Liman, Jan

[["dc.bibliographiccitation.artnumber","e1811"],["dc.bibliographiccitation.journal","Cell Death and Disease"],["dc.bibliographiccitation.volume","6"],["dc.contributor.author","Koch, J. C."],["dc.contributor.author","Bitow, F."],["dc.contributor.author","Haack, J."],["dc.contributor.author","D'Hedouville, Z."],["dc.contributor.author","Zhang, J-N"],["dc.contributor.author","Tönges, L."],["dc.contributor.author","Michel, U."],["dc.contributor.author","Oliveira, L. M. A."],["dc.contributor.author","Jovin, T. M."],["dc.contributor.author","Liman, Jan"],["dc.contributor.author","Tatenhorst, L."],["dc.contributor.author","Bähr, M."],["dc.contributor.author","Lingor, P."],["dc.date.accessioned","2017-09-07T11:43:42Z"],["dc.date.available","2017-09-07T11:43:42Z"],["dc.date.issued","2015"],["dc.description.abstract","Many neuropathological and experimental studies suggest that the degeneration of dopaminergic terminals and axons precedes the demise of dopaminergic neurons in the substantia nigra, which finally results in the clinical symptoms of Parkinson disease (PD). The mechanisms underlying this early axonal degeneration are, however, still poorly understood. Here, we examined the effects of overexpression of human wildtype alpha-synuclein (alpha Syn-WT), a protein associated with PD, and its mutant variants alpha Syn-A30P and -A53T on neurite morphology and functional parameters in rat primary midbrain neurons (PMN). Moreover, axonal degeneration after overexpression of alpha Syn-WT and -A30P was analyzed by live imaging in the rat optic nerve in vivo. We found that overexpression of alpha Syn-WT and of its mutants A30P and A53T impaired neurite outgrowth of PMN and affected neurite branching assessed by Sholl analysis in a variant-dependent manner. Surprisingly, the number of primary neurites per neuron was increased in neurons transfected with alpha Syn. Axonal vesicle transport was examined by live imaging of PMN co-transfected with EGFP-labeled synaptophysin. Overexpression of all alpha Syn variants significantly decreased the number of motile vesicles and decelerated vesicle transport compared with control. Macroautophagic flux in PMN was enhanced by alpha Syn-WT and -A53T but not by alpha Syn-A30P. Correspondingly, colocalization of alpha Syn and the autophagy marker LC3 was reduced for alpha Syn-A30P compared with the other alpha Syn variants. The number of mitochondria colocalizing with LC3 as a marker for mitophagy did not differ among the groups. In the rat optic nerve, both alpha Syn-WT and -A30P accelerated kinetics of acute axonal degeneration following crush lesion as analyzed by in vivo live imaging. We conclude that alpha Syn overexpression impairs neurite outgrowth and augments axonal degeneration, whereas axonal vesicle transport and autophagy are severely altered."],["dc.description.sponsorship","Open-Access Publikationsfonds 2015"],["dc.identifier.doi","10.1038/cddis.2015.169"],["dc.identifier.gro","3141868"],["dc.identifier.isi","000358788800011"],["dc.identifier.pmid","26158517"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/12015"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/1967"],["dc.language.iso","en"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.intern","Merged from goescholar"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","PUB_WoS_Import"],["dc.relation.issn","2041-4889"],["dc.rights","CC BY 4.0"],["dc.rights.uri","https://creativecommons.org/licenses/by/4.0"],["dc.subject","Central nervous system; Molecular neuroscience; Parkinson's disease"],["dc.title","Alpha-Synuclein affects neurite morphology, autophagy, vesicle transport and axonal degeneration in CNS neurons"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","1013"],["dc.bibliographiccitation.issue","6"],["dc.bibliographiccitation.journal","Journal of