Brain MRI changes in chronic liver disease

Cirrhotic patients are known to have abnormally high signal principally in the globus pallidus on non-contrast T1-weighted MRI. The purpose of this study was to relate MR changes to clinical and pathological features of chronic liver disease. We confirmed abnormally high signal in the globus pallidus on T1-weighted images in 25 of 28 patients with chronic liver disease, showing that it also occurs in patients who have not yet progressed to cirrhosis. Changes were seen in patients both with and without clinical portosystemic shunting. This abnormality is not responsible for hepatic encephalopathy. Cholestatic disease was more likely to produce marked changes than non-cholestatic disease. No statistically significant correlation was demonstrated between the severity of liver disease and the degree of MR abnormality. However, marked improvement in MR appearances was seen after successful liver transplantation. Received: 23 February 1996; Revised version received: 29 July 1996; Accepted: 5 November 1996     

Chronic acquired hepatic failure: MR imaging of the brain at 1.5 T

The results of MR imaging of the brain at 1.5 T in 42 adults with non-Wilsonian chronic hepatic failure are reported. T1-weighted images demonstrated increased signal in the globus pallidus in 30 patients and in the putamen in 21, while T2-weighted images demonstrated no corresponding alteration in signal intensity. Symmetric low intensity in the central portion of the globus pallidus on spin-density and T2-weighted images in two patients correlated with regions of calcification on CT scans. Increased intensity on T1-weighted images also occurred in the mesencephalon surrounding the red nucleus (17/42) and in the quadrigeminal plate (4/42). Three patients demonstrated increased intensity in the pons on T2-weighted images unassociated with clinical brainstem dysfunction. Increased intensity on T1-weighted images was seen in the anterior pituitary in 28 of 35 patients. Alterations in signal intensity were not demonstrated in the cerebral cortex or cerebellum. MR findings did not correlate with laboratory indices of hepatic or thyroid function, with histologic liver diagnosis, or with neurologic status at the time of MR evaluation. Increased signal intensity in the basal ganglia, pituitary gland, and mesencephalon surrounding the red nuclei is characteristic of chronic hepatocellular dysfunction. Deposition of an as yet unidentified paramagnetic substance or altered intracellular water relaxation associated with the proliferation of astrocyte cytoplasmic organelles is postulated as the likely mechanism for this previously undescribed MR manifestation of chronic acquired hepatic failure.     

High-intensity basal ganglia lesions on T1-weighted images in two toddlers with elevated blood manganese with portosystemic shunts

We report two toddlers with portosystemic shunts who had symmetrical high-signal globus pallidus lesions on T1- but not T2-weighted MRI, and measurement of whole blood manganese at 2 years of age. These cases suggest that portosystemic shunts can cause elevation of blood manganese and result in manganese accumulation in the globus pallidus, causing high signal on T1-weighted images even in asymptomatic toddlers. Received: 13 April 1998 Accepted: 23 July 1998     

High-signal basal ganglia on T1-weighted images in a patient with Sydenham's chorea

We report a 16-year-old girl with Sydenham's chorea. Choreiform movements involved both sides of her body. MRI 2 months after the onset revealed abnormal increased signal on T2-weighted images and enlargement of the caudate and putamen bilaterally. MRI 5 months later showed resolution of the swelling, but with increased signal on T1-weighted images in the putamen, globus pallidus and the head of the caudate nucleus bilaterally, with slightly increased signal intensity on T2-weighted images. Received: 19 January 1998 Accepted: 19 January 1998     

