Metachromatic leukodystrophy. Diffusion MR imaging and proton MR spectroscopy

Metachromatic leukodystrophy is characterized by dysmyelination caused by a deficiency of arylsulfatase-A. In a 17-month-old boy with metachromatic leukodystrophy, an echo-planar diffusion MR sequence revealed a restricted diffusion pattern in the deep white matter, manifested by high-signal on b = 1000 s/mm² images, and low ADC values (0.56 × 10^-3 mm²/s). Proton MR spectroscopy revealed a marked decrease in choline, a metabolite related to myelin turnover. These observations consisting of a restricted diffusion pattern on diffusion MR imaging, and decreased choline peaks on proton spectroscopy, likely represented dysmyelination in metachromatic leukodystrophy.     

Subacute sclerosing panencephalitis findings at MR imaging, diffusion MR imaging, and proton MR spectroscopy

A case of subacute sclerosing panencephalitis in a 2-year-old boy is reported. In addition to asymmetric lesions in the parietotemporal lobes, right thalamus, and globus pallidus, symmetric patterns were notable in the brain stem, middle cerebellar pedincles, and dentate nuclei. Proton MR spectroscopy revealed markedly decreased N-acetylaspartate peaks and normal choline and myo-inositol levels in the lesions. Diffusion MR imaging revealed an elevated diffusion pattern manifested with high apparent diffusion coefficient values (1.14-1.60 x 10(-3) mm(2)/s) compared with those in normal-appearing brain tissue (0.65-1.00 x 10(-3) mm(2)/s) and subtle high signal intensity characteristics on diffusion-weighted images obtained at b = 1000 s/mm(2).     

MR imaging and proton MR spectroscopy in adult Krabbe disease

We present the MR imaging findings in four patients (two pairs of siblings from two unrelated families) with adult Krabbe disease. In the first family, clinical presentation mimicked familial spastic paraplegia. Their MR images showed selective, increased signal intensity on T2-weighted sequences along the corticospinal tracts, most prominently in the proband and barely detectable in her brother. Proton MR spectroscopy showed increased choline and myo-inositol in the affected white matter. In the second family, the clinical presentation differed in that the signs of pyramidal tract involvement were asymmetrical, with concomitant asymmetry on MR images in one. In adults, Krabbe disease may present on MR imaging with selective pyramidal fiber involvement.     

Neuro-Behcet''s disease: diffusion MR imaging and proton MR spectroscopy

We herein report the case of a 53-year-old woman with Behcet's disease and an acute T2-hyperintense lesion in left side of the pons. Echo-planar "trace" diffusion MR imaging revealed high signal intensity changes at the lesion site on b = 1000 s/mm(2) images, initially suggesting restricted diffusion. On corresponding apparent diffusion coefficient maps, however, the lesion had high signal intensity and high apparent diffusion coefficient values (1.22 x 10(-3) mm(2)/s), compared with the contralateral normal side of the pons (0.86 x 10(-3) mm(2)/s) and compared with the normal temporal white matter (0.80 x 10(-3) mm(2)/s). This was consistent with the presence of increased diffusion, hence vasogenic edema. Proton MR spectroscopy excluded acute infarction. This particular pattern (high signal intensity on b = 1000 s/mm(2) images in association with high apparent diffusion coefficient values) likely represented the acute inflammatory process associated with disrupted brain-blood barrier in the fulminant form of neuro-Behcet's disease. Follow-up examinations 相似文献   

MR imaging and localized proton MR spectroscopy in late infantile neuronal ceroid lipofuscinosis.

PURPOSELate juvenile neuronal ceroid lipofuscinosis (NCL) is a lysosomal neurodegenerative disorder caused by the accumulation of lipopigment in neurons. Our purpose was to characterize the MR imaging and spectroscopic findings in three children with late infantile NCL.METHODSThree children with late infantile NCL and three age-matched control subjects were examined by MR imaging and by localized MR spectroscopy using echo times of 135 and 5. Normalized peak integral values were calculated for N-acetylaspartate (NAA), choline, creatine, myo-inositol, and glutamate/glutamine.RESULTSMR imaging revealed volume loss of the CNS, most prominently in the cerebellum. The T2-weighted images showed a hypointense thalamus and hyperintense periventricular white matter. Proton MR spectra revealed progressive changes, with a reduction of NAA and an increase of myo-inositol and glutamate/glutamine. In long-standing late infantile NCL, myo-inositol became the most prominent resonance. Lactate was not detectable.CONCLUSIONMR imaging in combination with proton MR spectroscopy can facilitate the diagnosis of late infantile NCL and help to differentiate NCL from other neurometabolic disorders, such as mitochondrial or peroxisomal encephalopathies.     

