Brain MR Imaging in dietarily treated phenylketonuria

Magnetic resonance imaging is the most efficient imaging modality to evaluate brain gray and white matter of patients with metabolic diseases [1, 2, 3]. The main purpose of out study was to investigate the relation between brain MRI abnormalities and the phenylalanine (phe) and tyrosine (tyr) blood levels in 38 phenylketonuria (PKU) patients. Increased periventricular white matter intensity on T2-weighted brain images was the only pahtologic finding in 24 patients. Brain MRI abnormalities were scored (4) and correlated with the individual mean phe and phe/ tyr levels during 1 year preceding MR examination and with phe tolerance. The appearance of MRI abnormalities on brain T2-weighted images correlates with a threshold mean phe level (averaged over the year preceding the examination).     

MR imaging of Joubert's syndrome

Joubert's syndrome is a rare developmental defect of the cerebellar vermis associated with episodic hyperpnea and apnea, abnormal eye movements, and mental retardation. The condition is usually diagnosed clinically during the neonatal period. This article reports nine patients with the syndrome (six males, three females; ages ranging from 2.5 to 9 yrs), and describes MR imaging findings in seven of these. Besides the previously described characteristic and relatively common changes of the syndrome, the MR imaging findings in these patients revealed thinned optic tracts, enlarged temporal horns in the absence of hydrocephalus, high-signal of the cerebral periventricular white matter, abnormal signal in the decussation of the superior cerebellar peduncles, and abnormal embryonic vessels associated with the dysplastic folia of the cerebellar hemispheres.     

MR imaging of abnormally rotated medulla oblongata, and a criterion for assessment of its normal orientation

In the MR imaging study of a 4-yr-old patient the medulla oblongata was detected to be rotated 25 degrees counter-clockwise with respect to the remainder of the brain and skull. A similar condition was not visually detected in the evaluation of consecutive 1100 CT and 500 MR imaging studies. The orientation of the medulla oblongata was then geometrically assessed on selected, axial inversion recovery MR images in a series of 50 normal pediatric patients. It was detected that in 70% of cases (n = 35) a line connecting the anterior median fissure and the posterior median sulcus of the medulla oblongata (interfissural line) was parallel to or blended with a vertical reference line passing through the third ventricle (midplane axis). In 30% of cases (n = 15) the interfissural line deviated from the midplane axis up to 10 degrees. It appears that deviation of the medulla oblongata to this extent should be considered to be within normal limits.     

Peroneal tendons: Use of kinematic MR imaging of the ankle to determine subluxation

The purpose of this study was to develop a technique for kinematic MRI of the ankle to evaluate subluxation of the peroneal tendons. A special device was used to perform incremental, passive positioning of the ankle from dorsiflexed to plantarflexed positions for the kinematic MRI examination. A fast spoiled gradient-recalled acquisition in the steady state pulse sequence was used to obtain axial images to assess the peroneal tendons during different positions of the ankle. Seven asymptomatic volunteers and five patients with suspected peroneal tendon subluxation were studied. There was no transverse displacement of the peroneal tendons observed in the asymptomatic subjects nor in two of the patients. Two patients had peroneal tendon subluxation observed on the kinematic MRI studies, and one patient had the peroneal tendons maintained in a displaced position in all ankle positions. The preliminary results suggest that kinematic MRI of the ankle is a potentially useful technique to facilitate evaluation of patients with suspected subluxation of the peroneal tendons, particularly in instances in which subluxation is position-dependent, and spontaneous reduction of the tendons may occur.     

Sneddon's syndrome: MR imaging findings

Sneddon's syndrome is a systemic disease characterized by generalized livedo racemosa and cerebrovascular disease. We present a case of Sneddon's syndrome which has typical dermatological lesions and occipital infarct demonstrated by both CT and MRI.     