Neurochemistry"],["dc.bibliographiccitation.lastpage","1023"],["dc.bibliographiccitation.volume","129"],["dc.contributor.author","Liman, Jan"],["dc.contributor.author","Deeg, S."],["dc.contributor.author","Voigt, A."],["dc.contributor.author","Voßfeldt, H."],["dc.contributor.author","Dohm, C. P."],["dc.contributor.author","Karch, A."],["dc.contributor.author","Weishaupt, Jochen"],["dc.contributor.author","Schulz, J. B."],["dc.contributor.author","Bähr, M."],["dc.contributor.author","Kermer, P."],["dc.date.accessioned","2017-09-07T11:46:13Z"],["dc.date.available","2017-09-07T11:46:13Z"],["dc.date.issued","2014"],["dc.description.abstract","Spinocerebellar ataxia type 3 (SCA3) is one of at least nine inherited neurodegenerative diseases caused by an expansion of a polyglutamine tract within corresponding disease-specific proteins. In case of SCA3, mutation of Ataxin-3 results in aggregation of misfolded protein, formation of intranuclear as well as cytosolic inclusion bodies and cell death in distinct neuronal populations. Since cyclin-dependent kinase-5 (CDK5) has been shown to exert beneficial effects on aggregate formation and cell death in various polyglutamine diseases, we tested its therapeutic potential for SCA3. Our data show increased caspase-dependent Ataxin-3 cleavage, aggregation, and neurodegeneration in the absence of sufficient CDK5 activity. This disease-propagating effect could be reversed by mutation of the caspase cleavage site in Ataxin-3. Moreover, reduction of CDK5 expression levels by RNAi in vivo enhances SCA3 toxicity as assayed in a Drosophila model for SCA3. In summary, we present CDK5 as a potent neuroprotectant, regulating cleavage and thereby toxicity of Ataxin-3 and other polyglutamine proteins. We propose that increased caspase-dependent cleavage of mutated Ataxin-3, because of missing CDK5 shielding, leads to aggregation and cell death. Moreover, reduction of CDK5 expression levels by RNAi in vivo enhances SCA3 toxicity as assayed in a Drosophila model for SCA3. We think that CDK5 functions as a shield against cleavage-induced toxification and thereby is an interesting target for therapeutic intervention in polyQ disease in general."],["dc.identifier.doi","10.1111/jnc.12684"],["dc.identifier.gro","3142111"],["dc.identifier.isi","000337760500011"],["dc.identifier.pmid","24548080"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/4666"],["dc.language.iso","en"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","PUB_WoS_Import"],["dc.relation.eissn","1471-4159"],["dc.relation.issn","0022-3042"],["dc.title","CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegeneration"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.artnumber","163"],["dc.bibliographiccitation.journal","BMC Neurology"],["dc.bibliographiccitation.volume","13"],["dc.contributor.author","Wasser, Katrin"],["dc.contributor.author","Karch, André"],["dc.contributor.author","Gröschel, Sonja"],["dc.contributor.author","Witzenhausen, Janin"],["dc.contributor.author","Gröschel, Klaus"],["dc.contributor.author","Bähr, Mathias"],["dc.contributor.author","Liman, Jan"],["dc.date.accessioned","2017-09-07T11:47:04Z"],["dc.date.available","2017-09-07T11:47:04Z"],["dc.date.issued","2013"],["dc.description.abstract","Background: In-stent restenosis (ISR) is an important factor endangering the long-term safety and efficacy of carotid artery angioplasty and stenting (CAS). It is plausible that soft vulnerable plaques are more likely to be injured during CAS procedure and are therefore more likely to initiate the cascade finally leading to ISR. The aim of this study was to investigate if plaque morphology detected by a simple applicable Duplex ultrasound score before CAS can be used as a predictor for ISR. Methods: Within a prospectively collected single-centre CAS database of 281 patients (comprising 300 arteries) with high-grade carotid artery stenosis, who underwent CAS between May 2003 and January 2013, we conducted a nested case-control study. Plaque morphology before CAS was analysed by a blinded investigator and each parameter of the Total Plaque Risk Score (TPRS) as well as the whole score was evaluated with regard to its diagnostic validity for ISR. Results: We analysed the data of 10 patients with ISR and 