Diagnosis of Sturge-Weber syndrome: comparison of the efficacy of CT and MR imaging in 14 cases.

Sturge-Weber syndrome is a neurocutaneous syndrome that includes facial and leptomeningeal angiomas. Imaging findings include cerebral lobar atrophy, brain calcifications, choroid plexus enlargement, cranial diplo? prominence, and venous abnormalities. We compared the efficacy of CT and MR imaging in making the diagnosis in 14 consecutive patients. CT, with and without contrast enhancement, was performed in all patients, and 11 of the 14 had MR imaging (eight before and after administration of IV gadopentetate dimeglumine). MR imaging was better than CT in showing the extent and degree of brain parenchymal atrophy, the presumed ischemic changes affecting the gray and white matter, and the cranial diploetic prominence on the affected side. MR imaging after contrast administration permitted a better evaluation of the extent and patency of the leptomeningeal angiomatous malformation and the parenchymal venous anomalies. CT was better than MR imaging in showing the presence and extent of cortical calcifications. Enhanced CT and MR imaging were equal in evaluating the prominence of the ipsilateral choroid plexus. Our experience indicates that contrast-enhanced MR imaging is the method of choice in the diagnosis of Sturge-Weber syndrome. Unenhanced CT should be used only if MR findings are normal, to exclude the presence of intracranial calcifications.     

The Sturge-Weber syndrome: comparison of MR and CT characteristics

Four patients with Sturge-Weber syndrome were evaluated with CT and MR. MR demonstrated the characteristic features of the disease: cerebral atrophy (four patients), ipsilateral bone and sinus hypertrophy (three), ocular findings (one), intracranial calcification (four), prominent deep venous system (three), and enlarged choroid plexus (two). CT demonstrated the following: cerebral atrophy (four), ipsilateral bone and sinus hypertrophy (three), calcification (four), gyral enhancement (two), prominent deep venous system (two), and enlarged choroid plexuses (three). The features of Sturge-Weber syndrome were visualized equally well with MR and CT with the exception of intracranial calcification. Conventional spin-echo MR revealed fewer calcifications, and those visualized appeared smaller than with CT. Gradient-echo acquisition sequences were more effective in the detection of intracranial calcification.     

Resective surgery for intractable epilepsy in children: radiologic evaluation.

Epilepsy surgery is gaining popularity for the treatment of children with intractable seizures in whom either a focal or extensive unilateral structural brain lesion is demonstrated. We evaluated the pre- and postoperative imaging findings in 29 patients (aged 22 days to 19 years) who underwent hemispherectomies, 12 total and 17 subtotal. Pathologic correlation was obtained in all cases. Preoperatively, positron emission tomography and electroencephalography demonstrated abnormalities in all of the 28 children studied, but frequently could not characterize the lesion. CT or MR or both demonstrated focal or unilateral lesions in only 19 of these but gave additional information regarding the nature of the lesion. Preoperative angiographic findings were abnormal in five of 17 patients studied and were particularly useful in the evaluation of the extent of abnormality in patients with Sturge-Weber syndrome. Postoperatively, CT and MR demonstrated early complications such as the development of epidural blood and fluid collections, parenchymal hemorrhage, infection, and early hydrocephalus. Postoperatively, MR demonstrated the early development of septations, the presence of subarachnoid hemorrhage, and/or the deposition of hemosiderin in four patients, findings that historically have been associated with the development of devastating clinical complications. From these data, a recommended protocol of radiologic evaluation for patients undergoing hemispherectomy has been established.     

Abnormal ocular enhancement in Sturge-Weber syndrome: correlation of ocular MR and CT findings with clinical and intracranial imaging findings.

