author","Di Fede, Giuseppe"],["dc.contributor.author","Laplanche, Jean-Louis"],["dc.contributor.author","Pocchiari, Maurizio"],["dc.contributor.author","Salmona, Mario"],["dc.contributor.author","Forloni, Gianluigi"],["dc.contributor.author","Brandel, Jean-Philippe"],["dc.contributor.author","Tagliavini, Fabrizio"],["dc.date.accessioned","2018-11-07T09:44:35Z"],["dc.date.available","2018-11-07T09:44:35Z"],["dc.date.issued","2014"],["dc.description.abstract","Background Creutzfeldt-Jakob disease (CJD) is a fatal, untreatable prion encephalopathy. Previous studies showed that doxycycline is effective in in-vitro and in-vivo models of disease, and patients with CJD who received compassionate treatment with doxycycline showed increased survival time compared with historical series. We therefore did a randomised, double-blind study of doxycycline versus placebo in CJD. Methods We recruited patients older than 18 years old who had a diagnosis of definite or probable sporadic CJD or genetic forms of the disease via Italian reference centres and the French national referral system. Patients were randomly assigned (ratio 1:1) to receive oral doxycydine (100 mg daily) or placebo under double-blind conditions from the day of randomisation to death. Centralised randomisation was done independently of enrolment or evaluation of patients using a minimisation method in Italy and a simple randomisation in France. Participants, caregivers, and clinicians were masked to group assignment. The primary efficacy variable was the survival time from randomisation. Interim analyses were planned to detect a significant effect of treatment as early as possible. This trial is registered with EudraCT, 2006-001858-27 for the Italian study and 2007-005553-34 for the French study. Findings From April 12, 2007, to Aug 19, 2010, in Italy, and from Jan 30, 2009, to Jan 10, 2012, in France, 121 patients with CID were enrolled in the study, 62 of whom were randomly assigned to the treatment group and 59 to the placebo group. The first interim analysis showed absence of superiority of doxycycline compared with placebo, and the trial was stopped for futility. Efficacy analyses did not show significant differences between patients treated with doxycycline and placebo with regard to survival times (HR 1.1, 95% CI 0.8-1. 7, p=0.50). Serious adverse events were judged not to be related to treatment, whereas a relation was deemed probable or possible for five non-serious adverse events that occurred in each treatment group. Interpretation Doxycycline at a dose of 100 mg per day was well tolerated but did not significantly affect the course of CJD, at variance with the results of previous observational studies. Our experience could be useful in the design of large multinational controlled trials of potential anti-prion molecules in this rare disease."],["dc.identifier.doi","10.1016/S1474-4422(13)70307-7"],["dc.identifier.isi","000330546200010"],["dc.identifier.pmid","24411709"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/34428"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Elsevier Science Inc"],["dc.relation.issn","1474-4465"],["dc.relation.issn","1474-4422"],["dc.title","Doxycycline in Creutzfeldt-Jakob disease: a phase 2, randomised, double-blind, placebo-controlled trial"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","735"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Journal of Neurosurgery"],["dc.bibliographiccitation.lastpage","741"],["dc.bibliographiccitation.volume","109"],["dc.contributor.author","Heinemann, Uta"],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Meissner, Bettina"],["dc.contributor.author","Kallenberg, Kai"],["dc.contributor.author","Kretzschmar, Hans A."],["dc.contributor.author","Schulz-schaeffer, Walter"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2018-11-07T11:10:39Z"],["dc.date.available","2018-11-07T11:10:39Z"],["dc.date.issued","2008"],["dc.description.abstract","Object. Creultzfeldt-Jakob disease (CJD) is a rare neurodegenerative disorder with diagnostic criteria defined as a combination of clinical symptoms, electroencephalography findings, cerebrospinal fluid (CSF) analysis, and MR imaging results. Special subtypes are known to present with,in atypical course and test findings that can complicate the clinical diagnosis. In such patients a brain biopsy can support the clinical approach. Methods. The authors studied the records on 26 brain biopsies conducted in patients with Suspected CID who had been referred to the CJD Surveillance Unit in Germany between 1993 and 2005. Results. Of the 26 included patients, 11 suffered front neuropathologically confirmed CJD. which in 5 cases had been deemed clinically \"probable\" and in 2 had been deemed \"possible.\" The disease in the remaining 4 patients had been categorized as \"other\" prior to neuropathological continuation of CJD. The results of 15 brain biopsies showed no features of prion disease. None of these 15 patients had received a probable diagnosis of CID, 4 had a possible diagnosis. and 11 had received a diagnosis of \"other.