Contribution of metals to brain MR signal intensity: review articles

Various metals are essential nutrients in humans, and metal shortages lead to a variety of deficiency diseases. Metal concentration abnormalities may cause metal deposition in the brain, and magnetic resonance imaging (MRI) is the most potent and sensitive technique now available for detecting metal deposition given the difficulties associated with performing brain tissue biopsy. However, the brain contains many kinds of metals that affect the signal intensity of MRI, which has led to numerous misunderstandings in the history of metal analysis. We reviewed the history of brain metal analysis with histologic findings. Typically, manganese overload causes high signal intensity on T1-weighted images (T1WI) in the globus pallidus, iron overload causes low signal intensity in the globus pallidus on T2-weighted images, and gadolinium deposition causes high signal intensity in the dentate nucleus, globus pallidus, and pulvinar of thalamus on T1WI. However, because nonparamagnetic materials and other coexisting metals also affect the signal intensity of brain MRI, the quantitative analysis of metal concentrations is difficult. Thus, when analyzing metal deposition using MRI, caution should be exercised when interpreting the validity and reliability of the obtained data.     

Parkinson plus syndrome: diagnosis using high field MR imaging of brain iron

The distribution of iron in the brain was analyzed using high field strength (1.5 T) magnetic resonance (MR) imaging in 14 healthy control individuals and six patients with Parkinson plus syndromes (multisystem atrophy and progressive supranuclear palsy) who were unresponsive to antiparkinsonian therapy. The normal topographic distribution of iron in the brain as indicated by high field MR images coincided precisely with the distribution of iron in the brain as determined by Perls staining for ferric iron. In Parkinson plus syndromes, there were abnormally increased concentrations of iron (decreased T2 relaxation times) in the putamen, and less prominent increases in the caudate nucleus and lateral pars compacta of the substantia nigra. In high field strength MR images of normal patients, the decreased signal intensity in the globus pallidus is more prominent than that of the putamen. In MR images of patients with Parkinson plus syndromes, the decreased signal intensity of the putamen is more prominent than that of the globus pallidus.     

Magnetization transfer contrast of various regions of the brain in liver cirrhosis.

BACKGROUND AND PURPOSE: T1-weighted MR images show high signal intensity in the pallidum of many patients with liver cirrhosis. The purpose of this study was to evaluate quantitative changes in MR signals in patients with liver cirrhosis by using the magnetization transfer technique. METHODS: Magnetization transfer ratios were measured in seven different regions of the brain in 37 patients with liver cirrhosis and in 37 healthy volunteers. RESULTS: The magnetization transfer ratios in patients with liver cirrhosis were significantly lower than those in control subjects in the globus pallidus, putamen, thalamus, corona radiata, and subcortical white matter. CONCLUSION: Abnormal magnetization transfer ratios may be found in otherwise normal-appearing cerebral regions.     

Atypical MRI features of Wilson's disease: high signal in globus pallidus on T1-weighted images

Most reports of MRI in Wilson's disease have been of abnormal low-signal lesions on T1-weighted images and high signal intensity on T2-weighted images. In contrast, we report three patients who had high-signal lesions in the globus pallidus on T1-weighted images, a finding seen in patients with portal-systemic encephalopathy. The possible causes include the paramagnetic effect of copper or iron and accumulation of Alzheimer type II glial cells. Received: 26 January 1996 Accepted: 1 April 1996     

Reduced signal intensity on MR images of thalamus and putamen in multiple sclerosis: increased iron content?

High-field-strength (1.5-T) MR imaging was used to evaluate 47 patients with definite multiple sclerosis and 42 neurologically normal control patients. Abnormal, multiple foci of increased signal intensity on T2-weighted images, most prominent in the periventricular white matter, were apparent in 43 of 47 MS patients and in two of 42 control patients. A previously undescribed finding of relatively decreased signal intensity most evident in the putamen and thalamus on T2-weighted images was seen in 25 of 42 MS patients and correlated with the degree of white-matter abnormality. In the normal control patients a prominently decreased signal intensity was noted in the globus pallidus, as compared with the putamen or thalamus, correlating closely with the distribution of ferric iron as determined in normal Perls'-stained autopsy brains. The decreased signal intensity (decreased T2) is due to ferritin, which causes local magnetic field inhomogeneities and is proportional to the square of the field strength. The decreased T2 in the thalamus and striatum in MS may be related to abnormally increased iron accumulation in these locales with the underlying mechanism remaining speculative.     

