BACKGROUND AND PURPOSE: Various lines of evidence implicate cerebral involvement beyond the motor cortex in ALS, including the cingulate gyrus and the thalamus. The purpose of this study was to assess neurodegeneration in these regions in vivo by using MRSI. MATERIALS AND METHODS: Fourteen patients with ALS and 14 healthy controls underwent MRSI by using a coronal acquisition scheme. The NAA/Cho ratio was quantified in the MCC, thalamus, and motor cortex (PCG). RESULTS: NAA/Cho was reduced in the MCC in patients with ALS compared with the controls (P = .0004). There was no difference in NAA/Cho in the thalamus (P = .59). We also found a strong correlation of NAA/Cho among the PCG, MCC, and the thalamus in controls, which was absent in patients with ALS. CONCLUSIONS: Neurodegeneration beyond the motor cortex is present in the MCC in ALS. The significant correlation of NAA/Cho among the PCG, MCC, and the thalamus in healthy subjects likely reflects the neuronal connectivity among these regions. The loss of these relationships in patients with ALS suggests that such connectivity is not responsible for the pattern of degeneration in these regions.
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