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  • 1
    Electronic Resource
    Electronic Resource
    Hallervorden-Spatz-Krankheit Befundkonstellation am nigrostriatalen System (2000)
    Springer
    Der Nervenarzt 71 (2000), S. 660-665 
    Details
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Hallervorden-Spatz-Krankheit ; Parkinsonsyndrom ; MRT ; 123J-β-CIT ; 123J-IBZM ; Keywords Hallervorden-Spatz Disease ; Parkinson's syndrome ; MRI ; 123I-β-CIT ; 123I-IBZM
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Hallervorden-Spatz disease (HSD), a rare extrapyramidal motor illness, is usually only confirmed after death. In vivo diagnosis has relied hitherto on the combination of typical magnetic resonance imaging (MRI) findings (the “eye of the tiger” sign) and heterogeneous clinical symptoms of movement disorder which have been regarded as almost pathognomonic. We report on the diagnostic contribution of 123J-β-CIT single photon emission computed tomography (SPECT) and 123J-IBZM SPECT in akinetic-rigid Parkinson's syndrome occurring in a case of HSD. In contrast to Parkinson's disease and multisystem atrophies, the results of both tests were normal. This constellation of findings shows that the degeneration lies primarily outside the nigrostriatal system, supporting arguments for the nosologic distinction of HSD from other extrapyramidal illnesses.
    Notes: Zusammenfassung Die Diagnose der Hallervorden-Spatz-Krankheit (HSD), einer seltenen extrapyramidal-motorischen Erkrankung, konnte gewöhnlich erst postmortal gesichert werden. Bislang galt der typische MRT-Befund mit dem “Tigerauge-Zeichen” in Kombination mit der heterogen klinischen Symptomatik von Bewegungsstörungen als nahezu pathognomonisch und diente zur Diagnosestellung zu Lebzeiten. In der vorliegenden Kasuistik soll der diagnostische Beitrag von 123J-β-CIT und 123J-IBZM-SPECT bei einem akinetisch-rigiden Parkinsonsyndrom im Rahmen einer HSD dargestellt werden. Für beide Untersuchungen wurden im Gegensatz zum M. Parkinson und Multisystematrophien Normalbefunde gefunden. Diese Befundkonstellation zeigt einerseits, dass die Degeneration primär außerhalb des nigrostriatalen Systems liegt, und andererseits unterstützt sie die Diskussion der nosologischen Abgrenzung der HSD von anderen extrapyramidalen Erkrankungen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001150050643
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  • 2
    Electronic Resource
    Electronic Resource
    The influence of head position and head reorientation on the axis of eye rotation and the vestibular time constant during postrotatory nystagmus (1992)
    Springer
    Experimental brain research 91 (1992), S. 121-128 
    Details
    ISSN: 1432-1106
    Keywords: Vestibuloocular reflex ; Velocity storage ; Active head tilt ; Semicircular canals ; Otolith ; Human
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Reorienting the head with respect to gravity during the postrotatory period alters the time course of postrotatory nystagmus (PRN), hastening its decline and thereby reducing the calculated vestibular time constant. One explanation for this phenomenon is that the head reorientation results in a corresponding reorientation of the axis of eye rotation with respect to head coordinates. This possibility was investigated in 10 human subjects whose eye movements were monitored with a three-dimensional magnetic field — search — coil technique using a variety of head reorientation paradigms in a randomized order during PRN following the termination of a 90°/s rotation about earth vertical. Average eye velocities were calculated over two time intervals: from 1 s to 2 s and from 7 s to 8 s after cessation of head rotation. The time constant was estimated as one third of the duration of PRN. For most conditions, a reorientation of the head with respect to gravity 2 s after the rotation had stopped did not significantly alter the direction of the eye velocity vector of PRN with respect to head coordinates. This strongly indicates that, in humans, PRN is mainly stabilized in head coordinates and not in space coordinates, even if the otolith input changes. This finding invalidates the notion that the shortening of PRN due to reorientation of the head could be due to a change of the eye velocity vector towards a direction (torsion), which is not detectable with the eye recording methods (electrooculography) used in earlier studies. The results regarding the vestibular time constant basically confirm earlier findings, showing a strong dependence on static head position, with the time constant being lowest if mainly the vertical canals are stimulated (60° nose up and 90° left ear down). In addition, the time constant was drastically shortened for tilts away from upright. The reduction in vestibular time constant with head reorientation cannot be explained solely on the basis of the dependence of the time constant on static head position. A clear example is provided by head reorientations back towards the upright position, which results in a decrease in the time constant, rather than an increase that would be expected on the basis of static head position.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00230020
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    Paper (German National Licenses)
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