Transcranial Doppler ultrasonography in neurologically asymptomatic children and young adults with sickle cell disease

The purpose of the study was to evaluate transcranial Doppler ultrasonography for identifying cerebrovascular disease in neurologically asymptomatic children and young adults with sickle cell disease. A total of 47 consecutive patients with sickle cell disease (28 females, 19 males; age range 8 months to 29 years, mean age 9 years 6 months) were evaluated by transcranial color and duplex Doppler ultrasonography via transtemporal and occipital (2-MHz probe) as well as by transocular (5-MHz probe) approach. Eleven vessels (middle, posterior, anterior cerebral artery, vertebral artery, ophthalmic artery on each side and basilar artery) were analyzed in each patient. Following nine transcranial Doppler findings predictive for cerebrovascular disease, patients with one or more of those abnormal sonographic findings underwent MR imaging and MR angiography. In 8 patients with abnormal transcranial Doppler the MR angiography was normal. Thirty-one patients demonstrated normal results. In 15 of 16 patients with one or more abnormal Doppler findings (34% of all studied patients) MR imaging and MR angiography were performed. The MR angiography disclosed cerebrovascular stenosis in 7 patients (15% of all patients, 44% of those with pathological transcranial Doppler findings). In one of those patients MR imaging revealed silent peripheral ischemic infarction as well. Our findings indicate the usefulness of transcranial Doppler ultrasonography to reveal occult cerebrovascular lesions in neurologically asymptomatic patients with sickle cell disease. It should regularly be performed in all sickle cell patients in order to detect patients at risk for later stroke. Patients with homozygous disease and a high frequency of preceding sickle cell crises should be followed most closely. Electronic Publication     

Quantitative MRI of the brain in children with sickle cell disease reveals abnormalities unseen by conventional MRI

Conventional MRI (cMRI) has shown that brain abnormalities without clinical stroke can manifest in patients with sickle cell disease (SCD). We used quantitative MRI (qMRI) and psychometric testing to determine whether brain abnormalities can also be present in patients with SCD who appear normal on cMRI. Patients 4 years of age and older with no clinical evidence of stroke were stratified by cMRI as normal (n = 17) or abnormal (n = 13). Spin-lattice relaxation time (T1) of gray and white matter structures was measured by the precise and accurate inversion recovery (PAIR) qMRI method. Patient cognitive ability was assessed with a standard psychometric instrument (WISC-III or WISC-R). In all 30 patients with SCD, qMRI T1 was lower than in 24 age- and race-matched controls, in cortical gray matter (P < .0006) and caudate (P < .0009), as well as in the ratio of gray-to-white matter T1 (P < .008). In the 17 patients who were shown to be normal by cMRI, qMRI T1 was still lower than in controls, in both cortical gray matter (P < .02) and caudate (P < .004). Histograms of voxel T1 show that the proportion of voxels with T1 values intermediate between gray and white matter (ie, consistent with encephalomalacia) was 9% higher than controls in patients shown to be normal by cMRI (P < .05) and 15% higher than controls in patients shown to be abnormal by cMRI (P < .0005). The full scale intelligence quotient (FSIQ) of all patients with SCD was 75, compared to the FSIQ of 88 in a historical control group of patient siblings (P < .001). The FSIQ of patients shown to be normal by cMRI was 79, significantly lower than the FSIQ of patient siblings (P < .04). The FSIQ of 71 in patients shown to be abnormal by cMRI was significantly lower than both the patient siblings (P < .005) and the patients shown to be normal by cMRI (P < .04). Patients shown to be abnormal by cMRI scored lower than patients shown to be normal by cMRI, specifically on the subtests of vocabulary (P = .003) and information (P = .03). Cognitive impairment is thus significant, even in patients with SCD who were shown to be normal by cMRI, suggesting that cMRI may be insensitive to subtle neurologic damage that can be detected by qMRI. Because cognitive impairment can occur in children normal by cMRI, our findings imply that prophylactic therapy may be needed earlier in the course of SCD to mitigate neurologic damage.     

