Proton MR spectroscopy of adult-onset dentatorubral-pallidoluysian atrophy.
OBJECTIVE
To quantify impairment of the basal ganglia (globus pallidus and thalamus) in adult-onset dentatorubral-pallidoluysian atrophy (DRPLA).


METHODS
Five patients with genetically definite adult-onset DRPLA (aged 51 to 65 years, mean 55.6 years) and 5 age- and sex-matched healthy controls underwent conventional magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (MRS) of the brain in the voxels predominantly containing the globus pallidus or the thalamus.


RESULTS
Conventional MRI studies showed apparently normal intensities in the globus pallidus and thalamus. MRS showed that the choline (Cho)/creatine (Cr) ratio for the patients' globus pallidus, the region preferentially affected in DRPLA, was significantly higher than that in the controls (p<0.05). The N-acetylaspartate (NAA)/Cr ratio for the globus pallidus and the Cho/Cr and NAA/Cr ratios for the thalamus, the region relatively spared in this disease, did not differ significantly between the patients and controls.


CONCLUSIONS
MRS may sensitively and specifically detect biochemical alterations in susceptible regions of patients with adult-onset DRPLA.
Kin
Tesseki
T
Department of Neurology, Nara Medical University, Kashihara, Japan.
Hirano
Makito
M
Taoka
Toshiaki
T
Takamure
Miwa
M
Furiya
Yoshiko
Y
Kichikawa
Kimihiko
K
Ueno
Satoshi
S
D000368:Aged; D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000378:metabolism; D001284:Atrophy; D001480:Basal Ganglia Diseases / Q000378:metabolism* / Q000473:pathology; D016022:Case-Control Studies; D002794:Choline / Q000378:metabolism; D003401:Creatine / Q000378:metabolism; D018891:Dentate Gyrus / Q000378:metabolism* / Q000473:pathology; D004831:Epilepsies, Myoclonic / Q000378:metabolism* / Q000473:pathology; D005917:Globus Pallidus / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D018709:Statistics, Nonparametric; D013788:Thalamus / Q000378:metabolism / Q000473:pathology
D001224:Aspartic Acid; C000179:N-acetylaspartate; D003401:Creatine; D002794:Choline