50 patients without ISR. There were no significant differences with respect to baseline characteristics, vascular risk factors, and degree of stenosis between patients with and without ISR. The duration of follow-up was longer in patients with ISR (p = 0.024) and these patients were more likely to show increased PSV (p = 0.012) immediately after CAS than patients without ISR. Neither individual parameters of the TPRS score nor the score as a whole were suitable as a diagnostic test for ISR development. Conclusions: In the present study we could demonstrate that the non-contrast enhanced DUS of the pre-interventional plaque formation cannot be used as a predictor for the development of ISR. Evaluating a more sophisticated, but not routinely available approach e. g. by ultrasound based plaque perfusion imaging or CT based plaque analysis could be helpful in the future in order to assess the role of plaque morphology in the context of ISR development."],["dc.identifier.doi","10.1186/1471-2377-13-163"],["dc.identifier.gro","3142254"],["dc.identifier.isi","000329060500003"],["dc.identifier.pmid","24191865"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/9475"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/6243"],["dc.language.iso","en"],["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.relation.issn","1471-2377"],["dc.rights","CC BY 2.0"],["dc.rights.uri","https://creativecommons.org/licenses/by/2.0"],["dc.title","Plaque morphology detected with Duplex ultrasound before carotid angioplasty and stenting (CAS) is not a predictor of carotid artery in-stent restenosis, a case control study"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","423"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","International Journal of Stroke"],["dc.bibliographiccitation.lastpage","429"],["dc.bibliographiccitation.volume","14"],["dc.contributor.author","Schnieder, Marlena"],["dc.contributor.author","Siddiqui, Tariq"],["dc.contributor.author","Karch, André"],["dc.contributor.author","Bähr, Mathias"],["dc.contributor.author","Hasenfuß, Gerd"],["dc.contributor.author","Schroeter, Marco R"],["dc.contributor.author","Liman, Jan"],["dc.date.accessioned","2020-12-10T18:38:36Z"],["dc.date.available","2020-12-10T18:38:36Z"],["dc.date.issued","2018"],["dc.identifier.doi","10.1177/1747493018816511"],["dc.identifier.eissn","1747-4949"],["dc.identifier.issn","1747-4930"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/77384"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-354"],["dc.title","Low flow in the left atrial appendage assessed by transesophageal echocardiography is associated with increased stroke severity—Results of a single-center cross-sectional study"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","21"],["dc.bibliographiccitation.journal","Brain Research"],["dc.bibliographiccitation.lastpage","26"],["dc.bibliographiccitation.volume","1198"],["dc.contributor.author","Liman, Jan"],["dc.contributor.author","Faida, Lena"],["dc.contributor.author","Dohm, Christoph P."],["dc.contributor.author","Reed, John C."],["dc.contributor.author","Bähr, Mathias"],["dc.contributor.author","Kermer, Pawel"],["dc.date.accessioned","2017-09-07T11:48:46Z"],["dc.date.available","2017-09-07T11:48:46Z"],["dc.date.issued","2008"],["dc.description.abstract","BAG1 is a potent neuroprotectant as well as a marker of differentiation in neuronal cells. It is known that BAG1 mainly localizes to the nucleus during neuronal development, whereas BAG1 shifts to the cytosol upon neuronal differentiation suggesting that distinct BAG1 functions depend on its subcellular localization. Here, we show that forced BAG1 expression within the nucleus when compared to full-length BAG1 expression and to control cells completely abolishes the neuroprotective effects of BAG1 supporting the notion that cytosolic interaction with Hsp70 is mandatory for BAG1 mediated neuroprotection. At the same time, we observed that cells can no longer differentiate into post-mitotic neurons when BAG1 is only present in the nucleus. In