MR imaging of the brain: tumors

The radiologic modality that most likely provides the imaging information needed in a patient suspected of having a brain tumor is MR imaging. A brain tumor can be reliably ruled out if the MR examination is performed properly and experts interpret the results as negative. If there is a tumor, however, its exact location and topography must be determined. Important for therapy and prognosis are also tumor properties such as histologic type and grade, as well as effects on adjacent brain structures. Although potentially a noninvasive method of in vivo neuropathology, MR is still far from being sufficiently specific, as dissimilar lesions may look the same despite the use of refined imaging protocols. The evolution of MR imaging continues, however, making further methodologic improvement likely. Presently, advanced methods, such as diffusion- and perfusion-weighted MR imaging, functional MR imaging, neuronavigation based on MR imaging data, and the use of MR imaging during surgery (intraoperative MR imaging), influence the way patients are treated. Likewise, follow-up imaging (monitoring) of tumor patients by MR has become more effective, and experience has shown how to distinguish reactive changes from recurrent tumor. In the future, MR imaging may gain importance in the development of novel therapeutic concepts.     

T1-Weighted MR imaging of the brain using a fast inversion recovery pulse sequence

The purpose of this paper was to develop and evaluate a fast inversion recovery (FIR) technique for T1-weighted MR imaging of contrast-enhancing brain pathology. The FIR technique was developed, capable of imaging 24 sections in approximately 7 minutes using two echoes per repetition and an alternating echo phase encoding assignment. Resulting images were compared with conventional T1-weighted spin echo (T1SE) images in 18 consecutive patients. Compared with corresponding T1SE images, FIR images were quantitatively comparable or superior for lesion-to-background contrast and contrast-to-noise ratio (CNR). Gray-to-white matter and cerebrospinal fluid (CSF)-to-white matter contrast and CNR were statistically superior in FIR images. Qualitatively, the FIR technique provided comparable lesion detection, improved lesion conspicuity, and superior image contrast compared with T1SE images. Although FIR images had greater amounts of image artifacts, there was not a statistically increased amount of interpretation-interfering image artifact. FIR provides T1-weighted images that are superior to T1SE images for a number of image quality criteria.     

Perianal complications of Crohn disease: MR imaging findings

The aim of this study was to revisit anal anatomy, to explain surgical terminology in perianal complications of Crohn disease, and to show the MR imaging findings of perianal fistulas and abscesses. To this end more than 200 patients were studied using surface coils (Helmholtz; phased array) at 1.0 and 1.5 T. Transverse and coronal T1- and T2-weighted images were obtained. Parks' classification was used to describe perianal abscesses and fistulas. This pictorial essay shows the normal anal anatomy and pathologic findings such as subcutaneous, para-anal, ischiorectal, intersphincteric, and supralevatoric abscesses and fistulas. MR imaging with surface coils is well suited to showing the anal anatomy and to reliably describing perianal abscesses and fistulas according to surgical terminology. Received: 7 October 1996; Revision received 4 December 1996; Accepted 10 December 1996     

Pulmonary aplasia: MR angiography findings

A 26-year-old woman presented with absence of the left lung. A diagnosis of pulmonary aplasia (main bronchus ending in a blind pouch) was established by bronchoscopy. Although a CT scan and MR imaging suggested the correct diagnosis. MR angiography enabled optimal demonstration of the vascular changes due to the anomaly (single main pulmonary artery and vein) and an associated anomaly (azygous continuation of the inferior vena cava).     

CT and MR findings of brain aspergillosis

CT and MR findings of a case of brain aspergillosis with histopathologic correlation are reported. On both CT and MR images, there were multiple lesions in the corticomedullary junction (CMJ) that appeared to disrupt the cortical sulci and that were not enhanced by intravenous contrast material. In most of these lesions, there were centrally located structures that were enhanced by intravenous contrast material and that appeared to be continuous from markedly enhanced adjacent dilated cortical vessels. Histopathologic examination of the autopsy specimen showed multiple hemorrhagic infarcts in the CMJ with remaining dilated cortical vessels that had been thrombosed by aspergillus hyphae.     

van der Knaap syndrome: MR imaging findings including FLAIR, diffusion imaging, and proton MR spectroscopy