PURPOSETo estimate the prevalence of abnormal ocular enhancement in children with Sturge-Weber syndrome as detected with MR imaging and CT and to correlate this with the clinical, fundoscopic, and intracranial imaging findings.METHODSFifteen children, 4 years old or younger, with Sturge-Weber syndrome were examined with enhanced CT and MR imaging. Eleven children had unilateral intracranial involvement and 4 had bilateral involvement, for a total of 19 abnormal hemispheres and related orbits. The presence of ocular enhancement was compared with the fundoscopic findings independently. Ocular enhancement was correlated with the extent of leptomeningeal disease, the severity of the cutaneous lesion, and the presence of glaucoma by the calculation of likelihood ratios and 95% confidence limits.RESULTSSeven of the 15 patients had abnormal ocular enhancement, which was present in 10 (53%) of the eyes associated with the 19 abnormal hemispheres. MR imaging showed choroidal hemangioma in 7 of 8 patients in whom hemangiomas were shown at fundoscopy. The likelihood of ocular enhancement was increased with the presence of bilateral disease, extensive facial nevi, and glaucoma; there was no significant correlation with the extent of hemispheric involvement.CONCLUSIONBoth enhanced MR imaging and CT can show diffuse choroidal hemangioma in patients with Sturge-Weber syndrome. However, MR imaging is more sensitive and is recommended to aid in the detection of abnormalities with preventable late complications.     

Choroid plexus size in young children with Sturge-Weber syndrome.

PURPOSETo assess the size of the choroid plexus in young children with unilateral and bilateral Sturge-Weber syndrome.METHODSSubjects included 15 children 4 years old or younger with Sturge-Weber syndrome. Eleven cases were unilateral and four were bilateral. Unilateral or bilateral involvement was determined by the distribution of abnormal leptomeningeal enhancement on MR images. The diameters of the choroid plexus were measured on contrast-enhanced axial MR images. The choroid plexus of the affected and unaffected sides in these cases were compared with those of 15 age-matched children without Sturge-Weber syndrome who were being examined for seizures.RESULTSOur results show a wide variation in the size of the choroid plexus in children with Sturge-Weber syndrome; however, plexus associated with a hemisphere affected by Sturge-Weber syndrome were significantly larger than those on the unaffected side and in the age-matched control group. The size of the choroid plexus was positively correlated with the extent of leptomeningeal involvement as demonstrated by abnormal contrast enhancement.CONCLUSIONThe choroid plexus is enlarged early in the course of Sturge-Weber syndrome in both unilateral and bilateral cases. There is a positive correlation between choroid plexus size and extent of leptomeningeal involvement in children with Sturge-Weber syndrome.     

MR imaging of Sturge-Weber syndrome: role of gadopentetate dimeglumine and gradient-echo techniques

Five patients with Sturge-Weber syndrome were evaluated by conventional noncontrast spin-echo MR imaging, a gradient-recalled echo (GRE) technique, and T1-weighted spin-echo imaging after administration of gadopentetate dimeglumine. In four of five cases the full extent of intracranial disease was appreciated only on the postcontrast images. In one patient precontrast and GRE images were entirely normal, while only the postcontrast study demonstrated extensive involvement of both brain and retina. Nevertheless, some abnormal vessels with higher flows were seen better on precontrast T2-weighted images than on postcontrast T1-weighted images. GRE techniques demonstrated calcifications to best advantage, in one case even better than on CT. Contrast enhancement with gadopentetate dimeglumine is necessary for the complete MR evaluation of patients with suspected Sturge-Weber syndrome. Traditional noncontrast T2-weighted and GRE images may provide additional complementary information.     

新生儿和婴儿期Sturge-Weber综合征的CT诊断

目的:探讨新生儿和婴儿期Sturge-Weber综合征的CT表现。方法:回顾性分析1例新生儿期和7例婴儿期Sturge-Weber综合征患儿的临床和CT资料,均行常规CT平扫,其中5例同时行增强CT。结果:8例均有面部三叉神经分布区皮肤血管瘤,4例患儿出现患侧皮层钙化,4例未出现皮层钙化的患儿显示受累脑实质密度略增高,伴灰白质分界不清。8例患儿均表现为局部脑外间隙增宽及脑沟增宽等脑萎缩样改变,3例随访患儿出现脑萎缩。5例行增强扫描的患儿出现脑回样强化,4例患侧的侧脑室脉络丛增大,1例双侧Sturge-Weber综合征于10个月随访时出现双侧脉络丛增大。结论:CT对新生儿和婴儿期Sturge-Weber综合征的诊断有重要价值。     

Cranial CT and MR in the Klippel-Trenaunay-Weber syndrome.

