Proton Magnetic Resonance Spectroscopy in Kennedy Syndrome

Abstract

Spectroscopic data fail to provide further evidence for altered energy metabolism in KD. Metabolite changes in the brainstem indicate a reduction of the neuronal marker NA or elevated Cho. These findings may reflect neuronal loss or gliosis consistent with the known pathologic features. In a subset of patients, altered metabolite ratios best explained by neuronal loss suggest subclinical involvement of the motor region. The extent of metabolite changes does not correlate with the trinucleotide repeat length.

Proton magnetic resonance spectroscopy in Kennedy disease

“…We used 1 H-MRS to measure metabolic changes in KD patients and found NAA/Cr ratio in the motor cortex was reduced in KD patients. Karitzky et al previously reported reduced NAA/Cho and NAA/Cr ratios in the motor cortex and reduced NAA/Cho ratio in the brainstem of patients with KD at long echo-time spectra [15]. In our study, the method was similar to that of Karitzky et al Our study demonstrated reduced NAA/Cr ratio in the motor cortex of KD patients, which is in agreement with their study.…”

Proton magnetic resonance spectroscopy in Kennedy disease

“…We used 1 H-MRS to measure metabolic changes in KD patients and found NAA/Cr ratio in the motor cortex was reduced in KD patients. Karitzky et al previously reported reduced NAA/Cho and NAA/Cr ratios in the motor cortex and reduced NAA/Cho ratio in the brainstem of patients with KD at long echo-time spectra [15]. In our study, the method was similar to that of Karitzky et al Our study demonstrated reduced NAA/Cr ratio in the motor cortex of KD patients, which is in agreement with their study.…”

Proton MRS in Kennedy disease: Absolute metabolite and macromolecular concentrations

“…KD has not been widely investigated by 1 H MRS. Both, a reduced NAA/Cho ratio in the brainstem and motor cortex, and a reduced NAA/Cr ratio in the motor cortex, have been previously reported by Karitzky et al (9). In Huntington disease, another CAG repeat disease, an increased ratio of Glx/Cr was found spectroscopically (10).…”

“…A correlation between the metabolite ratios and the number of CAG repeats could not be found in the previous work of Karitzky et al (9). The reason for the new detection of this coherence may again be the use of short echo‐time spectra.…”

“…In a previous study (9), reduced NAA/Cho and NAA/Cr ratios in the motor cortex and a reduced NAA/Cho ratio in brainstem were found in the patients with KD at long echo‐time spectra. These findings are not confirmed by our present study, where no differences were found between the patients with KD and the controls in the absolute concentrations of NAA, Cho, and Cr in the pons and in the motor cortices.…”

Multimodal Evoked Potentials of Kennedy's Disease

“…25 The correlations were not significant between functional findings (TMS and MRS) and clinical parameters (age at onset, disease duration, or CAG-repeat length). 3,25 Based upon the current concept, the main pathological findings of KD were at the spinal cord and brainstem. 21 However, a few reports showed that KD might also involve cerebral hemispheres.…”

Proton magnetic resonance spectroscopy in Kennedy disease

“…We used 1 H-MRS to measure metabolic changes in KD patients and found NAA/Cr ratio in the motor cortex was reduced in KD patients. Karitzky et al previously reported reduced NAA/Cho and NAA/Cr ratios in the motor cortex and reduced NAA/Cho ratio in the brainstem of patients with KD at long echo-time spectra [15]. In our study, the method was similar to that of Karitzky et al Our study demonstrated reduced NAA/Cr ratio in the motor cortex of KD patients, which is in agreement with their study.…”

Proton MRS in Kennedy disease: Absolute metabolite and macromolecular concentrations

“…KD has not been widely investigated by 1 H MRS. Both, a reduced NAA/Cho ratio in the brainstem and motor cortex, and a reduced NAA/Cr ratio in the motor cortex, have been previously reported by Karitzky et al (9). In Huntington disease, another CAG repeat disease, an increased ratio of Glx/Cr was found spectroscopically (10).…”

“…A correlation between the metabolite ratios and the number of CAG repeats could not be found in the previous work of Karitzky et al (9). The reason for the new detection of this coherence may again be the use of short echo‐time spectra.…”

“…In a previous study (9), reduced NAA/Cho and NAA/Cr ratios in the motor cortex and a reduced NAA/Cho ratio in brainstem were found in the patients with KD at long echo‐time spectra. These findings are not confirmed by our present study, where no differences were found between the patients with KD and the controls in the absolute concentrations of NAA, Cho, and Cr in the pons and in the motor cortices.…”

Multimodal Evoked Potentials of Kennedy's Disease

“…25 The correlations were not significant between functional findings (TMS and MRS) and clinical parameters (age at onset, disease duration, or CAG-repeat length). 3,25 Based upon the current concept, the main pathological findings of KD were at the spinal cord and brainstem. 21 However, a few reports showed that KD might also involve cerebral hemispheres.…”

Proton magnetic resonance spectroscopy in Kennedy disease

“…We used 1 H-MRS to measure metabolic changes in KD patients and found NAA/Cr ratio in the motor cortex was reduced in KD patients. Karitzky et al previously reported reduced NAA/Cho and NAA/Cr ratios in the motor cortex and reduced NAA/Cho ratio in the brainstem of patients with KD at long echo-time spectra [15]. In our study, the method was similar to that of Karitzky et al Our study demonstrated reduced NAA/Cr ratio in the motor cortex of KD patients, which is in agreement with their study.…”

Proton MRS in Kennedy disease: Absolute metabolite and macromolecular concentrations

“…KD has not been widely investigated by 1 H MRS. Both, a reduced NAA/Cho ratio in the brainstem and motor cortex, and a reduced NAA/Cr ratio in the motor cortex, have been previously reported by Karitzky et al (9). In Huntington disease, another CAG repeat disease, an increased ratio of Glx/Cr was found spectroscopically (10).…”

“…A correlation between the metabolite ratios and the number of CAG repeats could not be found in the previous work of Karitzky et al (9). The reason for the new detection of this coherence may again be the use of short echo‐time spectra.…”

“…In a previous study (9), reduced NAA/Cho and NAA/Cr ratios in the motor cortex and a reduced NAA/Cho ratio in brainstem were found in the patients with KD at long echo‐time spectra. These findings are not confirmed by our present study, where no differences were found between the patients with KD and the controls in the absolute concentrations of NAA, Cho, and Cr in the pons and in the motor cortices.…”

Multimodal Evoked Potentials of Kennedy's Disease

“…25 The correlations were not significant between functional findings (TMS and MRS) and clinical parameters (age at onset, disease duration, or CAG-repeat length). 3,25 Based upon the current concept, the main pathological findings of KD were at the spinal cord and brainstem. 21 However, a few reports showed that KD might also involve cerebral hemispheres.…”