A patient is reported with diffuse leukoencephalopathy associated with cystic degeneration of the white matter of the brain (van der Knaap syndrome). The changes were studied by fluid attenuated inversion recovery (FLAIR), and diffusion-weighted MR imaging. The FLAIR sequence revealed suppressed signal of the cysts, and widespread high-signal white matter changes associated with thinned cortices. On diffusion-weighted MR imaging, apparent diffusion coefficient (ADC) values ranged from 3.0 × 10^–3 to 2.7 × 10^–3 mm²/s in the temporal cysts, similar to that of CSF. The ADC values within the parenchyma ranged between 2 × 10^–3 and 2.1 × 10^–3 mm²/s, a value falling between normal parenchyma and cerebrospinal fluid, compared with a control group of three healthy subjects. The changes were also evaluated by proton MR spectroscopy, and were compared with a control group of 12 cases. Magnetic resonance spectroscopy revealed apparently increased NAA/Cr ratios in most parts of the brain. The NAA/Cho ratios were either high or low, and the Cho/Cr ratios were increased or normal in different regions. Received: 27 October 1999; Revised: 9 December 1999; Accepted: 20 December 1999     

Rapid MR imaging of a vascular challenge to focal ischemia in cat brain

Deoxygenated blood was effectively used as a magnetic resonance (MR) susceptibility contrast agent to distinguish perfused and nonperfused (ischemic) regions in a focal ischemia model in cat brain at 2 T. Modulation of cerebral blood oxygenation levels in response to apnea was followed in real time with T2*-weighted (gradient-recalled) echo-planar MR imaging. Signal loss in the T2*-weighted images occurred only in perfused tissues as blood became globally deoxygenated. These data complemented information from diffusion-weighted and contrast agent bolus–-tracking images. In addition, observation of the signal recovery behavior on reventilation in both normal and ischemic brain offered potentially useful information about the state of the cerebral autoregulatory mechanism.     

Invited. MR findings of Minamata disease — Organic mercury poisoning

We describe MR findings in patients with Minamata disease who have been followed for a long time. All patients examined were affected after daily eating of a large quantity of methylmercury-contaminated seafood, from 1955 to 1958, and showed typical neurological findings. On MR images, the visual cortex, the cerebellar vermis and hemispheres, and the postcentral cortex are significantly atrophic in Minamata disease. The visual cortex is slightly hypointense on T1-weighted images and hyperintense on T2-weighted images, probably representing the pathologic changes of status spongiosus. MRI can demonstrate the lesions located in the calcarine area, cerebellum, and postcentral gyri, which are probably related to three of the characteristic manifestations of this disease: the constriction of the visual fields, ataxia, and sensory disturbance, respectively.     

Echo-planar MR imaging of human brain oxygenation changes

Echo-planar magnetic resonance (MR) imaging was used to observe signal intensity changes in the human brain during hypoxia. Increasing arterial blood levels of deoxyhemoglobin (0%–42%) during prolonged apnea were monitored with a pulse oximeter and correlated with gray matter and white matter signal attenuation of 13% and 20%, respectively. The results suggest the possibility of using deoxyhemoglobin boluses as a physiologic, intravascular susceptibility contrast agent for assessment of local cerebral oxygen utilization.     

Cranial midline abnormalities in Dubowitz syndrome: MR imaging findings

We present a case of Dubowitz syndrome with growth hormone deficiency. An MR study revealed congenital midline abnormalities including corpus callosum dysgenesis, hypoplastic anterior pituitary gland and stalk with an ectopic neurohypophysis. Electronic Publication     

Functional MRI of the brain: localisation of eloquent cortex in focal brain lesion therapy

The aim of this study was to assess the feasibility of functional MRI (fMRI) in a clinical environment on a large patient group, and to evaluate the pretherapeutic value of localisation of eloquent cortex. Forty patients with focal brain lesions of different origin were studied using fMRI. Functional information was obtained using motor, somatosensory, auditory and phonological stimuli depending on the localisation of the lesions. To obtain information about the spatial accuracy of fMRI, the results were compared with postoperative electrocortical stimulation. Two patients with secondary trigeminal neuralgia were scanned using a motor protocol and were implanted with an extradural plate electrode. Imaging was successful in 40 of 42 patients (including the 2 with trigeminal neuralgia). These patients were analysed for strength of activation, the relation of the lesion to activation sites and the presence of mass effect. The correlation between these data and surgical findings provided significant additional clinical information. Functional MRI can be accurately performed in patients with focal brain lesions using a dedicated approach. Functional MRI offers important clinical information as a contribution to a decrease in posttherapeutic morbidity. The accuracy of the technique can be confirmed by other modalities, including invasive cortical electrostimulation. Received 19 November 1997; Revision received 23 February 1998; Accepted 3 March 1998     

MR imaging of normal rat brain at 0.35 T and correlated histology.

