Herbert Schreiber

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Facioscapulohumeral muscular dystrophy (FSHD) is associated with a decreased number of D4Z4 repeats on chromosome 4q35. Diagnostic difficulties arise from atypical clinical presentations and from an overlap in D4Z4 numbers between controls and FSHD individuals. Thus, a molecular genetic test result with a borderline D4Z4 number has its limitations for the(More)
M C Walter, J A Petersen, R Stucka, D Fischer, R Schröder, M Vorgerd, A Schroers, H Schreiber, C O Hanemann, U Knirsch, A Rosenbohm, A Huebner, N Barisic, R Horvath, S Komoly, P Reilich, W Müller-Felber, D Pongratz, J S Müller, E A Auerswald, H Lochmüller . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .(More)
We performed a longitudinal study of frontal and temporal lobe functions in patients with amyotrophic lateral sclerosis (ALS) and compared the evolution of cognitive performance with that of motor deficits in patients with spinal and bulbar–onset of the disease. Fifty two patients suffering from sporadic ALS according to the El Escorial criteria were(More)
OBJECTIVE To characterize the earliest symptoms of X-linked bulbospinal neuronopathy (Kennedy disease [KD]) during the course of the disease, including a definition of the age of onset. METHODS We describe the earliest symptoms, signs on clinical investigation, electrophysiological and muscle biopsy specimen findings, and creatine kinase levels in 34(More)
Facioscapulohumeral muscular dystrophy (FSHD) is associated with a decreased number of D4Z4 repeats on chromosome 4q35. Diagnostic difficulties arise from atypical clinical presentations and from an overlap in D4Z4 numbers between controls and FSHD individuals. Thus, a molecular genetic test result with a borderline D4Z4 number has its limitations for the(More)
Cholecystokinin (CCK) is co-localized with dopamine (DA) in neurons of the mesolimbic-frontocortical dopamine (DA) system, considered essential for the pathology of psychotic behavior and associated attention deficits. The present experiments in 13 healthy men aimed at examining the effects of the CCK analog ceruletide on attention as reflected by(More)
The timing aspects of human frontal lobe function are discussed in the light of the results of three experiments on movement-related cerebral potentials. Experiment I is based on use of a sequential tracking task and experiment II a motor learning task; experiment III deals with frontal hemispheric specialisation by comparing self-initiated writing and(More)
As part of the frontotemporal dementias, primary progressive aphasia (PPA) is typically characterized by nonfluent speech with paraphasias, but there is growing evidence that also a fluent variant of PPA exists. We describe a patient suffering from PPA who adds to the broad clinical spectrum of this disorder. Moreover, we report for the first time that PPA(More)
Twenty right-handed subjects participated in a study investigating the cerebral potentials related to three complex actions: (1) writing one's own signature, (2) drawing a pentagram, and (3) fast meaningless scribbling. The Bereitschaftspotential (BP, readiness potential) started as early as 3 s prior to writing, 2.5 s prior to drawing, but only 1.5 s prior(More)
Twenty-three offspring of schizophrenic parents (mean age = 12.1 years, SD = 3.2) and 61 adolescent control subjects (mean age = 12.3 years, SD = 3.3) were compared in their performance of the following psychometric tests: simple reaction times (RTs), warned RTs in a monomodal and cross-modal design, d2 concentration test, a motor perseveration test, and a(More)