\" Three of the cases classified as other and none of those with CJD presented with pleocytosis in the CSF in 73% of the other cases, biopsy sampling did not reveal any results characteristic of CJD but did not provide specific findings on which to base a differential diagnosis. Autopsy confirmed the biopsy diagnosis of CJD in all cases, and additionally confirmed that CJD was not present in 3 patients who had nondiagnostic biopsy results. Conclusions. Biopsy sampling nay be helpful in the diagnostic approach to rare cases of dementia for which a reliable diagnosis cannot be established on the basis of clinical symptoms, CSF parameters, electroencephalography. and MR imaging results."],["dc.description.sponsorship","Federal Ministry of Health [1369-341]"],["dc.identifier.doi","10.3171/JNS/2008/109/10/0735"],["dc.identifier.isi","000259549100021"],["dc.identifier.pmid","18826363"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/53255"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Amer Assoc Neurological Surgeons"],["dc.relation.issn","0022-3085"],["dc.title","Brain biopsy in patients with suspected Creutzfeldt-Jakob disease"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","762"],["dc.bibliographiccitation.issue","8"],["dc.bibliographiccitation.journal","European Journal of Neurology"],["dc.bibliographiccitation.lastpage","771"],["dc.bibliographiccitation.volume","15"],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Kallenberg, Kai"],["dc.contributor.author","Collie, Donald A."],["dc.contributor.author","Meissner, Bettina"],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Heinemann, U."],["dc.contributor.author","Varges, Daniel. A."],["dc.contributor.author","Summers, D. M."],["dc.contributor.author","Kretzschmar, Hans A."],["dc.contributor.author","Talbot, T."],["dc.contributor.author","Will, Robert G."],["dc.contributor.author","Zerr, I."],["dc.date.accessioned","2018-11-07T11:12:23Z"],["dc.date.available","2018-11-07T11:12:23Z"],["dc.date.issued","2008"],["dc.description.abstract","Background and purpose: To establish radiological features in the atypical MV2 subtype of sCJD compared with the classical MM1 subtype, as well as region- and sequence-dependent inter-observer correlation. Methods: MRI hyperintensity of basal ganglia (BG), cortex and thalamus was evaluated in 31 MM1 and 32 MV2 patients. Each MR scan was analyzed independently by two neuroradiologists blinded to PRNP genotype/prion protein type. Results: Cumulative T2-sensitivity for BG hyperintensity was higher in the MV2 subtype (84% for both observers versus 61% in observer 1/42% in observer 2 in MM1 patients). Significant inter-observer agreement was found for BG and thalamus on T2, FLAIR, PD and DWI, but for cortex only on DWI. Thalamic changes were significantly more frequent in MV2 than in MM1 patients (cumulative sensitivity 86% vs. 12.5% on DWI). Discussion: The high frequency of thalamic hyperintensity in the MV2 subtype allowed differentiation from MM1 patients. Good inter-observer agreement was found for BG and thalamus in all sequences. DWI showed the highest inter-observer correlation independent of the investigated brain region and was therefore not only highly sensitive but also relatively independent of investigator bias. Since inter-observer correlation for cortical hyperintensity in T2, FLAIR and PD is relatively low, the cortical changes should not be over-interpreted with these sequences."],["dc.description.sponsorship","Department of Health"],["dc.identifier.doi","10.1111/j.1468-1331.2008.02209.x"],["dc.identifier.isi","000257715400013"],["dc.identifier.pmid","18684308"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/53654"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Wiley-blackwell"],["dc.relation.issn","1468-1331"],["dc.relation.issn","1351-5101"],["dc.title","MRI in the classical MM1 and the atypical MV2 subtypes of sporadic CJD: an inter-observer agreement study"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.journal","International Journal of Medical Microbiology"],["dc.bibliographiccitation.volume","298"],["dc.contributor.author","Fincke, Fabian"],["dc.contributor.author","Achak, H."],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Heinemann, U."],["dc.contributor.author","Zerr, I."],["dc.date.accessioned","2018-11-07T11:11:33Z"],["dc.date.available","2018-11-07T11:11:33Z"],["dc.date.issued","2008"],["dc.format.extent","104"],["dc.identifier.isi","000259589400418"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/53462"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Elsevier Gmbh, Urban & Fischer Verlag"],["dc.publisher.place","Jena"],["dc.relation.conference","60th Annual Meeting of the Deutschen-Gesellschaft-fur-Hygiene-und-Mikrobiologie"],["dc.relation.eventlocation","Dresden, GERMANY"],["dc.relation.issn","1438-4221"],["dc.title","Clinical trial of doxycycline as a therapeutic approach in Creutzfeldt-Jakob disease"],["dc.type","conference_abstract"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","533"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Annals of