Computed tomography and magnetic resonance imaging of the brain in Hurler's disease

Computed tomographic (CT) and magnetic resonance imaging (MRI) scans of the brain in five patients with Hurler's disease are described and compared to the few available reports in the literature. Computed tomographic scans revealed low attenuation areas in the centrum semiovale and peritrigonal white matter. Ventriculomegaly was not a prominent feature in our patients, compared to those previously reported. In two patients, CT were normal. The most prominent magnetic resonance imaging abnormalities were the presence of radially oriented cystic areas in the centrum semiovale, peritrigonal white matter, corpus callosum, and pericallosal region. Magnetic resonance imaging abnormalities were present in all patients, even when CT scans were normal. Abnormalities on CT and MRI scans tended to be more prevalent in the posterior regions. Magnetic resonance imaging proved to be a more reliable imaging method in Hurler's disease. T1-weighted images delineated the cystic areas more clearly, whereas T2-weighted images were more sensitive in detecting small white-matter abnormalities. Magnetic resonance imaging abnormalities correlated well with known neuropathologic alteration in this disease. It is suggested that the cystic areas seen on MRI correspond to perivascular lacunae seen in histopathologic material.     

Computed tomography and magnetic resonance imaging in adult-onset leukodystrophy

Five clinically affected and nine at-risk members of a kindred with an autosomal dominant adult-onset leukodystrophy simulating chronic progressive multiple sclerosis were studied with computed tomography (CT) and magnetic resonance imaging (MRI). Computed tomographic scans showed white matter lucencies occurring earliest and most prominently in the frontoparietal region. The lesions were nondiscrete, diffuse, and bilaterally symmetric. These changes were more clearly visualized as areas of increased signal intensity with T2-weighted MRI. Magnetic resonance imaging also showed increased signal intensity in the brain stem, cerebellar white matter, or both of four patients. Both MRI and CT differentiated this entity from multiple sclerosis, but MRI was superior to CT in detailing the extent of white matter involvement.     

Three-dimensional computed tomography angiography and magnetic resonance angiography of carotid bifurcation stenosis.

PURPOSE: To evaluate the role of computed tomography angiography (CTA) and magnetic resonance angiography (MRA) in the quantification of atherosclerotic stenosis of carotid artery bifurcation in comparison with digital substraction angiography (DSA) and Doppler sonography (DS). MATERIALS AND METHODS: Twenty-five patients with atherosclerotic disease of the carotid arteries with proven stenosis by DSA, had spiral CTA, MRA using two- and three-dimensional time-of-flight gradient echo techniques, and DS using Doppler flow signal recording (total 47 carotid artery bifurcations). The degree of stenosis was measured according to the North American Symptomatic Carotid Endarterectomy Trial criteria: total occlusion (100%), severe (70-99%), moderate (30-69%) and mild (0-29%). The degree of stenosis measured by CTA, MRA and DS was compared to DSA, used as the gold standard. RESULTS: Ninety-seven percent of MRA measures were equivalent to DSA, and 3% were underestimated; 96% of CTA measures were equivalent to DSA, and 4% were underestimated; 77% of DS measures were equivalent to DSA, 21% were overestimated and 2% were underestimated. CONCLUSIONS: CTA and MRA are equally accurate methods in quantifying the degree of carotid bifurcation stenosis.     

Computed tomography and magnetic resonance imaging in late-onset globoid cell leukodystrophy (Krabbe disease)

A five-year-old white male presented with a history of progressive loss of vision that was subsequently followed by progressive corticospinal dysfunction. Evaluation revealed the presence of leukodystrophy which was confirmed by a deficiency of the enzyme, galactosylceramide beta-galactosidase. We present the clinical, computed tomographic, and magnetic resonance imaging features of this late-onset form of globoid cell leukodystrophy.     

Computed tomography, magnetic resonance imaging and pathological correlations in a case of Binswanger's disease

The results of 3 computed tomography (CT) examinations carried out over a 7 year period and of a post-mortem magnetic resonance (MR) study showed aspects of a white matter disease in a hypertensive patient suffering from vascular dementia. Histopathology revealed the primary cause of dementia to be a white matter degeneration sparing the U fibers. Rarefaction of both the myelin sheaths and the axons was present together with severe thickening of the medullary arteries. These findings support the existence of Binswanger's disease (BD) as a distinct variety of arteriosclerotic dementia. CT and MR imaging are valuable aids for diagnosis. However, since there are many other causes of CT and MR demonstrated diffuse white matter degeneration in the elderly, a conclusive diagnosis of BD requires pathological confirmation.     

