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Striatal dopamine function in a family with multiple SCA-3 phenotypes

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Abstract

We present single photon emission computed tomography (SPECT) studies using 123IFP-CIT (DAT scan) and 123I-IBZM imaging, performed on four members of a family with genetically proven spinocerebellar ataxia type 3 (SCA-3). DAT scan showed significant asymmetric decreased binding in the striatum in the two members with predominant parkinsonian phenotype, with mild and symmetric decreased binding in the member with the cerebellar phenotype, and normal in the asymptomatic member. The IBZM SPECT studies showed mild and asymmetrical reduction of the striatal dopamine D2 receptor density (parkinsonian members). SCA-3 can present with a levodopa responsive parkinsonism phenotype, and an abnormal DAT scan showing predominant impairment of presynaptic dopamine function.

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Authors and Affiliations

  1. Department of Neurology, Hospital General Yagüe, Avda Cid 96, 09005, Burgos, Spain

    Esther Cubo & Fernando Iglesias

  2. Burgos, Spain

    Mateo Diez López

  3. Department of Neurology, Hospital Universitario Marques de Valdecilla, University of Cantabria, Santander, Spain

    Jon Infante Ceberio & José Berciano

  4. Department of Neurology, Clínica Universitaria and Medical School of Navarra, Neuroscience Centre, CIMA, University of Navarra, Pamplona, Navarra, Spain

    José A. Obeso

  5. Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Instituto Carlos III, Ministerio de Investigación y Ciencias, Madrid, Spain

    Jon Infante Ceberio, José Berciano & José A. Obeso

  6. Department of Nuclear Medicine, Hospital Divino Vallés, Burgos, Spain

    Isabel Lanchas Alfonso & Begoña Miguel Martinez

Authors
  1. Esther Cubo
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  2. Mateo Diez López
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  3. Jon Infante Ceberio
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  4. Isabel Lanchas Alfonso
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  5. Begoña Miguel Martinez
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  6. José Berciano
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  7. Fernando Iglesias
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  8. José A. Obeso
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Correspondence to Esther Cubo.

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Cubo, E., López, M.D., Ceberio, J.I. et al. Striatal dopamine function in a family with multiple SCA-3 phenotypes. J Neurol 258, 308–310 (2011). https://doi.org/10.1007/s00415-010-5724-z

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  • DOI: https://doi.org/10.1007/s00415-010-5724-z

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