This report describes the intracranial CT and MR findings in two cases of Klippel-Trenaunay-Weber Syndrome. The findings are 1) markedly enhancing choroid plexuses, 2) severe cerebral atrophy, 3) cerebral calcifications, and 4) angiomatous leptomeningeal enhancement. The findings may resemble those seen in cases of bilateral Sturge-Weber syndrome. The two diseases should be distinguishable by the external stigmata. The authors raise the question of a spectrum of involvement in the angiodysplasias of Klippel-Trenaunay-Weber syndrome and Sturge-Weber syndrome with considerable overlap.     

Computed tomography in cerebral hemiatrophy.

The clinical and radiographic findings in four cases of cerebral hemiatrophy are presented. CT findings reflect the underlying gross pathologic changes and are in agreement with those seen on plain skull radiography and pneumoencephalography. The most impressive finding was unilateral loss of cerebral volume with ipsilateral displacement of the midline structures. The differential diagnosis primarily includes Sturge-Weber syndrome and linear sebaceous nevus syndrome. The recognition of compensatory calvarial changes should indicate that the cerebral abnormalities are the result of an atrophic or hypoplastic process that began in early life.     

Diffusion tensor imaging and magnetic resonance spectroscopy of the brain in a patient with Sturge-Weber syndrome

Quantitative brain MR spectroscopy (MRS) and diffusion tensor imaging (DTI) were used to characterize one patient with Sturge-Weber syndrome. Choline increases and N-acetylaspartate decreases were observed in pathologic frontal gray matter tissue compared to contralateral unaffected brain tissue without any change in the diffusion tensor imaging parameters (fractional anisotropy, apparent diffusion coefficient). The N-acetylaspartate decreases and/or choline increases observed here and in eight previously described Sturge-Weber patients probably reflect neuronal loss or dysfunction and demyelination as a result of recurrent seizures.     

脑颜面血管瘤综合征的CT表现(附8例报告)

目的 结合病理分析脑颜面血管瘤综合征的CT特点。资料与方法回顾分析8例脑颜面血管瘤综合征的临床和cT资料。结果脑颜面血管瘤综合征8例CT影像均有特征性表现，为一侧或两侧顶枕叶或额顶颞枕叶表浅部位的脑回状或弯曲条带状钙化伴病侧大脑半球发育不全或萎缩。同时合并脑软化灶者2例，合并同侧颅骨增厚者2例。结论 CT能够很好地显示脑颜面血管瘤综合征的宽大脑回状及弯曲条带状钙化的特征。诊断具有特征性。     

Asymmetrical hemithorax volume loss due to Wegener's granulomatosis

Three patients with Wegener's granulomatosis (WG) established by clinical, serological and histological criteria were noted to have marked asymmetrical hemithorax volume loss on thoracic CT. Lung dimensions were analysed from the CT in each case. Evidence of airways disease, parenchymal abnormalities and pleural changes was evaluated on CT, in order to establish the aetiology of the volume loss. Previous pulmonary infection and thoracic intervention were excluded by the clinical data. The three patients had chronic treated thoracic WG for 1-9 years. There was severe asymmetrical pleural disease in one case and parenchymal disease with evidence of fibrotic healing but no evidence of bronchial disease in two cases. Marked asymmetrical volume loss of a hemithorax is a previously unreported finding and should be added to the features of primary chronic thoracic WG. This finding does not require investigation for additional pathology.     

Head injury: early results of comparing CT and high-field MR

The sensitivity and specificity of CT and high-field MR (1.5 T) were compared in an evaluation of 30 patients with head injuries (eight acute, 15 subacute, and seven chronic). By using T1- and T2-weighted images, it was possible to detect various stages of hemorrhages and their separation from edema. In the acute category, both CT and MR showed acute hemorrhagic lesions, but only MR demonstrated coexisting chronic hematomas or small hypothalamic or brainstem infarctions. MR was far superior to CT in the detection and characterization of subacute injuries, including shearing injuries, hemorrhagic and nonhemorrhagic contusions, and subdural hematomas. In chronic injury, atrophy was demonstrated by both techniques, but only MR showed parenchymal abnormalities and old hemorrhages. Its ease in monitoring patients and its greater speed make CT the procedure of choice for the evaluation of acute cases. CT also provides information that is useful in deciding between surgery and medical management. However, the more precise anatomic depiction of MR and its sensitivity to parenchymal abnormalities make MR the key to correct prognosis in patients with subacute or chronic injury.     