Cranial involvement in sickle cell disease

Purpose

To evaluate cranial findings in patients with neurologically symptomatic sickle cell disease (SCD).

Materials and methods

We studied 50 consecutive patients with SCD and neurologic symptoms. All patients underwent brain MR examinations: all 50 underwent classic MR imaging; 42, diffusion-weighted MR imaging; 10, MR angiography; four, MR venography; and three patients, digital subtraction angiography.

Results

Of the 50 SCD patients, 19 (38%) had normal MR findings, and 31 (62%) showed abnormalities on brain MR images. Of the 50 patients, 16 (32%) had ischemic lesions; two (4%), subarachnoid hemorrhage; one (2%), moya-moya pattern; one (2%), posterior reversible encephalopathy; one (2%), dural venous sinus thrombosis; 12 (24%), low marrow signal intensity and thickness of the diploic space; 12 (24%), cerebral atrophy; and two (4%), osteomyelitis. Twenty-seven patients (54%) presented with headache, which was the most common clinical finding.

Conclusions

The cranial involvement is one of the most devastating complications of SCD. Early and accurate diagnosis is important in the management of cranial complications of SCD.     

Whistling face syndrome: MR imaging findings in the brain

Whistling face syndrome or cranio-carpotarsal dysplasia is a very rare disorder that consists of a characteristics facies and digital abnormalities. Magnetic resonance imaging investigation of the brain in this syndrome has not been reported previously. This communication describes the brain malformations in an infant with the syndrome, which covered delayed myelination, thickened and infolded cortices (cortical dysplasia), dysplastic corpus callosum, and agenesis of the inferior vermis.     

Rapid three-dimensional T1-weighted MR imaging with the MP-RAGE sequence.

The authors investigated the application of three-dimensional (3D) magnetization-prepared rapid gradient-echo (MP-RAGE) imaging to the acquisition of small (32 x 128 x 256) T1-weighted 3D data sets with imaging times of approximately 1 minute. A theoretical model was used to study the contrast behavior of brain tissue. On the basis of these theoretical results, 3D MP-RAGE sequences were implemented on a 1.5-T whole-body imager. Thirty-two-section 3D data sets demonstrating good signal-to-noise ratios and resolution and strong T1-weighted contrast were obtained in 1 minute. Compared with standard short TR/TE spin-echo sequences with the same imaging times and comparable sequence parameters, the 3D MP-RAGE sequence delivered increases of more than 50% in the white matter/gray matter signal difference-to-noise and white matter signal-to-noise ratios, and provided almost twice as many sections. These sequences may find a clinical role in 3D scout imaging and screening and in patients with claustrophobia or trauma.     

CT and MR imaging of CNS lymphomatoid granulomatosis

Summary The clinical CT and MR imaging features of a pathologically confirmed case of lymphomatoid granulomatosis are presented. The disease was clinically confined to the central nervous system and the diagnosis was only made after brain biopsy had been performed. MR imaging revealed extensive non-confluent regions of white matter abnormality. Although uncommon, lymphomatoid granulomatosis should be included in the differential diagnosis of causes of periventricular and deep white matter lesions, even in the absence of pulmonary lesions. Specific therapy may produce clinical regression of disease.     

Thin-section MR imaging of rat brain at 4.7 T.

The brains of anesthetized 7-month-old male hooded rats were imaged in coronal, sagittal, and horizontal planes at 4.7 T. Images were obtained with a section thickness of 0.6 mm and in-plane pixel size of 0.18-0.20 mm, resulting in finer combined spatial and contrast resolution than in most previously published reports. This allowed detailed anatomic assignment of many brain structures on the basis of comparison with a histologic brain atlas. T1, apparent T2, and water proton density values of gray matter, white matter, and cerebrospinal fluid (CSF) were derived from saturation-recovery and multi-echo measurements. These values were used to calculate expected contrast-to-noise ratios as a function of TR and TE in spin-echo imaging sequences. The optimal simultaneous contrast between gray and white matter and between CSF and gray matter was obtained on images with moderate T2 weighing, with a TR of 3.6 seconds and a TE of 45 msec. The use of thin sections was found to be essential for resolving many fine structures, and the improved sensitivity provided by the high magnetic field strength was crucial for imaging such thin sections at adequate signal-to-noise ratios.     