MR imaging of CNS involvement in children affected by chronic liver disease

BACKGROUND AND PURPOSE: MR imaging sheds new light on CNS involvement in the course of acquired chronic liver disease; however, the exact pathogenetic mechanisms of hepatic encephalopathy and associated MR abnormalities remain unclear. Our purpose was to relate MR signal intensity abnormalities of the CNS to clinical, biochemical, and pathologic features of childhood-onset chronic liver disease. METHODS: Twenty-one patients (12 male and nine female patients) were included in the study; two had Crigler-Najjar disease type 2, 17 had chronic liver disease of different causes, and two had idiopathic copper toxicosis. Twelve patients had histologically proved liver cirrhosis, with a median disease duration of 175 months at the time of MR study. None had clinical symptoms of hepatic encephalopathy. MR imaging was performed using spin-echo T1- and T2-weighted sequences. RESULTS: Eleven patients had abnormal MR imaging findings of the brain revealed by T1-weighted MR sequences; two of the 11 had idiopathic copper toxicosis. The affected sites were the hypothalamus and globus pallidus, presenting symmetrical and bilateral high signal intensities, or the pituitary gland, which appeared homogeneously hyperintense, or both findings. Eight of the 12 patients with cirrhosis had abnormal MR signals of the brain. In these, the median cirrhosis duration was shorter (169 months) than in the remaining four patients with normal MR signals (177 months). A significant correlation was found between abnormal MR signals of the brain and cirrhosis (P = .008) and factor V activity (P = .008). CONCLUSION: MR imaging confirms the presence of abnormal brain signals in the globus pallidus, hypothalamus, and pituitary gland in patients with childhood-onset liver disease in the absence of clinical symptoms of encephalopathy. Signal intensity abnormalities are likely caused by an as yet unidentified metabolic process partially correlated with the severity of liver disease.     

Manganese accumulation in the brain: MR imaging

Manganese (Mn) accumulation in the brain is detected as symmetrical high signal intensity in the globus pallidi on T1-weighted MR images without an abnormal signal on T2-weighted images. In this review, we present several cases of Mn accumulation in the brain due to acquired or congenital diseases of the abdomen including hepatic cirrhosis with a portosystemic shunt, congenital biliary atresia, primary biliary cirrhosis, congenital intrahepatic portosystemic shunt without liver dysfunction, Rendu-Osler-Weber syndrome with a diffuse intrahepatic portosystemic shunt, and patent ductus venosus. Other causes of Mn accumulation in the brain are Mn overload from total parenteral nutrition and welding-related Mn intoxication.     

A preliminary study revealing a new association in patients undergoing maintenance hemodialysis: manganism symptoms and T1 hyperintense changes in the basal ganglia

BACKGROUND AND PURPOSE: Patients undergoing parenteral nutrition and those with portosystemic encephalopathy secondary to chronic liver disease and acquired and congenital portosystemic venous shunts frequently present manganese deposition in the basal ganglia, detected by MR imaging as hyperintense areas on T1-weighted sequences. We also observed similar abnormalities in the basal ganglia of patients with chronic renal failure undergoing maintenance hemodialysis. Our aim was to evaluate the pallidal signal intensity on T1-weighted images in a series of patients undergoing hemodialysis, with further evaluation of serum manganese levels and neurologic correlation, comparing them with patients with chronic renal failure without dialytic treatment. MATERIALS AND METHODS: We performed MR imaging examinations in 9 patients with chronic renal failure, 5 of whom were undergoing hemodialysis. An experienced neuroradiologist scrutinized the presence of symmetric hyperintensities in the basal ganglia on T1-weighted sequences. We also determined the serum manganese levels and performed the neurologic evaluations in all patients. RESULTS: All patients undergoing hemodialysis presented elevated serum manganese levels and symmetric hyperintensities within the globus pallidus. In this group, 4 patients presented with parkinsonian symptoms, myoclonus, and syndromes with vestibular and vestibular-auditory symptoms. The patients without dialytic treatment presented with neither bilaterally increased T1 MR imaging signal intensity within the globus pallidus nor symptoms of manganism. CONCLUSION: Our preliminary results demonstrated the occurrence of bilateral pallidal hyperintensity on T1-weighted images in all patients undergoing hemodialysis associated with high serum manganese levels, revealing a new association.     