Sturge-Weber-Syndrom

BACKGROUND: The purpose of this study was to evaluate ultrasound, MRI, and CT investigations in children with Sturge-Weber syndrome. PATIENTS AND METHODS: From 1996 to 2005, a total of 15 investigations of the brain performed in 6 children with Sturge-Weber syndrome were reviewed. We evaluated four ultrasound, five CT, and six MRI exams. RESULTS: With ultrasound an increase of the echogenicity of the periventricular white matter was depicted in the first days of life. MRI is the best modality to demonstrate the vascular malformation, the impaired cerebral venous drainage, and the atrophy of one hemisphere. CONCLUSIONS: One-sided periventricular hyperechogenicity on ultrasound can be an early sign of Sturge-Weber syndrome in children with a nevus flammeus. MRI is the method of choice to diagnose Sturge-Weber syndrome and to follow up these children if the neurological status of the patients changes.     

MR of craniocerebral hemiatrophy.

The magnetic resonance (MR) findings of three patients with cerebral hemiatrophy, the so-called Dyke-Davidoff-Masson syndrome, which is characterized by variable degrees of unilateral loss of cerebral volume and compensatory changes of the calvarium are presented. The condition was due to middle cerebral artery stroke in all patients. The pathologic alterations of cerebral tissue and the brainstem were reflected in detail on the MR studies. MR findings in addition to the primary vascular insult included prominence of the cortical sulci and perimesencephalic cistern in one subject with acquired infarction, but an absence of such generalized sulcal prominence in two cases of congenital infantile paralysis. Otherwise the secondary ipsilateral pathologic observations were quite similar in the patients with congenital and acquired ischemic disease and encompassed a unilaterally small cerebral hemisphere together with ipsilateral diploic calvarial expansion, elevation of the petrous bone and orbital roof, and hypoplasia/atrophy of the cerebral peduncle. Although computed tomography (CT) and MR are complimentary, it is felt that MR represents the imaging procedure of choice with respect to the assessment of the etiology and extent of cerebral parenchymal involvement in patients presenting with a clinical combination of congenital or early onset of seizures, hemiparesis/plegia, and/or craniofacial asymmetry.     

CT sign of brain swelling without concomitant parenchymal hypoattenuation: comparison with diffusion- and perfusion-weighted MR imaging

PURPOSE: To retrospectively evaluate the apparent diffusion coefficient (ADC) on magnetic resonance (MR) images and the perfusion parameters of lesions that show brain swelling without concomitant parenchymal hypoattenuation on computed tomographic (CT) scans. MATERIALS AND METHODS: Review board approval was obtained, and informed consent was waived. A total of 14 patients (seven men and seven women; mean age, 64 years +/- 11) were retrospectively selected from the consecutive 172 patients with acute cerebral ischemia who underwent CT within 6 hours of symptom onset. All patients had brain swelling without parenchymal hypoattenuation, including loss of gray-white matter distinction on CT scans, and they underwent diffusion- and perfusion-weighted MR imaging shortly after CT. CT attenuation, ADC, and perfusion parameters of relative cerebral blood volume (CBV), time to peak (TTP), and relative cerebral blood flow (CBF) were calculated for gray and white matter of the lesion. The measured values were compared with those of the contralateral hemisphere by using the paired t test; comparison of values of perfusion parameters among three subgroups was performed with the Kruskal-Wallis test. Arterial occlusions were determined with MR angiography or conventional angiography. RESULTS: The mean interval between initial CT and MR imaging was 2.4 hours +/- 0.9 (range, 0.4-3.4 hours). The ADC of lesions was similar to that of contralateral normal tissue (mean ADC ratio for gray matter and white matter, 0.99 and 0.97, respectively) (P > .05). Lesions had an increased relative CBV (P < .001), a mild to moderate TTP delay (P < .001), and a variable but not statistically significant reduction of relative CBF. The mean relative CBF of gray matter was less in patients who had complete infarction (0.81 +/- 0.16) than that in patients with partial infarction (0.99 +/- 0.16) or those with a normal radiologic outcome (1.12 +/- 0.22), but this difference was not statistically significant (P > .05). Proximal cerebral artery occlusions were found in all patients. In five (36%) patients, the lesion did not progress to infarction at follow-up. CONCLUSION: The CT sign of brain swelling without concomitant parenchymal hypoattenuation in patients with acute cerebral ischemia does not represent severe ischemic damage and may suggest ischemic penumbral or oligemic tissue.     