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     

MR imaging and spectroscopy in Lhermitte-Duclos disease

Lhermitte-Duclos disease is a rare abnormality occurring in the cerebellum with only 130 cases reported in the literature. There is debate as to whether this abnormality is a hamartoma, a malformation or a tumour. In this case report we discuss the spectroscopy findings from two patients presenting with this disease. The patients, one 40-year-old Caucasian woman with a 6-year history of headaches, unsteady gait and falls, deterioration in vision and another 28-year-old Caucasian man with a 1-year history of headaches and a previous history of a transient stroke, were found to have this lesion in the cerebellum. Proton spectroscopic data were obtained using a single-voxel PRESS technique (TE=135 ms, TR=1600 ms), from the region of the abnormality. The results were expressed as ratios under the three prominent resonances representing choline (Cho), creatine (Cr), and N-acetyl (NA) moieties. The metabolite ratios were compared to normative data. The two cases demonstrated reduced ratios in NA/Cho and NA/Cr in relation to the controls. The ratios of Cho/Cr appeared closer to the normal mean ratio. There were peaks attributable to lactate in both cases. The low NA/Cr and NA/Cho ratios could be due to the apparent lack of neuronal architecture and the presence of embryonic neural tissue, which does not express NA, indicating more favourably towards a benign hamartoma rather than a tumour.     

Different findings in Tc-99m MDP bone scintigraphy of patients with sickle cell disease: report of three cases

Objective Sickle cell anemia is an inherited disorder caused by abnormal hemoglobin, the S hemoglobin. Although vaso-occlusive crises can occur virtually in any organ, they are particularly common in the bony skeleton of affected patients. Bone marrow necrosis, bone infarcts, osteomyelitis, and aseptic necrosis are common complications in patients with sickle cell disease. Beside these abnormalities of the skeletal system, diffuse micro or macro calcification resulting from both splenic infarction and repeated vaso-occlusive episodes in the kidneys can be shown by technetium-99m methylenediphosphonate (Tc-99m MDP) bone scintigraphy. We present here the different osseous and extraosseous abnormalities noted on bone scintigraphies of three patients with sickle cell anemia. Methods Whole-body bone scan was performed after injecting 740 MBq of Tc-99m MDP in three patients with sickle cell disease. Results Tc-99m MDP whole-body image of the first patient showed non-uniform uptake in the anterior and posterior aspects of multiple ribs and bilateral femurs and tibias that was attributed to repetitive infarcts. Additionally, increased activity in shoulders, right elbow, and right knee was consistent with arthritis. Tc-99m MDP image of the second patient demonstrated avascular necrosis of the left femoral head and diffuse activity in the enlarged kidneys. Increased activity in the spleen that was attributed to repetitive infarcts was visualized in bone scan of the third patient. Conclusions In light of the findings in these cases, bone scintigraphy is a reliable imaging method in detecting both osseous and extraosseous abnormalities of sickle cell disease and may be used initially.     

Three-dimensional MR imaging and display of intracranial disease: Improvements with the MP-RAGE sequence and gadolinium

Three-dimensional (3D) image rendering was performed in 14 patients who had undergone magnetic resonance (MR) imaging for focal brain lesions. The MR study included the magnetization-prepared rapid gradient-echo (MP-RAGE) sequence with 64 or 128 partitions. Resultant contiguous sections 2.5 or 1.25 mm thick, respectively, were obtained. Images were acquired before and after administration of gadopen-letate dimeglumine. Resultant 3D data sets were processed on a commercially available workstation. Correlative surgical observation was performed in four cases. All data sets were successfully processed into 3D images. The precontrast images proved superior to gadolinium-enhanced images for brain surface rendering. Postcontrast images proved superior for reconstruction of tumors and vascular structures. The 64-partition data set proved sufficient for all postprocessing. Coronal orientation was preferred to sagittal orientation for surface rendering because it provided optimal orthogonal orientation of sulcal and gyral brain surface features. Three-dimensional rendition allowed easy superposition of lesion, brain, vessels, and scalp features-all useful for surgical planning. The central sulcus was easily recognized in the mid-line partitions and traced mediolaterally for projection on the cortical surface. MP-RAGE provides a 3D data set that can be obtained in just over 3 minutes, from which clinically useful 3D renderings are possible. The rapidity of acquisition and capability for 3D rendering provides additional clinical utility.     