Differentiation of tuberculous from pyogenic brain abscesses with in vivo proton MR spectroscopy and magnetization transfer MR imaging

BACKGROUND AND PURPOSE: MR imaging features are nonspecific with respect to the causative organism for patients with brain abscesses. On the basis of the hypothesis that the biochemical environment depends on the infecting organism and might be different in tuberculous compared with pyogenic brain abscesses, this study attempted to determine whether pyogenic brain abscesses can be differentiated from tuberculous brain abscesses by use of magnetization transfer (MT) MR imaging and in vivo proton MR spectroscopy. METHODS: Twenty-seven patients with a total of 33 pyogenic brain abscesses and three patients with a total of 12 tuberculous abscesses were evaluated with in vivo MR spectroscopy and MT MR imaging. The diagnosis in all cases was based on the culture of the causative organisms and histopathology whenever done as a part of clinical management. RESULTS: All 27 patients with pyogenic brain abscesses had lipid and lactate levels of 1.3 ppm and amino acid levels of 0.9 ppm with or without the presence of succinate, acetate, alanine, and glycine, while the three patients with tuberculous abscesses showed only such lipid and lactate levels. The MT ratio from the wall of the pyogenic abscesses was significantly higher (P <.001) than that from the tuberculous abscess wall. CONCLUSION: It might be possible to differentiate tuberculous abscesses from pyogenic abscesses by using MT MR imaging and in vivo MR spectroscopy, which could be of value in influencing the management of such cases.     

Use of proton MR spectroscopy and MR imaging to assess obesity

We used ¹H MR spectroscopy and MR imaging at 9.4-T to quantify and localize fat and water in the abdominal regions of 12 lean, normal, and obese mice. The D₂O dilution method which measures also the equilibrium plasma D₂O concentration by ²H MR spectroscopy was used to quantify body water and fat. In obese mice, the intensity of the fat ¹H resonance was about 120% that of the water ¹H resonance, about threefold higher than its value (about 45%) in normal mice. In lean mice, the fat/water intensity ratio was about 1:4, about half that in normal mice. Total body water was similar in obese and normal mice (19.9 ± 1.5 and 18.7 ± 1.3 mL) despite their very different body weights (50.1 ± 3.1 g and 30.2 ± 3.1 g, respectively), but slightly lower in lean mice (14.8 ± 1.2 mL water; 22.1 g ± 2.0g weight). Selective methylene-proton images showed marked accumulation of fat in the abdomen and the retroperitoneal and subcutaneous spaces of obese mice. Selective water-proton images allowed clear resolution of the renal cortex, medulla, papilla, and urinary pelvis. The readily measurable resonance intensity ratio of abdominal fat to water is a sensitive index by which to characterize obesity.     

Magnetic resonance imaging and proton MR spectroscopy in Wilson's disease

MRI of the brain and liver using T2 relaxation time measurements and proton spectroscopy (1H-MRS) of the brain was performed in four siblings with Wilson's disease (one with clinical disease and three asymptomatic) as well as age- and sex-matched control subjects. The T2 values of the liver were correlated with liver biopsy results. 1H-MRS of the left and right globus pallidus was obtained. The patient with clinical disease was examined three times, and two of three asymptomatic siblings twice. MR images of the brain were abnormal in all four patients. High signal intensity areas in the posterior thalamus, general atrophy and pontine myelinolysis were present in the patient with clinical manifestations. The T2 measurements of these areas confirmed the results of image analysis. Apart from general brain atrophy, the changes in the patient with clinical disease were largely reversible. The T2 values were significantly different from those of the control subjects only in the globus pallidus. The NAA/Cho, NAA/Cr and Cho/Cr ratios from the 1H-MR spectra of globus pallidus showed no significant difference between patients and control subjects. The mean values of NAA/Cho and NAA/Cr were lower in patients with Wilson's disease than in the control subjects. One of the patients had hepatic steatosis, but the liver T2 values were no different to those of the control subjects. In conclusion, the MRI findings reflect the success of the specific therapy in patients. MRI thus seems to be useful in the follow-up of Wilson's disease.     

Pantothenate kinase-associated neurodegeneration: MR imaging,proton MR spectroscopy,and diffusion MR imaging findings

We herein report the case of a 15-year-old male patient with pantothenate kinase-associated neurodegeneration. The classic "eye-of-the-tiger" appearance was initially present on the globus pallidi on T2-weighted MR images and had disappeared by the time of the 10-month follow-up examination. Fluid-attenuated inversion recovery images revealed marked hypointensity in the globus pallidi and dentate nuclei and high signal intensity changes in the deep cerebral white matter. Proton MR spectroscopy revealed markedly decreased N-acetylaspartate in the globus pallidi, associated with decreased N-acetylaspartate and increased myoinositol in the deep cerebral white matter. Diffusion MR images (b=1000 s/mm(2)) were negative (normal appearing) for deep cerebral white matter lesions, whereas apparent diffusion coefficient values were slightly increased (1.08-1.12 x 10(-3) mm(2)/s), compared with the apparent diffusion coefficient values from the normal white matter regions. Apparent diffusion coefficient values in the globus pallidi were lower than those in the unaffected thalamus.     

Breathhold abdominal and thoracic proton MR spectroscopy at 3T.

The clinical utility of proton MR spectroscopy ((1)H-MRS) has been well demonstrated in the brain, prostate, and breast. The aims of this work were to investigate 1) the feasibility of abdominal and thoracic (1)H-MRS at 3T, 2) the benefits of breathholding to MRS in these regions, and 3) the utility of multiple breathhold averaging for MRS. Breathholding either eliminated or markedly reduced phase and frequency shifts and outer voxel contamination that were associated with the motion of the abdomen and the thorax during breathing. Breathholding was found to be essential to spectroscopic investigation of the thorax. Spectra of renal cell carcinoma metastases in the abdomen and thorax were obtained utilizing multiple breathhold averaging. These spectra exhibited a resonance at 3.2 ppm attributed to the trimethylamine moiety of choline metabolites. The results of this study suggest a practical strategy for implementation of (1)H-MRS in the body.     

Multivoxel proton MR spectroscopy and hemodynamic MR imaging of childhood brain tumors: preliminary observations.

PURPOSETo assess multivoxel proton MR spectroscopy combined with MR imaging and hemodynamic MR imaging in the evaluation of brain tumors in children and young adults.METHODSFifteen patients with brain tumors and 10 healthy children underwent MR imaging and MR spectroscopy on a 1.5-T system. Ten patients with tumors had both MR spectroscopy and hemodynamic MR imaging. MR spectroscopy data sets with 1 cm3 to 3.4 cm3 resolution were acquired within 8.5 minutes by using a point-resolved spectroscopic, chemical-shift imaging technique in two dimensions with volume preselection. MR imaging was performed using fast spin-echo techniques. Hemodynamic MR imaging data were acquired every 2.5 seconds at one anatomic level using a spoiled gradient-echo sequence during intravenous bolus administration of contrast material.RESULTSAssessment with multivoxel MR spectroscopy and hemodynamic MR imaging added about 30 minutes to the total MR examination time. Normal tissue exhibited spectral peaks from biologically significant compounds such as N-acetylaspartate (NAA), choline-containing compounds (Cho), and total creatine (tCr). Twelve biopsy-proved tumors exhibited prominent Cho, reduced NAA, variable tCr, and/or lactate or lipids, and two showed increased hemodynamic parameters. Three of the tumors treated with radiation did not reveal prominent levels of Cho. Tissue necrosis had no Cho, NAA, or tCr, and reduced hemodynamics.CONCLUSIONSPreliminary findings by MR spectroscopy combined with MR imaging and hemodynamic MR imaging suggest that regions of active tumor may be differentiated from areas of normal tissue and areas of necrosis. These findings may enable metabolic and hemodynamic characterization of childhood brain tumors as well as suggest their response to therapy.     

MR proton spectroscopy in multiple sclerosis.