addition, phospho-Erk levels are decreased in those cells indicating that BAG1 has to translocate to the cytosol for Raf-dependent MAPK activation. (C) 2008 Elsevier B.V. All rights reserved."],["dc.identifier.doi","10.1016/j.brainres.2008.01.010"],["dc.identifier.gro","3143337"],["dc.identifier.isi","000254106400003"],["dc.identifier.pmid","18242589"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/840"],["dc.language.iso","en"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.relation.issn","0006-8993"],["dc.title","Subcellular distribution affects BAG1 function"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.artnumber","190"],["dc.bibliographiccitation.issue","1"],["dc.bibliographiccitation.journal","Journal of Medical Case Reports"],["dc.bibliographiccitation.volume","5"],["dc.contributor.author","Liman, Jan"],["dc.contributor.author","von Gottberg, Philipp"],["dc.contributor.author","Bähr, Mathias"],["dc.contributor.author","Kermer, Pawel"],["dc.date.accessioned","2018-03-08T09:21:27Z"],["dc.date.available","2018-03-08T09:21:27Z"],["dc.date.issued","2011"],["dc.description.abstract","Introduction Infectious ileopectineal bursitis is a rare complication after total hip replacement and is associated mainly with rheumatoid arthritis. The main complications are local swelling and pain, but communication of the inflamed bursa with the joint can occur, leading to subsequent cartilage damage and bone destruction. Case presentation We report a case of a 47-year-old Caucasian woman without rheumatoid arthritis who reported pain and palsy in her left leg almost one year after total hip replacement. She was diagnosed with an ileopectineal bursitis after total hip replacement, leading to femoral nerve palsy. The diagnosis was obtained by thorough clinical examination, the results of focused computed tomography and magnetic resonance imaging. Conclusion To the best of our knowledge, this is the first report of non-infectious ileopectineal bursitis in a patient without rheumatoid arthritis as a complication of total hip replacement. This rare case underlines the importance of proper neurologic examination of persistent conditions after orthopedic intervention in otherwise healthy individuals. We believe this case should be useful for a broad spectrum of medical specialties, including orthopedics, neurology, radiology, and general practice."],["dc.identifier.doi","10.1186/1752-1947-5-190"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/6375"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/12889"],["dc.language.iso","en"],["dc.notes.intern","GRO-Li-Import"],["dc.notes.intern","Merged from goescholar"],["dc.notes.status","final"],["dc.relation.doi","10.1186/1752-1947-5-190"],["dc.relation.issn","1752-1947"],["dc.relation.issn","1752-1947"],["dc.rights","CC BY 2.0"],["dc.rights.uri","https://creativecommons.org/licenses/by/2.0"],["dc.title","Femoral nerve palsy caused by ileopectineal bursitis after total hip replacement: a case report"],["dc.type","journal_article"],["dc.type.internalPublication","unknown"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","287"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","The Neuroradiology Journal"],["dc.bibliographiccitation.lastpage","293"],["dc.bibliographiccitation.volume","32"],["dc.contributor.author","Schnieder, M"],["dc.contributor.author","Psychogios, MN"],["dc.contributor.author","Maier, IL"],["dc.contributor.author","Tsogkas, I"],["dc.contributor.author","Schregel, K"],["dc.contributor.author","Kleinknecht, A"],["dc.contributor.author","Knauth, M"],["dc.contributor.author","Bähr, M."],["dc.contributor.author","Liman, Jan"],["dc.contributor.author","Behme, D"],["dc.date.accessioned","2020-12-10T18:38:38Z"],["dc.date.available","2020-12-10T18:38:38Z"],["dc.date.issued","2019"],["dc.identifier.doi","10.1177/1971400918791700"],["dc.identifier.eissn","2385-1996"],["dc.identifier.issn","1971-4009"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/77395"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-354"],["dc.title","The