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Der Nuklearmediziner 2002; 25(3): 193-201
DOI: 10.1055/s-2002-34108
Dopaminerges System

© Georg Thieme Verlag Stuttgart · New York

Zerebrale Neurotransmission bei Chorea Huntington und Morbus Wilson[*]

Cerebral Neurotransmission in Huntington’s Disease and Wilson’s DiseaseH. Barthel, O. Sabri
  • Klinik und Poliklinik für Nuklearmedizin, Universität Leipzig
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Publication History

Publication Date:
16 September 2002 (online)

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Zusammenfassung

Bei der Chorea Huntington und dem Morbus Wilson handelt es sich um erbliche Erkrankungen mit unterschiedlicher neuropsychiatrischer Symptomatik, welche im Wesentlichen auf Funktionsstörungen von im Basalganglienbereich lokalisierten Neuronen zurückgeführt werden. Untersuchungen der dopaminergen Neurotransmission mit verschiedenen PET- und SPECT-Radiopharmaka ergaben dementsprechende Defizite, welche für beide Erkrankungen konkordant das prä- und postsynaptische Kompartment betrafen. Jüngere Studien deuten darüber hinaus auf Störungen anderer Neurotransmitter-Systeme, wie z. B. des serotonergen, GABAergen und Opioid-Systems, hin. Außerhalb von wissenschaftlichen Fragestellungen ist die nuklearmedizinische Bildgebung bei beiden Erkrankungen in der Primärdiagnostik eher selten erforderlich. Im Falle der Chorea Huntington ergeben sich jedoch Indikationen bei der differenzialdiagnostischen Abgrenzung zu anderen Erkrankungen mit ähnlicher Initialsymptomatik bzw. bei der Feststellung der organischen Manifestation des Gendefektes. Darüber hinaus könnte die nuklearmedizinische Neurotransmitterdiagnostik in der Zukunft bei beiden Erkrankungen wertvolle Informationen bei der Unterstützung therapeutischer Entscheidungen bzw. beim Therapiemonitoring liefern.

Abstract

Huntington’s disease and Wilson’s disease are hereditary disorders with different neuropsychiatric symptoms. In both cases, these symptoms are mainly attributed to functional alterations of neurons, which are located in the basal ganglia. According deficits have been found by investigating the dopaminergic neurotransmission with different PET and SPECT tracers. For both diseases, these deficits revealed to concordantly involve the pre- and postsynaptic compartment. Apart from the dopaminergic system, more recent studies showed alterations of other neurotransmitter systems, like the serotonergic, GABA-ergic and opioide system. Except for scientific studies, nuclear medicine imaging is not regularly required for primary diagnosis of both disorders. In the case of Huntington’s disease, however, imaging can be helpful for differential diagnosis to other diseases with similar initial symptoms and to determine the organic manifestation of the gene defect. In addition, neurotransmitter imaging with radiortracers could gain more relevance in the future in supporting decisions on specific treatments or for therapy monitoring in both diseases.

Schlüsselwörter

Chorea Huntington - Morbus Wilson - Neurotransmission - SPECT - PET

Key words

Huntington's disease - Wilson's disease - Neurotransmission - SPECT - PET

1 Professor Wolfgang Becker (‡ 18. Mai 2002) in ehrenvollem Gedenken gewidmet

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1 Professor Wolfgang Becker (‡ 18. Mai 2002) in ehrenvollem Gedenken gewidmet

Dr. Henryk Barthel

PET Oncology Group

MRC Clinical Sciences Centre

Faculty of Medicine

Imperial College

Hammersmith Hospital Campus

Du Cane Road

London W12 0NN

United Kingdom

Phone: +44/20/83 83-37 98

Fax: +44/20/83 83-20 29

Email: henryk.barthel@ic.ac.uk