title: Dopamine Transporter SPECT Imaging in Corticobasal Syndrome.
abstract: OBJECTIVE
  To investigate dopaminergic function in a large cohort of patients with corticobasal syndrome (CBS) and describe its relationship with clinical features in comparison to Parkinson's disease and healthy control subjects. In addition, we assessed prevalence and features of individuals with CBS and in vivo evidence of preserved nigral neuronal density.
  
  
  BACKGROUND
  Substantia nigra pars compacta (SNc) neuronal degeneration is a mandatory pathological criterion for definite corticobasal degeneration, though sporadic autopsy-proven cases with ante-mortem imaging evidence of preserved nigral terminals have been recently described.
  
  
  METHODS
  In this multicenter study, we investigated presynaptic nigrostriatal function in 36 outpatients fulfilling clinical criteria for "probable corticobasal degeneration" (age 71±7.3 years; disease duration 3.9±1.6 years), 37 PD and 24 healthy control subjects using FP-CIT single photon emission computed tomography. Clinical, neuropsychological, and magnetic resonance imaging assessment was performed to characterize CBS patients. Linear discriminant analysis was used to categorize normal vs. pathological scans.
  
  
  RESULTS
  FP-CIT binding reduction in patients with CBS was characterized by larger variability, more uniform reduction throughout the striatum and greater hemispheric asymmetry compared to PD. Moreover, there was no significant correlation between tracer uptake values and clinical features such as disease duration and severity. Despite all CBS subjects showed obvious bilateral extrapyramidal signs, FP-CIT uptake was found to be normal bilaterally in four CBS patients and only unilaterally in other four cases. Extensive clinical, neuropsychological and imaging assessment did not reveal remarkable differences between CBS subjects with normal vs. pathological FP-CIT uptake.
  
  
  CONCLUSIONS
  Our findings support the hypothesis that extrapyramidal motor symptoms in CBS are not invariably associated with SNc neuronal degeneration and that supranigral factors may play a major role in several cases. CBS individuals with normal FP-CIT uptake do not show any clinical or cognitive feature suggesting a different pathology than CBD.
authors.affiliation: Parkinson Institute, Istituti Clinici di Perfezionamento, Milan, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Cilia
authors.forename: Carlo
authors.initials: C
authors.lastname: Rossi
authors.forename: Daniela
authors.initials: D
authors.lastname: Frosini
authors.forename: Duccio
authors.initials: D
authors.lastname: Volterrani
authors.forename: Chiara
authors.initials: C
authors.lastname: Siri
authors.forename: Cristina
authors.initials: C
authors.lastname: Pagni
authors.forename: Riccardo
authors.initials: R
authors.lastname: Benti
authors.forename: Gianni
authors.initials: G
authors.lastname: Pezzoli
authors.forename: Ubaldo
authors.initials: U
authors.lastname: Bonuccelli
authors.forename: Angelo
authors.initials: A
authors.lastname: Antonini
authors.forename: Roberto
authors.initials: R
authors.lastname: Ceravolo
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology; D016022:Case-Control Studies; D050483:Dopamine Plasma Membrane Transport Proteins / Q000378:metabolism*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D019636:Neurodegenerative Diseases / Q000175:diagnosis / Q000473:pathology*; D009474:Neurons / Q000378:metabolism*; D010300:Parkinson Disease / Q000175:diagnosis / Q000473:pathology; D013378:Substantia Nigra / Q000473:pathology*; D013577:Syndrome; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods*
chemical_list: D050483:Dopamine Plasma Membrane Transport Proteins