TY  - JOUR
A1  - Albrecht, Franziska
A1  - Mueller, Karsten
A1  - Ballarini, Tommaso
A1  - Lampe, Leonie
A1  - Diehl-Schmid, Janine
A1  - Fassbender, Klaus
A1  - Fliessbach, Klaus
A1  - Jahn, Holger
A1  - Jech, Robert
A1  - Kassubek, Jan
A1  - Kornhuber, Johannes
A1  - Landwehrmeyer, Bernhard
A1  - Lauer, Martin
A1  - Ludolph, Albert C.
A1  - Lyros, Epameinondas
A1  - Prudlo, Johannes
A1  - Schneider, Anja
A1  - Synofzik, Matthis
A1  - Wiltfang, Jens
A1  - Danek, Adrian
A1  - Otto, Markus
A1  - Schroeter, Matthias L.
T1  - Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging
JF  - Cortex
N2  - Alien limb phenomenon is a rare syndrome associated with a feeling of non-belonging and disowning toward one's limb. In contrast, anarchic limb phenomenon leads to involuntary but goal-directed movements. Alien/anarchic limb phenomena are frequent in corticobasal syndrome (CBS), an atypical parkinsonian syndrome characterized by rigidity, akinesia, dystonia, cortical sensory deficit, and apraxia. The structure function relationship of alien/anarchic limb was investigated in multi centric structural magnetic resonance imaging (MRI) data. Whole-group and single subject comparisons were made in 25 CBS and eight CBS-alien/anarchic limb patients versus controls. Support vector machine was used to see if CBS with and without alien/anarchic limb could be distinguished by structural MRI patterns. Whole-group comparison of CBS versus controls revealed asymmetric frontotemporal atrophy. CBS with alien/anarchic limb syndrome versus controls showed frontoparietal atrophy including the supplementary motor area contralateral to the side of the affected limb. Exploratory analysis identified frontotemporal regions encompassing the pre-/and postcentral gyrus as compromised in CBS with alien limb syndrome. Classification of CBS patients yielded accuracies of 79%. CBS-alien/anarchic limb syndrome was differentiated from CBS patients with an accuracy of 81%. Predictive differences were found in the cingulate gyrus spreading to frontomedian cortex, postcentral gyrus, and temporoparietoocipital regions. We present the first MRI-based group analysis on CBS-alien/anarchic limb. Results pave the way for individual clinical syndrome prediction and allow understanding the underlying neurocognitive architecture. (C) 2019 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
KW  - Alien limb syndrome
KW  - Anarchic limb syndrome
KW  - Corticobasal syndrome
KW  - Diagnosis prediction
KW  - Support vector machine
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-221040
VL  - 117
ER  - 
TY  - JOUR
A1  - Diehl-Schmid, Janine
A1  - Licata, Abigail
A1  - Goldhardt, Oliver
A1  - Förstl, Hans
A1  - Yakushew, Igor
A1  - Otto, Markus
A1  - Anderl-Straub, Sarah
A1  - Beer, Ambros
A1  - Ludolph, Albert Christian
A1  - Landwehrmeyer, Georg Bernhard
A1  - Levin, Johannes
A1  - Danek, Adrian
A1  - Fliessbach, Klaus
A1  - Spottke, Annika
A1  - Fassbender, Klaus
A1  - Lyros, Epameinondas
A1  - Prudlo, Johannes
A1  - Krause, Bernd Joachim
A1  - Volk, Alexander
A1  - Edbauer, Dieter
A1  - Schroeter, Matthias Leopold
A1  - Drzezga, Alexander
A1  - Kornhuber, Johannes
A1  - Lauer, Martin
A1  - Grimmer, Timo
T1  - FDG-PET underscores the key role of the thalamus in frontotemporal lobar degeneration caused by C9ORF72 mutations
JF  - Translational Psychiatry
N2  - C9ORF72 mutations are the most common cause of familial frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). MRI studies have investigated structural changes in C9ORF72-associated FTLD (C9FTLD) and provided first insights about a prominent involvement of the thalamus and the cerebellum. Our multicenter, 18F-fluorodeoxyglucose positron-emission tomography study of 22 mutation carriers with FTLD, 22 matched non-carriers with FTLD, and 23 cognitively healthy controls provided valuable insights into functional changes in C9FTLD: compared to non-carriers, mutation carriers showed a significant reduction of glucose metabolism in both thalami, underscoring the key role of the thalamus in C9FTLD. Thalamic metabolism did not correlate with disease severity, duration of disease, or the presence of psychotic symptoms. Against our expectations we could not demonstrate a cerebellar hypometabolism in carriers or non-carriers. Future imaging and neuropathological studies in large patient cohorts are required to further elucidate the central role of the thalamus in C9FTLD.
KW  - diagnostic markers
KW  - psychiatric disorders
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-225308
VL  - 9
ER  -