CT and MR of angiomatous malformations of the choroid plexus in patients with Sturge-Weber disease

Eight patients with Sturge-Weber disease were evaluated by CT (six patients), MR (one patient), or both (one patient). CT scans of five of seven patients showed enlargement and increased enhancement of the choroid plexus on the same side as the facial and intracranial lesions. MRI showed similar findings in both patients examined. This enlargement, seen in six of eight cases of Sturge-Weber disease, is compatible with the presence of angiomatous malformations of the choroid plexus. It appears to be a common finding in this disease.     

Glutaric aciduria type I: value of diffusion-weighted magnetic resonance imaging for diagnosing acute striatal necrosis

Glutaric aciduria type I is a rare disorder of organic acid metabolism caused by deficiency of glutaryl-CoA dehydrogenase. We report the cranial computed tomography (CT) and magnetic resonance (MR) imaging findings in a 5-month-old girl with this disorder who presented with an acute dystonic syndrome. CT findings demonstrated only subtle loss of attenuation in the basal ganglia, MR spectroscopy was normal, and conventional MR images showed increased T2-signal limited to the putamina. Diffusion-weighted MR imaging demonstrated more extensive disease than was apparent either on CT or on the conventional MR images, including bilateral involvement of the putamina, globus pallidus, and caudate nuclei, consistent with acute necrosis of the corpus striatum and lentiform nuclei.     

Sturge-Weber综合征的影像学诊断(附11例报告及文献复习)

目的　探讨Sturge -Weber综合征的影像学表现。方法　收集 11例Sturge -Weber综合征的临床及影像学资料 ,10例有面部三叉神经分布区皮肤血管瘤。所有病例都进行了常规CT扫描 ,其中头颅X线平片 2例 ,进行MR检查 8例 ,增强CT检查 5例 ,MRI增强 3例。结果　患侧皮层钙化 11例 ;脑萎缩 9例 ,受累脑叶灰白质分界不清 ;侧脑室脉络丛增大及深静脉血管扩张 7例 ;一侧颅板板障增厚 8例 ;增强脉络丛明显强化并呈囊状扩大 5例 ,患侧脑回状强化及脑表面血管扩张 6例。结论　头颅平片诊断本病作用有限 ,CT和MRI综合分析是确诊本病和判断病情最有价值的影像学方法     

MR imaging of neurocysticercosis

Magnetic resonance (MR) was performed in 50 patients with neurocysticercosis. Comparison was made with other neuroradiological imaging modalities including CT, myelography, CT ventriculography, and CT myelocisternography. Eighteen patients were found to have intraventricular cysts. In several patients, these were multiple and 22 intraventricular cysts were discovered. Although 4 of the 22 ventricular cysts were missed by MR, T1-weighted images can play a significant role in the early detection of intraventricular cysticercosis cysts, showing the cyst wall (9 of 22), a high intensity mural nodule (6 of 22), and increased signal intensity of the cyst fluid (5 of 22). Cisternal cysts (14 cysts in 10 patients) could be identified; they appear similar to intraventricular cysts, but mural nodules are infrequently seen (1 of 14). Twenty-nine patients had 69 parenchymal cysts. An attempt was made to assess the viability of these parenchymal lesions by matching the CT and MR findings with the Escobar pathologic staging system. Neuroimaging findings seemed compatible with early parenchymal lesions in the vesicular stage in 11 instances. Findings in cases with later stage cysts tend to support the concept that a dying larva provokes pronounced inflammatory reaction in the adjacent brain. Computed tomography remains the superior modality for depicting parenchymal calcifications within dead larvae. A case of a spinal cysticercosis cyst demonstrated with MR (in a patient with extensive intracranial cisternal cysts and a fourth ventricular cyst) is described.