Comparative performance of magnetic resonance angiography and conventional angiography in moyamoya disease.

Patients with moyamoya disease verified by conventional cerebral angiography (CCA) were evaluated by means of magnetic resonance angiography (MRA), in order to clarify its advantages and limitations in comparison with CCA. This retrospective study was carried out in 13 hemispheres of seven patients with moyamoya disease (including one atypical patient). In MRA, magnetisation transfer contrast (MTC) and maximal intensity projection (MIP) techniques were used. A good correspondence between MRA and CCA was noted in 11 hemispheres (86.4%) on evaluating stenotic lesions and overestimation in the remaining 2. Underestimation of moyamoya vessels in MRA was noted in 3 hemispheres, since well developed moyamoya vessels were detectable, while poorly developed ones were not. No difference between MRA and CCA findings was found in 10 hemispheres (76.9%). MRA tends to overestimate the stenosis and underestimate moyamoya vessels. These characteristics may lead to wrong classification in the angiographic grade of patients with early and advanced stages and should be taken into consideration in interpreting MRA images of moyamoya disease (compatible rate of grade between MRA and CCA; 84.6%). In conclusion MRA with MTC and MIP techniques offers an acceptable quality of assessment of stenotic lesions and moyamoya vessels. MRA is a useful follow up method at present and in the near future it may replace CCA as the initial diagnostic tool.     

Neuromaging techniques in cerebrovascular disease: Computed tomography angiography/computed tomography perfusion

Computed tomography angiography (CTA) and computed tomography perfusion (CTP) describe contrast-enhanced techniques, which yield anatomic and functional data about the cerebral vasculature and tissue perfusion. The resulting information helps in the management of patients with cerebrovascular disease (CVD), whether it is chronic atherosclerosis or acute ischemic stroke. CTA and CTP are used to diagnose and grade atherosclerotic disease, to assess the individual patient's risk for development of acute stroke, and in a suspected acute ischemia they help confirm the diagnosis and possibly in stratifying patients for thrombolytic treatment. The information they provide is based on direct evaluation of the major arteries and the perfused lumen, on the morphology of an underlying atheroma, and on the assssment of tissue perfusion and collateral flow.     

Computed tomography and magnetic resonance imaging of the brain in hypoglycaemic encephalopathy

A diabetic man suffering from a hypoglycaemic attack presented to our hospital. Magnetic resonance imaging (MRI) findings were grossly abnormal and difficult to interpret. The computed tomographic appearance simulated multiple infarctions. The MRI appearance simulated a space occupying lesion. Literature review of the radiology and the pathophysiology of hypoglycaemic brain damage is provided. Comparison between our findings and previous reports is also given.     

颈部血管彩超联合头颅磁共振血管造影在缺血性脑血管病中的诊断价值

目的：探讨颈部血管彩超联合头颅磁共振造影（MRA）对缺血性脑血管病（ICVD）的诊断价值。方法选取脑血栓形成患者92例（A组）,短暂性脑缺血发作（T IA ）患者37例（B组）及健康体检者50例作为对照组（C组）,分别采用颈部血管彩超诊断;92例脑血栓形成患者中80例同时行头颅M RA检查。比较不同人群动脉粥样硬化的发生率及病变特点,比较两种诊断方法对颈内动脉闭塞诊断的吻合程度。结果颈部血管彩超检查结果表明,A、B组间粥样斑块形成、IM T厚度及软斑形成比较差异无统计学意义（P＞0.05）,与C组比较差异均有统计学意义（P＜0.05）;3组颈动脉粥样斑块分布比较差异无统计学意义（P＞0.05）。颈部血管彩超与头颅MRA对颈内动脉闭塞诊断的吻合率87.50％,对椎动脉闭塞诊断的吻合率75.00％,对轻度狭窄、中度狭窄及重度狭窄诊断的吻合率分别92.86％、94.12％及85.71％。结论颈部血管彩超可为ICVD病变患者颈动脉粥样硬化斑块的形成部位、管腔狭窄程度提供诊断依据,头颅M RA可进一步对颅内血管病变进行诊断,两者联合可更全面评价ICVD病变情况,为临床诊治提供参考。     

Brain tumors in children with neurofibromatosis: Computed tomography and magnetic resonance imaging

Comparisons were made between the results of computed tomography and magnetic resonance imaging (MRI) evaluations in 13 patients with neurofibromatosis. In 8 of 9 patients with intracranial tumors and in 2 of 4 patients without intracranial tumors additional findings were demonstrated by MRI.     