A custom-built small-animal transceiver was used for in vivo imaging of normal rat brain at 0.35 T, with the objective of identifying anatomic components by comparison of images with corresponding histologic sections. The cerebrum, cerebellum, brain stem, ventricles, hippocampus, and subarachnoid space were identified and cerebrospinal fluid (CSF) was differentiated from gray matter and white matter on coronal and transaxial magnetic resonance (MR) images. These images compare favorably with those obtained by others at higher field strengths in regard to delineating major neuroanatomic structures. It is concluded that this technique will be useful for investigating small-animal models of human neurologic disease involving morphologic and morphometric changes in gray matter, white matter, and CSF-filled spaces.     

Dysosteosclerosis: clinicoradiologic findings including brain MRI

Dysosteosclerosis is a very rare bone dysplasia associated with sclerosis and platyspondyly. This paper reports the clinicoradiologic and MR imaging findings in this rare condition. The primary brain MR imaging finding was retarded white matter matter myelination.     

Quantitative MR imaging of children with sickle cell disease: Striking T1 elevation in the thalamus

Nineteen patients with sickle cell disease (SCD) were examined with conventional MR imaging (cMRI), including T1- and T2-weighted sequences and MR angiography (MRA). qMRI mapping of T1 was also done using a precise and accurate inversion-recovery (PAIR) technique optimized and validated previously. In addition, 21 healthy African-American control subjects had the qMRI examination. Nonparametric Kruskal-Wallis analysis of variance of control subjects, of SCD patients without stroke, and of SCD patients with stroke showed that T1 increased with disease severity in the thalamus, frontal white matter, genu, and occipital white matter. T1 was significantly longer in SCD patients without stroke (n=13) than in control subjects (n=21) in the thalamus and frontal white matter. In addition, T1 values were significantly longer in SCD patients with stroke than in patients without stroke in the genu and frontal white matter. Abnormality of the thalamus was identified by qMRI in a substantial fraction of patients read as normal by both cMRI and MRA, suggesting that it may be possible to use T1 elevation to identify a subset of patients with SCD who are at elevated risk for stroke.     

MR imaging of sodium in the human brain with a fast three-dimensional gradient-recalled-echo sequence at 4 T

RATIONALE AND OBJECTIVES: Sodium ions play a vital role in cellular homeostasis and electrochemical activity throughout the human body. However, the in vivo detection of sodium (23Na) with magnetic resonance (MR) techniques is hindered by the fast transverse relaxation, low tissue equivalent concentration, and small gyromagnetic ratio of sodium ions compared with protons (1H). The goals of this study were to acquire MR images of sodium in the whole human brain by using a fast three-dimensional gradient-recalled-echo sequence and to investigate the effect that restrictions on specific absorption ratio have on MR imaging of sodium at 4 T. MATERIALS AND METHODS: A three-dimensional gradient-recalled-echo sequence with short echo time was developed for MR imaging of sodium. Slab encoding was removed and a hard excitation pulse was used. Five healthy human volunteers were examined in a whole-body MR imager with the use of a custom transmit-and-receive birdcage coil. Fields of view were selected to cover the entire brain: 38 x 38 cm in the axial plane, with 24 sections of 5.8 mm each or 12 sections of 1.1 cm each. The in-plane acquisition matrix was 64 x 128, and voxel size was 0.2 cm(3). RESULTS: Sodium in white matter was depicted with an acceptable signal-to-noise ratio of 20-25. The echo time, and hence the signal-to-noise ratio, was limited by the MR imager's maximum allowable gradient strength. To keep the specific absorption ratio below 3 W/kg (the limit established by the Food and Drug Administration), it was necessary to prolong the repetition time to 30 msec. CONCLUSION: The MR imaging protocol used in this study provided acceptable visualization of sodium in the whole brain in a tolerable total acquisition time of 15 minutes.