Evidence of delayed nigrostriatal dysfunction in corticobasal syndrome: A SPECT follow-up study

Roberto Ceravolo, Carlo Rossi, Roberto Cilia, Gloria Tognoni, Angelo Antonini, Duccio Volterrani, Ubaldo Bonuccelli

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: To demonstrate that degeneration of substantia nigra neurons may occur at later stages of disease in some patients with corticobasal syndrome (CBS) who evidenced preserved nigrostriatal pathway at a baseline FP-CIT SPECT study. Background: Current pathological criteria for the definite diagnosis of corticobasal degeneration consider substantia nigra cell loss as a mandatory finding. However, dopamine transporter SPECT imaging performed in a large cohort of CBS patients showed about 10% of normal scans. Methods: We describe 4 patients with clinical diagnosis of CBS and normal FP-CIT SPECT at baseline whose tracer uptake resulted pathological at 1-year follow-up scan. Clinical assessment has been performed at the time of SPECT scan. A semi-quantitative approach was performed for striatal FP-CIT binding values. Results: Baseline SPECT scans have been performed after 2.3 ± 1.5 years from onset. All CBS patients presented asymmetric rigid-akinetic parkinsonism (mean Hoehn-Yahr stage 2.5; UPDRS motor score 18) with poor levodopa response and ideo-motor limb apraxia. At follow-up, neurological examination revealed some additional features, including limb dystonia, language impairment, postural instability, ocular gaze impairment, alien limb. All patients showed pathological FP-CIT uptake at the SPECT performed 10-15 months apart from the baseline scan. Conclusions: Our longitudinal FP-CIT SPECT findings support in vivo the hypothesis that substantia nigra neuronal loss may occur at later stages in some patients with CBS, despite early extrapyramidal symptoms.

Original languageEnglish
Pages (from-to)557-559
Number of pages3
JournalParkinsonism and Related Disorders
Volume19
Issue number5
DOIs
Publication statusPublished - May 2013

Keywords

  • Corticobasal syndrome
  • Parkinsonism
  • SPECT

ASJC Scopus subject areas

  • Geriatrics and Gerontology
  • Clinical Neurology
  • Neurology

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