problem of strict image-based inclusion criteria for mechanical thrombectomy – an analysis of stroke patients with an initial low CBV-ASPECTS score"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.journal","Frontiers in Neurology"],["dc.bibliographiccitation.volume","11"],["dc.contributor.author","Maier, Ilko L."],["dc.contributor.author","Hofer, Sabine"],["dc.contributor.author","Eggert, Eva"],["dc.contributor.author","Schregel, Katharina"],["dc.contributor.author","Psychogios, Marios-Nikos"],["dc.contributor.author","Frahm, Jens"],["dc.contributor.author","Bähr, Mathias"],["dc.contributor.author","Liman, Jan"],["dc.date.accessioned","2021-04-14T08:31:11Z"],["dc.date.available","2021-04-14T08:31:11Z"],["dc.date.issued","2020"],["dc.description.abstract","Age-related degeneration of the cervical spinal column is the most common cause of spinal cord lesions. T1 mapping has been shown to indicate the grade and site of spinal cord compression in low grade spinal canal stenosis (SCS). Aim of our study was to further investigate the diagnostic potential of a novel T1 mapping method at 0.75 mm resolution and 4 s acquisition time in 31 patients with various grades of degenerative cervical SCS. T1 mapping was performed in axial sections of the stenosis as well as above and below. Included subjects received standard T2-weighted MRI of the cervical spine (including SCS-grading 0-III), electrophysiological, and clinical examination. We found that patients with cervical SCS showed a significant difference in T1 relaxation times within the stenosis (727 ± 66 ms, mean ± standard deviation) in comparison to non-stenotic segments above (854 ± 104 ms, p \\u0026lt; 0.001) and below (893 ± 137 ms, p \\u0026lt; 0.001). There was no difference in mean T1 in non-stenotic segments in patients (p = 0.232) or between segments in controls (p = 0.272). Mean difference of the T1 relaxation times was significantly higher in grade III stenosis (234 ± 45) vs. in grade II stenosis (176 ± 45, p = 0.037) vs. in grade I stenosis (90 ± 87 ms, p = 0.010). A higher difference in T1 relaxation time was associated with a central efferent conduction deficit. In conclusion, T1 mapping may be useful as a tool for SCS quantification in all grades of SCS, including high-grade stenosis with myelopathy signal in conventional T2-weighted imaging."],["dc.identifier.doi","10.3389/fneur.2020.574604"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/83508"],["dc.language.iso","en"],["dc.notes.intern","DOI Import GROB-399"],["dc.publisher","Frontiers Media S.A."],["dc.relation.eissn","1664-2295"],["dc.rights","http://creativecommons.org/licenses/by/4.0/"],["dc.title","T1 Mapping Quantifies Spinal Cord Compression in Patients With Various Degrees of Cervical Spinal Canal Stenosis"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","18"],["dc.bibliographiccitation.issue","1-2"],["dc.bibliographiccitation.journal","Cerebrovascular Diseases"],["dc.bibliographiccitation.lastpage","25"],["dc.bibliographiccitation.volume","45"],["dc.contributor.author","Maier, Ilko L."],["dc.contributor.author","Tsogkas, Ioannis"],["dc.contributor.author","Behme, Daniel"],["dc.contributor.author","Bähr, Mathias"],["dc.contributor.author","Knauth, Michael"],["dc.contributor.author","Psychogios, Marios-Nikos"],["dc.contributor.author","Liman, Jan"],["dc.date.accessioned","2018-04-23T11:48:23Z"],["dc.date.available","2018-04-23T11:48:23Z"],["dc.date.issued","2017"],["dc.description.abstract","Background: Endovascular treatment (EVT) has been shown to significantly improve functional outcome in patients with acute large cerebral vessel occlusions. To date, no evidence-based recommendations on blood pressure management after successful EVT exist. Previous studies showed an association between high pre-EVT systolic blood pressure (SBP) and functional outcome, but do not answer the question on how to manage blood pressure after successful recanalization. The purpose of this study was to determine the role of blood pressure measurements as a predictor for early