caa a22 4500 445810807 CHVBK 20180317145211.0 cr unu---uuuuu 170323e20110201xx s 000 0 eng 10.1007/s00415-010-5724-z doi (NATIONALLICENCE)springer-10.1007/s00415-010-5724-z Striatal dopamine function in a family with multiple SCA-3 phenotypes [Elektronische Daten] [Esther Cubo, Mateo López, Jon Ceberio, Isabel Alfonso, Begoña Martinez, José Berciano, Fernando Iglesias, José Obeso] 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. Springer-Verlag, 2010 Dopamine nationallicence SCA-3 nationallicence Parkinsonism nationallicence DAT scan nationallicence Cubo Esther Department of Neurology, Hospital General Yagüe, Avda Cid 96, 09005, Burgos, Spain aut López Mateo Burgos, Spain aut Ceberio Jon Department of Neurology, Hospital Universitario Marques de Valdecilla, University of Cantabria, Santander, Spain aut Alfonso Isabel Department of Nuclear Medicine, Hospital Divino Vallés, Burgos, Spain aut Martinez Begoña Department of Nuclear Medicine, Hospital Divino Vallés, Burgos, Spain aut Berciano José Department of Neurology, Hospital Universitario Marques de Valdecilla, University of Cantabria, Santander, Spain aut Iglesias Fernando Department of Neurology, Hospital General Yagüe, Avda Cid 96, 09005, Burgos, Spain aut Obeso José Department of Neurology, Clínica Universitaria and Medical School of Navarra, Neuroscience Centre, CIMA, University of Navarra, Pamplona, Navarra, Spain aut Journal of Neurology Springer-Verlag 258/2(2011-02-01), 308-310 0340-5354 258:2<308 2011 258 415 https://doi.org/10.1007/s00415-010-5724-z text/html Onlinezugriff via DOI 1 brief-communication jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-010-5724-z text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Cubo Esther Department of Neurology, Hospital General Yagüe, Avda Cid 96, 09005, Burgos, Spain aut NATIONALLICENCE 700 1- López Mateo Burgos, Spain aut NATIONALLICENCE 700 1- Ceberio Jon Department of Neurology, Hospital Universitario Marques de Valdecilla, University of Cantabria, Santander, Spain aut NATIONALLICENCE 700 1- Alfonso Isabel Department of Nuclear Medicine, Hospital Divino Vallés, Burgos, Spain aut NATIONALLICENCE 700 1- Martinez Begoña Department of Nuclear Medicine, Hospital Divino Vallés, Burgos, Spain aut NATIONALLICENCE 700 1- Berciano José Department of Neurology, Hospital Universitario Marques de Valdecilla, University of Cantabria, Santander, Spain aut NATIONALLICENCE 700 1- Iglesias Fernando Department of Neurology, Hospital General Yagüe, Avda Cid 96, 09005, Burgos, Spain aut NATIONALLICENCE 700 1- Obeso José Department of Neurology, Clínica Universitaria and Medical School of Navarra, Neuroscience Centre, CIMA, University of Navarra, Pamplona, Navarra, Spain aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/2(2011-02-01), 308-310 0340-5354 258:2<308 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer