MR and proton MR spectroscopy of the brain in hyperhomocysteinemia caused by methylenetetrahydrofolate reductase deficiency.

MR imaging showed severe atrophy and large areas without myelination in the brain of a girl with methylenetetrahydrofolate reductase deficiency. Proton MR spectroscopy revealed mild signal reduction of N-acetylaspartate. After treatment with betaine, a second MR imaging study revealed a decrease in the size of the hypomyelinated zones that was paralleled by improved clinical status and laboratory findings.

Age-dependent changes in magnetization transfer contrast of white matter in the pediatric brain.

BACKGROUND AND PURPOSE: It is unknown to what extent magnetization transfer contrast (MTC) in white matter of the brain changes during myelination. The goal of this study was to measure the age-dependent changes of MTC in different regions of the pediatric brain and to evaluate their relation to T2 relaxation times. METHODS: Seventy children aged 1 week to 80 months without evidence of organic brain disease underwent MR imaging of the brain. A double-echo spin-echo (SE) sequence and an SE sequence with and without an off-resonance pulse were performed in the axial orientation. Using paired images, we calculated MTC ratios in 13 predefined regions of the brain and compared them with the T2 relaxation times measured in the same areas. Regression analysis was performed for both parameters to evaluate age dependency. RESULTS: MTC in white matter increased during myelination from a range of 13% to 19% to a range of 34% to 37%. At the same time, T2 relaxation times decreased from a range of 115 to 160 milliseconds to a range of 60 to 70 milliseconds after myelination. For both MTC and T2 relaxation times, age dependency could be expressed by a monoexponential function. CONCLUSION: A strong positive correlation exists between MTC ratios and the degree of myelination in the pediatric brain, and an inverse correlation exists between MTC and T2 relaxation times. Fast proton relaxation within macromolecules in the myelinated white matter and subsequent MT may be the most important reason for the decreasing T2 relaxation time of white matter during brain myelination.

Global estimation of myelination in the developing brain on the basis of magnetization transfer imaging: a preliminary study.

BACKGROUND AND PURPOSE: In the developing brain, myelination occurs in an orderly and predetermined sequence. The aim of this study was to determine whether such changes can be tracked using volumetric magnetization transfer imaging. METHODS: Three-dimensional magnetization transfer imaging was performed in 50 children (age range, 0.6-190 months) with no evidence of developmental delay or structural abnormalities. Volumetric magnetization transfer ratio (MTR) parameters generated of the whole brain were mean MTR and height and location of the MTR histogram peak. Relationships between volumetric MTR parameters and age were assessed using nonlinear regression analysis. RESULTS: With age, all volumetric MTR parameters changed exponentially in a way that was best expressed by the function y = a + b.exp(-x/c) (P < .0001). The peak height of the MTR histogram was the parameter that changed most predictably and that continued to change for the longest period of time. CONCLUSION: With this preliminary study, we show that by using volumetric MTR analysis, it is possible to monitor changes in the developing brain, presumably the myelination progress. This method has a potential role for detecting myelination disorders in the pediatric population, for studying the natural history of these diseases, and for monitoring the effects of treatment.

[Gadodiamide and gadolinium-DTPA as intravenous media in computed tomography]. / Gadodiamid und Gadolinium-DTPA als intravenöse Kontrastmittel in der Computertomographie.

PURPOSE: To evaluate the effect of intravenously applied gadolinium-based contrast medium in computed tomographic (CT) studies. MATERIAL AND METHODS: Serial dilutions of iohexol 300, Gd-DTPA and gadodiamide were scanned with CT in a phantom study using water filled tubes. For quantification of x-ray attenuation, the mean Hounsfield units (HU) were calculated from the CT scans. Five patients with contraindications against iodine contrast agents were examined with abdominal or thoracic CT before and after application of 0.2 mmol/kg body weight of a gadolinium-based contrast agent. In these patients attenuation values were obtained in ROI from unenhanced and enhanced CT scans. RESULTS: The phantom study revealed a 38.4% enhancement for Gd-DTPA and a 35.7% enhancement for gadodiamide scaled on the reference measurements with iohexol 300. Thus, 130.2 ml Gd-DTPA or 140.1 ml gadodiamide are needed to achieve the same attenuation as an i.v. injection of 50 ml iohexol 300. Consequently the corresponding dose of 1 mmol/kg body weight would exceed the manufacturer's recommended dose. In four patients with complete thoracic or abdominal CT, i.v. applied gadolinium-based contrast medium (0.2 mmol/kg) yielded no visible advantage. In these patients parenchymal enhancement did not exceed 25%. Dynamic CT of a patient with focal liver lesion revealed an arterial enhancement peak of 75%. CONCLUSION: Sufficient parenchymal enhancement in CT studies cannot be achieved with the available gadolinium-based contrast mediums. They might be helpful if only short time vascular enhancement is required.

[Magnetic resonance tomography and localized proton spectroscopy in 2 siblings with Canavan's disease]. / Magnetresonanztomographie und lokalisierte Protonenspektroskopie bei zwei Geschwisterkindern mit Morbus Canavan.

We present the findings of magnetic resonance imaging (MRI) and localised 1H magnetic resonance spectroscopy (MRS) in two brothers with Canavan's disease, a rare autosomal recessive leukodystrophy. Urine specimens of one child were evaluated by MRS. All examinations were performed in the same whole body 1.5 T superconducting magnet. MRI revealed the typical pattern of leukodystrophy including a more severe demyelination in the older child. The younger brother showed additional high signal lesions in the globi pallidi on T2-weighted images. MRS of the brain had an elevated ratio of N-acetyl-aspartate (NAA)/phosphocreatin + creatin (Cr) while the ratio of Cholin/Cr was reduced. in urine spectroscopy the concentration of NAA was markedly increased. The ratio of NAA/creatin + creatinin was 880 +/- 10% mmol/mol (normal: 5-21 mmol/mol). Diagnosis of Canavan's disease was supported by gas chromatographic urine examination with an 80-100 fold elevation of NAA concentration. Hence, the diagnosis of Canavan's disease could be established by increased ratio of NAA/Cr and decreased ratio of Cho/Cr relation in brain spectroscopy and high NAA concentration in urine spectroscopy.

The value of new MRI techniques in adrenoleukodystrophy.

PURPOSE: To evaluate the significance of new techniques in MRI examinations of the brain in children with X-linked adrenoleukodystrophy (ALD). MATERIALS AND METHODS: Five patients aged between 2 and 15 years with childhood ALD were examined with MRI. Following conventional spin-echo (CSE) sequence, a fast spin-echo (FSE) sequence with T2 contrast was performed in four patients, an FSE turbo-inversion-recovery (TIR) sequence in two, magnetization transfer contrast (MTC) imaging in two, and localized proton spectroscopy (MRS) in four patients. RESULTS: FSE compared favorably with CSE with a time saving of 70%. On TIR images the contrast between normal and demyelinated white matter was greater than on CSE and FSE images. Calculated MTC values revealed a severe MTC loss within the demyelinated regions and a moderate reduction in the border zones. In this way, calculation of MTC might be useful to differentiate between edematous changes and areas of irreversible demyelination. MRS revealed a reduction in N-acetylaspartate and an elevation in choline (Cho). The degree of MRS changes paralleled the severity of demyelination. A Cho elevation may precede visible demyelination on T2-weighted images. CONCLUSIONS: T2-weighted FSE sequences can replace CSE without any disadvantages and with effective time saving. The indication for MTC imaging and MRS in children with ALD is not yet finally defined. These new techniques may reveal the earliest signs of cerebral involvement or of disease progression, a matter of great importance in selecting the optimal therapeutic approach.

1H T1 and T2 measurements of the MR imaging contrast agents Gd-DTPA and Gd-DTPA BMA at 1.5T.

We report in vitro T1 and T2 relaxation studies for the open-chain complexes Gd-DTPA and Gd-DTPA BMA. Measurements were performed on phantoms containing aqueous and plasma solutions of different concentrations by MR imaging in a 1.5T superconducting whole-body scanner. Longitudinal relaxation times T1 were evaluated from serial turbo-FLASH experiments for concentrations less than 1 mM, whereas for larger concentrations the values were obtained from a standard inversion recovery (IR) sequence. Transverse relaxation times T2 were determined using multi-echo spin-echo MRI protocols. The T1 and T2 relaxivities of the nonionic Gd-DTPA BMA are similar to those of the Gd-DTPA. The temperature dependencies of the relaxivities were determined over a temperature interval ranging from 21 to 50 degrees C and were found to be slightly different for the two contrast agents. In the case of Gd-DTPA BMA a larger deviation of the expected temperature behavior of the relaxivities was observed as compared with Gd-DTPA. Deviations from a strictly linear dependence of relaxation times on temperature were found at lower concentrations in aqueous solutions. In plasma solutions a high T1/T2 ratio was observed for low concentrations, which decreased monotonically with increasing concentrations.

Mapping of the cortical motor hand area with functional MR imaging and MR imaging-guided laser-induced interstitial thermotherapy of brain tumors. Work in progress.

PURPOSE: To localize the cortical motor hand area with functional magnetic resonance (MR) imaging before and after MR imaging--guided laser-induced interstitial thermotherapy of tumors in the precentral brain region to control energy delivery and to improve safety. MATERIALS AND METHODS: Functional MR images were obtained in eight patients (five men, three women; aged 27-63 years) while they flexed their fingers. MR imaging--guided laser-induced interstitial thermotherapy was terminated when there was less than 8-12 mm between the border of the laser-induced lesion and the motor hand area anterior aspect. RESULTS: Seven patients had a statistically significant localized change in signal intensity in the central region of the contralateral hemisphere. This area was a spotlike circumscribed focus in three patients and scattered over a larger zone in four patients. Persistent deficits did not occur after thermotherapy in any patient. In three patients, onset of reversible perifocal edema in the motor hand area coincided with the development of hemiparesis, which completely resolved. No patient had activity within the tumor on functional MR images. CONCLUSION: Functional MR imaging findings can be used to prevent neurologic damage during MR imaging--guided laser-induced interstitial thermotherapy.

Determination of laser-induced temperature distributions using echo-shifted TurboFLASH.

An echo-shifted TurboFLASH sequence implemented on a clinical whole body MR scanner was used to determine thermal changes in tissue. With this snapshot-like data acquisition, temperature-related phase shifts were measured with a temporal resolution of 1.3 s. For different types of tissue (postmortem porcine brain, liver, and muscle) the temperature coefficients of the proton chemical shift were recorded during uniform heating of the specimen in a water bath. The specific temperature-dependent frequency shifts appeared similar to the proton chemical shift of free water (-0.01 ppm/degrees C). With this method, laser-induced ablation in postmortem porcine brain was monitored by temperature mapping. Comparison of the induced temperature profiles measured with NiCrNi-thermocouples with the MR calculated profiles demonstrated excellent temperature sensitivity and accuracy for this method of MR thermometry, with a maximum deviation of the determined temperatures of only 1.8 degrees C. This investigation was designed as a feasibility study for this rapid version of the phase mapping method, and no in vivo studies were performed.