Noninvasive angiography (magnetic resonance and computed tomography) in the diagnosis of ischemic cerebrovascular disease. Techniques and clinical applications

Noninvasive diagnostic imaging of the craniocervical and intracranial vasculature is a domain of computed tomography angiography (CTA), magnetic resonance angiography (MRA) and Doppler/duplex ultrasound, the latter not being the topic of this presentation. We give a methodological background for both, CTA and MRA, followed by a critical appraisal of both imaging modalities in the diagnosis of ischemic cerebrovascular disease. The contribution of noninvasive vascular imaging to vascular malformations (including aneurysms, fistulas and cerebral-vein thrombosis) is beyond the scope of this paper and therefore not covered.     

Computed tomography of Sturge-Weber disease.

Findings in computed tomography (CT) with or without contrast infusion were analyzed in 8 patients with Sturge-Weber disease. From the extent of calcification in the CT, Sturge-Weber disease can be classified into two types, localized and diffuse. The extent of calcification or cortical atrophy is predictable, if it can be shown that enhanced areas in contrast infusion CT truly represent leptomeningeal angiomatosis. Thus, using enhanced CT, the capacity of prediciton as to whether the patient will have a localized or diffuse type of disease will be possible with further observations.     

Novel magnetic resonance angiography stage grading for moyamoya disease

BACKGROUND: Magnetic resonance angiography (MRA) has been acknowledged as a noninvasive diagnostic modality for moyamoya disease. However, in terms of staging of moyamoya disease, conventional angiography is still the gold standard. Therefore, the purpose of this study was to establish MRA grades for moyamoya disease as an alternative to conventional angiography. METHODS: Twenty-two patients (44 sides) with moyamoya disease diagnosed by conventional angiography were evaluated by MRA during the past 5 years. MRA scores were assigned based on the severity of occlusive changes of the internal carotid artery, the horizontal portion of the middle cerebral artery, the anterior and the posterior cerebral arteries and the signals of the distal branches of these arteries. Total points ranged from 0 (normal) to 10 (most severe). RESULTS: MRA scores (0-10) were significantly consistent with the conventional angiographic staging. Four grades based on this novel MRA scores correlated well with Suzuki's stages, with high sensitivity and specificity. CONCLUSIONS: These novel MRA grades can be a reliable alternative to conventional staging. By employing these novel MRA grades, the use of conventional angiography can be avoided for the purpose of evaluation of the stages of moyamoya disease.     

Integration of digital angiography, magnetic resonance, x-ray computed tomography and positron emission tomography in stereotaxy

Consecutively to drastic changes which occurred in cerebral imagery techniques, we have developed a stereotactic apparatus and system based on the integration of several new techniques allowing visualisation of the brain: tomodensitometry (TDM), digital subtraction angiography (DSA), magnetic resonance (MR) and positron emission tomography (PET). TDM, DSA and MR can be performed in stereotactic conditions with the apparatus in situ. They give the computer the anatomic references necessary for all calculations. MR and PET images obtained without stereotactic apparatus can also be integrated into the stereotactic study at the condition that DSA was formerly performed in stereotactic conditions, i.e. with the apparatus in situ. The visualisation of the corpus callosum makes this integration possible. An optimal definition of cerebral tumors or target-structures for intracerebral electrode recording is thus obtained.     

Computed tomography in Alexander's disease

Computed tomography demonstrated contrast-enhancing lesions in the periventricular frontal regions, caudate nuclei, and thalami in an infant with Alexander's disease. The distribution of the enhancing lesions corresponded to the areas in which Rosenthal fibers were most prominent. These radiological findings have not been described in other white matter diseases; thus, they may help to distinguish Alexander's disease from Canavan's disease and decrease the necessity for diagnostic brain biopsy.