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.
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