PURPOSE: To elucidate the natural history of visualized MR abnormalities in patients with multiple sclerosis using proton spectroscopy. METHODS: MR imaging and proton spectroscopy (1H spectroscopy) were performed on 16 patients with clinically definite multiple sclerosis. All patients received gadopentetate dimeglumine (Gd-DTPA). RESULTS: Decreased levels of N-acetylaspartate (NAA) were demonstrated in 17 out of 21 lesions. No correlation was found between decreased NAA and Gd-DTPA enhancement. In five out of seven enhancing lesions, abnormal 1H spectra with extra peaks (termed marker peaks) at 2.1-2.6 ppm (ranging in absolute concentration from 10-50 mM protons) were observed. In nine out of 14 unenhancing lesions, no elevated marker peaks were observed. In the five other unenhancing lesions, the levels of these marker peaks were generally lower than the enhancing group. No correlation was found between the NAA levels and the levels of the marker peaks. We suggest two distinct biochemical processes: 1) decreased NAA reflecting neuronal cell loss, and 2) elevated marker peaks reflecting ongoing demyelination. CONCLUSIONS: Based upon these observations we infer that 1) the majority of enhancing lesions are demyelinating with extra peaks at 2.1-2.6 ppm representing a marker of this process, 2) enhancing lesions without this marker most likely represent edematous regions without significant demyelination, and 3) demyelination may be long in duration compared with transient blood-brain barrier disruption manifested by Gd-DTPA enhancement. Our results suggest that 1H spectroscopy has the ability to further categorize MR-demonstrated enhancing and unenhancing lesions in patients with multiple sclerosis and that it may be more sensitive than contrast enhancement in revealing the true time course of demyelination.     

Hypoxia-ischemic encephalopathy in full-term neonate: correlation proton MR spectroscopy with MR imaging

INTRODUCTION: To evaluate 1H Magnetic Resonance Spectroscopy (1HMRS) in the diagnosis of hypoxia-ischemic encephalopathy (HIE) of full-term neonates correlated with Magnetic Resonance Imaging (MRI). MATERIALS AND METHODS: Thirty-eight cases of full-term neonates diagnosed as HIE clinically were selected to perform MRI and 1HMRS examination. The ages ranged from 7 to 17 days, with median age of 8.2 days. In which, 26 cases were followed up and/or MRI reexamined at 6 months of age or later. Eight healthy neonates, with no evidence of birth asphyxia, also underwent 1HMRS for comparison. SE sequences were used for routine MR examination; point resolved spectroscopy sequence was required for 1HMRS. The metabolites in the spectra includes: N-acetylaspartate (NAA), choline compounds (CHO), creatine compounds (CR), myo-inositol (MI), lactate (LAC), glutamate and glutamine (Glu-Gln). RESULTS: The peaks of NAA were fall in two cases; the peaks of LAC, which were elevated, appeared as typical double-peaks appearance in 26 cases; the peaks of Glu-Gln, which were also elevated, appeared as zigzag appearance in nine cases. The peaks of CR were decreased in 11 cases, while those of MI were increased in seven cases. Mild type of lesions was present on MRI in 12 cases whose LAC/CR ratio lower than 0.5; mild and moderate types of lesions were present in 15 cases whose LAC/CR ratio between 0.5 and 1.5. Whereas, nine cases of severe lesions and two cases of moderate lesions were present on MRI in 11 cases whose LAC/CR ratio greater than 1.5. Twenty-six of 38 cases were followed up and/or MRI reexamined after 6 months, in which, sequelae were present in 12 cases. Among them, eight cases of sequelae in nine cases whose LAC/CR ratio greater than 1.5 were present (account for 88.89%). CONCLUSION: 1HMRS plays an important role to diagnose and predict outcome of HIE.     

MR imaging and proton spectroscopy of the brain in posttraumatic cortical blindness

Magnetic resonance (MR) imaging and localized proton MR spectroscopy of the occipital lobes were performed in a patient with cortical blindness following brain trauma. Computed tomography (CT) scans and MR images of the visual cortex were normal in the acute stage. Six weeks after the trauma, MR images showed cortical lesions in both occipital lobes, while the spectra showed elevated lactate and decreased N-acetyl aspartate levels relative to those of healthy volunteers. One year later, visual acuity had improved and follow-up studies revealed an increase in the ratios of N-acetyl aspartate to choline and creatine. These results demonstrate that parenchymal lesions may develop in brain regions that appear normal at CT and MR imaging during the acute stage after trauma. Metabolic changes can be observed in these areas by means of localized proton MR spectroscopy.     