Actinomycetes as the causative organism of osteomyelitis in sickle cell disease

The case of a 17-year-old girl with sickle cell anaemia who presented with extensive osteomyelitis due to actinomycetes is reported. Osteomyelitis in the long bones due to actinomycosis is extremely rare. A review of the literature reveals only six cases in which actinomycetes have been isolated from lesions affecting a long bone. The occurrence of this condition in sickle cell haemoglobinopathy has not been previously reported.     

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.     

Antenatal Diagnosis of Iniencephaly: Sonographic and MR Correlation: A Case Report

Iniencephaly is an uncommon and fatal neural tube defect involving the occiput and inion, this occurs together with rachischisis of the cervical and thoracic spine, and retroflexion of the head. We report the ultrasound (US) and magnetic resonance (MR) imaging findings of a case of iniencephaly with clubfeet and arthrogryposis. The diagnosis of iniencephaly is easy to make on ultrasound due to the typical star-gazing fetus. However, the details of the fetal brain and spinal cord may not be adequately delineated on US. We found MR imaging to be superior for depicting central nervous system abnormalities. MR imaging has evolved as an imaging modality and it is complementary to fetal US, yet US remains the screening modality of choice.     

Giant hemorrhagic myelolipoma in a patient with sickle cell disease

Adrenal myelolipoma is a rare, benign tumor consisting of adipose tissue and hematopoetic elements. It is generally diagnosed as an incidental finding due to its nonfunctioning, asymptomatic nature (Meaglia and Schmidt J Urol 147:1089, 1992). With increasing size, however, as seen in this case, myelolipomas can cause flank pain and abdominal distention. This lesion was diagnosed in a young male with sickle cell disease during a vaso-occlusive crisis.     

Quantitative measurements with localized 1H MR spectroscopy in children with Canavan's disease

Canavan's disease is an autosomal recessive hereditary leukodystrophy resulting from deficiency of the enzyme aspartoacylase. Two children suffering from this metabolic brain disease were examined using image-guided localized proton spectroscopy. The absolute concentrations of metabolites were determined. These data demonstrate, for the first time, that the well known increase of the N-Acetylaspartic acid (NAA)/Cho ratio in this disease may be not only due to a reduction of choline-containing compounds in brain tissue but, at least in specific cases, also due to an increase of the NAA concentration, which is a result of the enzyme defect.     

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.     

Musculoskeletal manifestations of sickle cell disease,diagnosis with whole body MRI

Purpose

The aim of work is to define the musculoskeletal abnormalities in patients with sickle-cell disease using whole body MRI.

Patients and methods

Twenty-seven patients with known sickle cell disease were included in this study complaining of acute painful vaso-occlusive crisis. All the patients complaining of bony pain in different body regions. Some patients complaining of bony swellings and joint pain. Whole body (W.B) MRI studies were performed for all the patients .Three coronal (T1, T2, and STIR) sequences were performed for whole-body MR imaging. In selected cases, dedicated examination of certain body parts was performed.

Results

Persistent red marrow, intramedullary bone hyperplasia and bone infarcts were seen in all patients. Vertebral bone infarcts were found in 23 patients. Bilateral proximal femoral head epiphysis avascular necrosis were found in 9 patients. Osteomylitis was diagnosed in 6 patients and septic arthritis in 2 patients.

Conclusion

Whole body MRI can help identifying muscloskeletal abnormalities in sickle cell disease in a single session. MRI is a useful imaging tool in distinguishing acute osteomylitis and bone infarct. Knowledge of the range of imaging findings is crucial in order to accurately depict the complication and initiate appropriate therapy.     

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.