Characterization of nigrostriatal dysfunction in spinocerebellar ataxia 17

Elena Salvatore, Andrea Varrone, Valeria Sansone, Maria Nolano, Amalia C. Bruni, Anna De Rosa, Lucio Santoro, Sabina Pappatà, Alessandro Filla, Giuseppe De Michele

Research output: Contribution to journalArticlepeer-review

Abstract

Extrapyramidal signs are a main feature of spinocerebellar ataxia 17 (SCA17). However, the extent of dopaminergic: dysfunction and its correlation with parkinsonian signs are not fully understood. In order to define this, we investigated five subjects from three different families with a pathological CAG/CAA expansion in the TATA-binding protein gene (SCA17), ranging from asymptomatic carrier to patient with advanced disease, by FP-CIT SPECT. Nigrostriatal dysfunction was present in patients manifesting a fully developed phenotype but not in preclinical and early stages. Dopamine transporter reduction was symmetrical and uniform in caudate and putamen and it correlated with the clinical severity of ataxia.

Original languageEnglish
Pages (from-to)872-875
Number of pages4
JournalMovement Disorders
Volume21
Issue number6
DOIs
Publication statusPublished - Jun 2006

Keywords

  • [I]-FP-CIT SPECT
  • Nigrostriatal dopaminergic pathway
  • SCA17
  • Spinocerebellar ataxia

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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