Salivary gland tumors at in vivo proton MR spectroscopy

PURPOSE: To prospectively evaluate whether proton magnetic resonance (MR) spectroscopy can be used to characterize salivary gland tumors (SGTs). MATERIALS AND METHODS: Ethics committee approval and informed consent were obtained. Hydrogen 1 ((1)H) MR spectroscopy was performed with echo times of 136 and 272 msec at 1.5 T in both SGTs and normal parotid glands. Spectra were analyzed in the time domain by using prior knowledge in the fitting procedure to obtain peak amplitudes of choline (Cho), creatine (Cr), and unsuppressed water. Mean Cho/Cr and Cho/water ratios for each subgroup of SGTs were obtained, and results were compared by using a nonparametric t test. RESULTS: Successful spectra were acquired in 56 patients (35 men, 21 women; mean age, 56 years) with a total of nine malignant tumors and 47 benign SGTs (24 Warthin tumors, 22 pleomorphic adenomas, one oncocytoma). At an echo time of 136 msec, Cho/Cr ratios were obtained in 26 (47%) of 55 spectra, with a mean value (+/- standard deviation) of 1.73 +/- 0.47, 5.49 +/- 1.86, 3.46 +/- 0.84, and 2.45 for malignant tumors, Warthin tumors, pleomorphic adenomas, and oncocytoma, respectively. Differences were significant between Warthin tumors and pleomorphic adenomas (P = .028) and between benign SGTs and malignant tumors (P < .001). At an echo time of 272 msec, Cho/Cr ratios were obtained in 16 (30%) of 53 spectra, with a mean value of 2.27 +/- 0.69, 6.92 +/- 1.47, and 3.67 +/- 1.23 for malignant tumors, Warthin tumors, and pleomorphic adenomas, respectively. Differences were also significant between Warthin tumors and pleomorphic adenomas (P = .041) and benign SGTs and malignant tumors (P = .004). There was a significant difference in mean Cho/water ratio for Warthin tumors versus pleomorphic adenomas at echo times of 136 msec (P = .003) and 272 msec (P = .002) but not for benign SGTs versus malignant tumors. CONCLUSION: (1)H MR spectroscopy may be used to characterize SGTs, but a larger study is required to validate these initial results.     

Reproducibility of hippocampal single-Voxel proton MR spectroscopy and chemical shift imaging

OBJECTIVE: We investigated between- and within-acquisition reproducibility of hippocampal metabolite ratios obtained using automated proton MR spectroscopy. SUBJECTS AND METHODS: We examined 30 healthy adults with a 1.5-T scanner four times on 3 days using single-voxel spectroscopy over the left hippocampus, chemical shift imaging over the left hippocampus, and chemical shift imaging over the bilateral hippocampi. Metabolite ratios were derived from the integral values of three major peaks: N:-acetylaspartate (NAA), choline-containing compounds (Cho), and creatine plus phosphocreatine (Cr). The random-effects model of one-way analysis of variance was used to evaluate the reproducibility in terms of coefficient of variation; the mixed-effects model was used to compare the results of different hippocampal regions and spectroscopic techniques. RESULTS: Most coefficients of variation for the NAA/(Cho+Cr) ratio were less than 20%. All the coefficients of variation for the posterior hippocampus (15-25%) were less than those for the anterior hippocampus (20-44%). The posterior hippocampal NAA/(Cho+Cr) ratio of unilateral chemical shift imaging had the lowest coefficient of variation (<16%). Single-voxel spectroscopy and unilateral chemical shift imaging had similar coefficients of variation for the anterior hippocampal NAA/(Cho+Cr) ratios (17-20%). There was a significant difference in metabolite ratios measured in different hippocampal regions (p<0.01) and in those acquired with different spectroscopic techniques (p<0.001). CONCLUSION: The NAA/(Cho+Cr) ratio is the most reproducible parameter for hippocampal MR spectroscopy on a 1.5-T scanner. Regional variation and technical differences in metabolite ratios must be considered when interpreting proton spectra of the hippocampus.