There is evidence from in vitro and animal experiments that oral creatine (Cr) supplementation might prevent or slow down neurodegeneration in Huntington's disease (HD). However, this neuroprotective effect could not be replicated in clinical trials, possibly owing to treatment periods being too short to impact on clinical endpoints. We used proton magnetic resonance spectroscopy ((1)H-MRS) as a surrogate marker to evaluate the effect of Cr supplementation on brain metabolite levels in HD.Twenty patients (age 46+/-7.3 years, mean duration of symptoms 4.0+/-2.1 years, number of CAG repeats 44.5+/-2.7) were included. The primary endpoint was metabolic alteration as measured by (1)H-MRS in the parieto-occipital cortex before (t1) and after 8-10 weeks (t2) of Cr administration. Secondary measures comprised the motor section of the Unified Huntington's Disease Rating Scale and the Mini Mental State Examination. (1)H-MRS showed a 15.6% decrease of unresolved glutamate (Glu)+glutamine (Gln; Glu+Gln=Glx; p<0.001) and a 7.8% decrease of Glu (p<0.027) after Cr treatment. N-acetylaspartate trended to fall (p=0.073) whereas total Cr, choline-containing compounds, glucose, and lactate remained unchanged. There was no effect on clinical rating scales. This cortical Glx and Glu decrease may be explained by Cr enhancing the energy-dependent conversion of Glu to Gln via the Glu-Gln cycle, a pathway known to be impaired in HD. Since Glu-mediated excitotoxicity is presumably pivotal in HD pathogenesis, these results indicate a therapeutic potential of Cr in HD. Thus, longterm clinical trials are warranted.

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The results of magnetic resonance imaging (MRI) were compared with those of arthroscopy in a prospective series of 244 patients. A dedicated system for MRI of limbs and peripheral joints--the 0.2-T Artoscan (Esaote, Italy)--was used for imaging knee joint lesions. T1-weighted spin-echo sagittal images, T2-weighted gradient-echo coronal images, and axial views for lesions of the femoropatellar joint were acquired. Paraxial sagittal and oblique coronal views were obtained for imaging of the cruciate ligaments. This protocol allowed excellent visualization of the cruciate ligaments and medial and lateral meniscus in almost all patients. Compared with arthroscopy performed within 48 h after imaging, the sensitivity, specificity, and accuracy were respectively 93%, 97%, and 95% for tears of the medial meniscus; 82%, 96%, and 93% for tears of the lateral meniscus; 100%, 100%, and 100% for tears of the posterior cruciate ligament; 98%, 98%, and 97% for tears of the anterior cruciate ligament; and 72%, 100%, and 92% for full-thickness articular cartilage lesions. The examination can be performed within 30-45 min at lower cost than diagnostic arthroscopy. MRI with a 0.2-T magnet is a safe and valuable adjunct to the clinical examination of the knee and an aid to efficient preoperative planning.