Clinical and imaging characterization of a patient with idiopathic progressive ataxia and palatal tremor

Eur J Neurol. 2007 Aug;14(8):944-6. doi: 10.1111/j.1468-1331.2007.01796.x.

Abstract

We describe clinical and imaging features of a patient with sporadic progressive ataxia and palatal tremor (PAPT) of unknown etiology. There was hypertrophy of bilateral inferior olivary nuclei with hyperintense T2-weighted signal and mild cerebellar atrophy at brain magnetic resonance imaging. 18F-fluoro-2-desoxy-d-glucose positron emission tomography scanning (FDG-PET) showed hypometabolism in the red nucleus, external globus pallidus and precuneus while FP-CIT-SPECT imaging revealed mild and progressive loss of striatal dopaminergic terminals. Our findings suggest that in idiopathic PAPT involvement of the dentato-rubro-olivary pathway occurs along with some dopaminergic dysfunction.

Publication types

  • Case Reports

MeSH terms

  • Basal Ganglia / metabolism
  • Basal Ganglia / pathology
  • Basal Ganglia / physiopathology
  • Basal Ganglia Diseases / diagnostic imaging
  • Basal Ganglia Diseases / pathology
  • Basal Ganglia Diseases / physiopathology*
  • Cerebellar Ataxia / diagnostic imaging
  • Cerebellar Ataxia / pathology
  • Cerebellar Ataxia / physiopathology*
  • Cerebellar Diseases / diagnostic imaging
  • Cerebellar Diseases / pathology
  • Cerebellar Diseases / physiopathology
  • Cerebellar Nuclei / metabolism
  • Cerebellar Nuclei / pathology
  • Cerebellar Nuclei / physiopathology
  • Diagnosis, Differential
  • Dopamine / deficiency*
  • Dopamine Plasma Membrane Transport Proteins / metabolism
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Myoclonic Cerebellar Dyssynergia / diagnostic imaging
  • Myoclonic Cerebellar Dyssynergia / pathology
  • Myoclonic Cerebellar Dyssynergia / physiopathology*
  • Myoclonus / diagnostic imaging
  • Myoclonus / pathology
  • Myoclonus / physiopathology*
  • Neural Pathways / metabolism
  • Neural Pathways / pathology
  • Neural Pathways / physiopathology
  • Olivary Nucleus / metabolism
  • Olivary Nucleus / pathology
  • Olivary Nucleus / physiopathology
  • Parkinson Disease / metabolism
  • Parkinson Disease / pathology
  • Positron-Emission Tomography
  • Red Nucleus / metabolism
  • Red Nucleus / pathology
  • Red Nucleus / physiopathology
  • Tomography, Emission-Computed, Single-Photon

Substances

  • Dopamine Plasma Membrane Transport Proteins
  • Dopamine