Neurology"],["dc.bibliographiccitation.lastpage","543"],["dc.bibliographiccitation.volume","58"],["dc.contributor.author","Boesenberg, C."],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Meissner, Bettina"],["dc.contributor.author","Kallenberg, Kai"],["dc.contributor.author","Bartl, Mario"],["dc.contributor.author","Heinemann, U."],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Stoeck, Katharina"],["dc.contributor.author","Varges, Daniel. A."],["dc.contributor.author","Windl, Otto"],["dc.contributor.author","Kretzschmar, Hans A."],["dc.contributor.author","Zerr, I."],["dc.date.accessioned","2018-11-07T10:55:04Z"],["dc.date.available","2018-11-07T10:55:04Z"],["dc.date.issued","2005"],["dc.description.abstract","Sporadic Creutzfeldt-jakob disease (sCJD) is a rare neurodegenerative disease with the greatest incidence occurring in patients between 60 and 70 years old. Younger patients may also be affected. In this study, we used all case material available from 52 patients with sCJD aged 50 years of younger at disease onset, who were identified between 1993 and 2003 in Germany. The objective of this study was to describe the psychiatric and neurological features of these young patients with emphasis on the different codon 129 genotypes and PrP types, and to compare them with elder patients with sCJD and patients with variant CJD. We also gave particular attention to electroencephalogram, magnetic resonance imaging, and 14-3-3 results, as well as to the neuropathological lesion profile. The clinical syndrome in young patients differs from elder patients with CJD with respect to clinical signs, disease duration, technical investigations, and neuropathological lesion profile. The psychiatric symptoms in young patients with sCJD are similar to the psychiatric symptoms expressed by patients with variant CJD; however, in contrast with the variant cases, young patients with sCJD experience development of prominent dementia early in the disease course."],["dc.identifier.doi","10.1002/ana.20568"],["dc.identifier.isi","000232309900006"],["dc.identifier.pmid","16037975"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/49703"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Wiley-blackwell"],["dc.relation.issn","1531-8249"],["dc.relation.issn","0364-5134"],["dc.title","Clinical course in young patients with sporadic Creutzfeldt-Jakob disease"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","119"],["dc.bibliographiccitation.issue","2"],["dc.bibliographiccitation.journal","Journal of Neurology Neurosurgery & Psychiatry"],["dc.bibliographiccitation.lastpage","125"],["dc.bibliographiccitation.volume","88"],["dc.contributor.author","Varges, Daniel. A."],["dc.contributor.author","Manthey, Henrike"],["dc.contributor.author","Heinemann, Uta"],["dc.contributor.author","Ponto, Claudia"],["dc.contributor.author","Schmitz, Matthias"],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Krasnianski, Anna"],["dc.contributor.author","Breithaupt, Maren"],["dc.contributor.author","Fincke, Fabian"],["dc.contributor.author","Kramer, Katharina"],["dc.contributor.author","Friede, Tim"],["dc.contributor.author","Zerr, Inga"],["dc.date.accessioned","2018-11-07T10:28:06Z"],["dc.date.available","2018-11-07T10:28:06Z"],["dc.date.issued","2017"],["dc.description.abstract","Objectives The main objective of the present study is to study the therapeutic efficiency of doxycycline in a double-blinded randomised phase II study in a cohort of patients with sporadic Creutzfeldt-Jakob disease (sCJD). Methods From the National Reference Center of TSE Surveillance in Germany, patients with probable or definite sCJD were recruited for a double-blinded randomised study with oral doxycycline (EudraCT 2006-003934-14). In addition, we analysed the data from patients with CJD who received compassionate treatment with doxycycline in a separate group. Potential factors which influence survival such as age at onset, gender, codon 129 polymorphism and cognitive functions were evaluated. The primary outcome measure was survival. Results Group 1: in the double-blinded randomised phase II study, 7 patients in the treatment group were compared with 5 controls. Group 2: 55 patients with sCJD treated with oral doxycycline were analysed and compared with 33 controls by a stratified propensity score applied to a Cox proportional hazard analysis. The results of both studies were combined by means of a random-effects meta-analysis. A slight increase in survival time in the doxycycline treatment group was observed (p=0.049, HR=0.63 (95% CI 0.402 to 0.999)). Conclusions On the basis of our studies, a larger trial of doxycycline should be performed in persons in the earliest stages of CJD."],["dc.identifier.doi","10.1136/jnnp-2016-313541"],["dc.identifier.isi","000393903700007"],["dc.identifier.pmid","27807198"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/43348"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","PUB_WoS_Import"],["dc.publisher","Bmj Publishing Group"],["dc.relation.issn","1468-330X"],["dc.relation.issn","0022-3050"],["dc.title","Doxycycline in early CJD: a double-blinded randomised phase II and observational study"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]