phosphocreatinine and isospaglumic-acid

Krabbe disease (globoid cell leukodystrophy [GLD]) is an autosomal recessive lysosomal disorder affecting the central and peripheral nervous system. The authors performed MRS to characterize metabolic alterations and their regional variation in brain tissue in GLD in vivo.. Abnormalities of cerebral metabolite concentrations were assessed in seven patients with biochemically proven GLD-four with infantile, two with juvenile, and one with adult subtype-using quantitative localized proton MRS of standardized brain regions.. In infantile GLD, pronounced elevation of both myo-inositol and choline-containing compounds in affected white matter reflected demyelination and glial proliferation. The accompanying decrease of N-acetylaspartate pointed to neuroaxonal loss. Gray matter showed similar, albeit much milder alterations. In juvenile GLD, MRS indicated astrocytosis with minor neuroaxonal damage in white matter. In a patient with adult GLD, results of MRS of affected white matter were close to normal. MRS data are in agreement with histopathologic features of GLD.. Proton MRS provides a powerful tool for assessing metabolic disturbances and the extent of brain damage noninvasively in GLD.

Topics: Adult; Aspartic Acid; Basal Ganglia; Brain; Child; Child, Preschool; Choline; Creatinine; Dipeptides; Female; Follow-Up Studies; Humans; Infant; Inositol; Lactates; Leukodystrophy, Globoid Cell; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Male; Middle Aged; Parietal Lobe; Phosphocreatine

BACKGROUND The relative efficacy of carotid endarterectomy (CEA)/thromboendarterectomy (TEA) and carotid artery stenting (CAS) already has been compared in randomized controlled trials and a meta-analysis, but only limited data exist describing the status of cerebral metabolism before and after these interventions. The aim of the present study was to compare metabolic changes before and after treatment of carotid stenosis and assess their potential clinical implications.   MATERIAL AND METHODS Patients with asymptomatic unilateral critical internal CAS were imaged with proton 3T magnetic resonance spectroscopy (H-MRS) because the technique is more sensitive than regular magnetic resonance imaging for detection of the early signs of ischemic events. Abnormal metabolite ratios detected with H-MRS may precede actual morphological changes associated with hypoperfusion as well as reperfusion changes. Ipsilateral and contralateral middle cerebral artery vascular territories were both evaluated before and after vascular intervention. H-MRS was performed within 24 h before and after surgery. Correlations in the metabolic data from H-MRS for N-acetylaspartic acid (NAA)+N-acetylaspartylglutamate, creatinine (Cr)+phosphocreatinine, and phosphocholine+glycerophosphocholine (Cho) were sought. RESULTS H-MRS voxels from 11 subjects were analyzed. Values for dCho/CrI, dCho/CrC and Cho/Naal (P<0.001) were significantly higher ipsilaterally than contralaterally. Ratios for dNaa/ChoC and Cho/NaaC were significantly higher on the non-operated side (P<0.001). CONCLUSIONS H-MRS may be helpful for assessment of patients with CAS, particularly because unlike other modalities, it reveals postoperative changes in metabolic brain status. Initial results indicate the important role of perioperative neuroprotective treatment.

Topics: Aged; Aged, 80 and over; Aspartic Acid; Brain; Carotid Artery, Internal; Carotid Stenosis; Creatinine; Dipeptides; Endarterectomy, Carotid; Female; Glycerylphosphorylcholine; Humans; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Male; Metabolome; Middle Aged; Middle Cerebral Artery; Phosphocreatine; Phosphorylcholine; Prospective Studies; Stents