Proton MR spectroscopy of adult-onset dentatorubral-pallidoluysian atrophy.

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To quantify impairment of the basal ganglia (globus pallidus and thalamus ) in adult-onset dentatorubral-pallidoluysian atrophy (DRPLA ).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 .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 .MRS may sensitively and specifically detect biochemical alterations in susceptible regions of patients with adult-onset DRPLA .