functional outcome in patients with successful EVT. Methods: Prospectively derived data from patients with acute large vessel occlusion within the anterior circulation and EVT was analyzed in this monocentric study. Mean systolic- and maximum SBP as well as SBP-peaks have been obtained for the first 24 h after successful EVT. Predictive value of SBP for discharge modified Rankin Scale (mRS) ≤2 has been investigated using logistic regression models. Results: From 168 patients with successful EVT, 74 (44%) had a favorable outcome with an mRS ≤2. Mean- (127 vs. 131 mm Hg, p = 0.035) and maximum SBP (157 vs. 169 mm Hg, p < 0.001) as well as the number of SBP-peaks (0 vs. 1.5, p = 0.004) were lower in patients with favorable outcomes. Multivariable logistic regression showed high mean- and maximum SBP to predict unfavorable outcomes. Cutoff mean SBP was 141 mm Hg and maximum SBP 159 mm Hg. Conclusions: High SBP in the first 24 h after recanalization of acute anterior cerebral vessel occlusions is associated with unfavorable functional outcome. Interventional studies are needed to determine the role of SBP management as a modifiable parameter in the early phase after successful EVT."],["dc.identifier.doi","10.1159/000484720"],["dc.identifier.gro","3142065"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/13499"],["dc.language.iso","en"],["dc.notes.intern","lifescience updates Crossref Import"],["dc.notes.status","final"],["dc.relation.issn","1015-9770"],["dc.title","High Systolic Blood Pressure after Successful Endovascular Treatment Affects Early Functional Outcome in Acute Ischemic Stroke"],["dc.type","journal_article"],["dc.type.internalPublication","unknown"],["dc.type.peerReviewed","no"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","175"],["dc.bibliographiccitation.issue","2"],["dc.bibliographiccitation.journal","European Journal of Paediatric Neurology"],["dc.bibliographiccitation.lastpage","178"],["dc.bibliographiccitation.volume","16"],["dc.contributor.author","Liman, Jan"],["dc.contributor.author","Wellmer, Andreas"],["dc.contributor.author","Rostasy, Kevin"],["dc.contributor.author","Baehr, Mathias"],["dc.contributor.author","Kermer, Pawel"],["dc.date.accessioned","2017-09-07T11:48:57Z"],["dc.date.available","2017-09-07T11:48:57Z"],["dc.date.issued","2012"],["dc.description.abstract","NBIA/HSS is a neurodegenerative disorder associated with iron accumulation in specific brain regions. To date, the diagnosis is obtained by typical MRI changes followed by genetic mutation analysis. This procedure is laborious and limited to a few specially equipped medical centres. Since transcranial sonography (TCS) is widely used for the early diagnosis of PD in adults displaying parenchymal metal deposits, it is likely to be a reliable diagnostic tool for the early diagnosis of NBIA. In 7 patients with proven NBIA and 13 age-matched controls without record of neurological disease TCS was performed by an experienced ultrasound examiner. Data were analysed by two blinded investigators regarding hyperechogenicity and size of the substantia nigra (SN). SN size and hyperechogenicity was significantly increased in patients with NBIA compared to controls (students t-test: p < 0.001). TCS appears to be a non-invasive and inexpensive screening technique in patients with suspected NBIA. Performed by an experienced physician, it could enable an earlier diagnosis and pre-selection of patients for the MRI scan and genetic testing, which are still the diagnostic gold standard. (C) 2011 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved."],["dc.identifier.doi","10.1016/j.ejpn.2011.07.009"],["dc.identifier.gro","3142571"],["dc.identifier.isi","000301216700011"],["dc.identifier.pmid","21816641"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/8936"],["dc.language.iso","en"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.relation.issn","1090-3798"],["dc.title","Transcranial ultrasound in neurodegeneration with brain iron accumulation (NBIA)"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]