Summary
Striatal and cortical atrophy are significant features in the pathology of Huntington's chorea (HCh). A correlative study revealed that the CT findings of atrophy in HCh (8 patients) parallel the clinical and neurophysiological findings. In offspring (8 subjects) of HCh patients, however, the CT changes were insignificant although neurophysiological data were already suspicious in four of them. One patient with hereditary non-progressive chorea showed no CT changes at all. Perhaps with a more sensitive CT scanning technique minor morphological alterations may also be detected and used for a pre-clinical diagnosis.
Zusammenfassung
Atrophien des Corpus striatum und der Hirnrinde sind typische pathologische Veränderungen bei Chorea Huntington (H.Ch.). Eine vergleichende Untersuchung konnte Parallelen zwischen computertomographischen Atrophiezeichen bei 8 Choreapatienten und neurophysiologischen Befunden zeigen. Bei 8 Nachkommen waren die Veränderungen im Computertomogramm (CT) unspezifisch und geringfügig, obwohl 4 von ihnen in den neurophysiologischen Untersuchungen auffällig waren. Ein Patient mit hereditärer nicht-progressiver Chorea zeigte keinerlei CT-Veränderungen. Es bleibt empfindlicheren CT-Scannern vorbehalten, feinere morphologische Veränderungen zu erfassen, die für die präklinische Diagnose der Chorea Huntington nützlich wären.
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Oepen, G., Ostertag, C. Diagnostic value of CT in patients with huntington's chorea and their offspring. J Neurol 225, 189–196 (1981). https://doi.org/10.1007/BF00313748
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DOI: https://doi.org/10.1007/BF00313748