Clinical and imaging characterization of a patient with idiopathic progressive ataxia and palatal tremor.
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.
Cilia
R
R
Parkinson Institute, Istituti Clinici di Perfezionamento, Milan, Italy. roberto.cilia@tiscali.it
Righini
A
A
Marotta
G
G
Benti
R
R
Marconi
R
R
Isaias
I U
IU
Pezzoli
G
G
Antonini
A
A
D001479:Basal Ganglia / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D001480:Basal Ganglia Diseases / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D002524:Cerebellar Ataxia / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D002526:Cerebellar Diseases / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D002529:Cerebellar Nuclei / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D004298:Dopamine / Q000172:deficiency*; D050483:Dopamine Plasma Membrane Transport Proteins / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D002527:Myoclonic Cerebellar Dyssynergia / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D009207:Myoclonus / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D009434:Neural Pathways / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D009847:Olivary Nucleus / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D010300:Parkinson Disease / Q000378:metabolism / Q000473:pathology; D049268:Positron-Emission Tomography; D012012:Red Nucleus / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D015899:Tomography, Emission-Computed, Single-Photon
D050483:Dopamine Plasma Membrane Transport Proteins; D004298:Dopamine