Basal ganglia hyperintensity on T1-weighted MRI in Rendu-Osler-Weber disease

The purpose of this study was to evaluate possible central nervous system (CNS) involvement in Rendu-Osler-Weber (ROW) disease in magnetic resonance imaging (MRI). Three patients with symptomatic ROW disease underwent brain MRI. Brain MRI depicted in all three of them increased signal intensity on T1-weighted images involving the globus pallidus and cerebral crura bilaterally. Laboratory studies of the two men showed iron deficiency anemia, while all three of them had normal liver function tests and increased manganese blood concentration. Gastroscopy and colonoscopy revealed a gastric and a cecal arteriovenous malformation (AVM) in the first one, while pulmonary and hepatic computed tomography (CT) angiography did not detect any intrahepatic shunts. Liver ultrasound in the second one revealed dilatation of intrahepatic artery branches consistent with intrahepatic shunts, while it was normal in the third patient. Chest radiographs were normal in all three patients. Pallidal T1 hyperintensity on T1-weighted imaging may be a biomarker of manganese overload in ROW disease.     

Hyperintense globus pallidus on T1-weighted MR imaging in acute kernicterus: is it common or rare?

Globus pallidus involvement is a well-known magnetic resonance (MR) imaging finding of acute kernicterus. However, it is not clear how early the involvement of globus pallidus occurs and whether or not it is seen in every case. Therefore, we aimed to investigate the globus pallidus involvement in 13 neonates with acute kernicterus by MR imaging. Thirteen neonates who were admitted with jaundice, encephalopathy and indirect hyperbilirubinemia (mean, 37.0 mg/dl) were prospectively evaluated with cranial MR imaging. Pathological signal changes were noted concerning the globus pallidus. Eight of the 13 patients demonstrated bilateral, symmetric increased signal intensity in the globus pallidus on T1-weighted MR imaging. These lesions were not apparent on T2-weighted images. Multiple parenchymal punctuate T1 hyperintense lesions were detected in one patient without globus pallidus involvement. This appearance was consistent with hemorrhage. The MR imaging findings of the other four patients showed no evidence of abnormality. The symmetric involvement of globus pallidus seen as hyperintense on T1-weighted MR imaging is a common and characteristic finding of acute kernicterus.     

MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation

BACKGROUND AND PURPOSE: Although brain MR imaging findings in adult Wilson disease have been described in considerable detail, a paucity of information currently exists regarding brain MR imaging findings in pediatric Wilson disease. The purpose of this study was to analyze the brain MR imaging findings in Wilson disease of childhood at the initial stage and during follow-up after treatment and to correlate these observations with clinical response. METHODS: We evaluated 50 patients with pediatric Wilson disease. Fifty initial and 20 follow-up MR images from 15 patients following penicillamine treatment were analyzed retrospectively, and the data were correlated with clinical findings. RESULTS: Patients were categorized into 3 groups on the basis of initial MR imaging findings. Group I (n = 23) showed normal MR imaging findings. Group II (n = 15) was characterized by T1-weighted images with increased signal intensity in the globus pallidus (n = 15, 100%) followed by the putamen, midbrain, and caudate nucleus. Group III (n = 12) demonstrated T2-weighted images with increased signal intensity in the putamen (n = 10, 83%), followed by the caudate nucleus, globus pallidus, thalamus, midbrain, and pons. There was a significant difference in mean age, the presence of neurologic symptoms, and Child-Pugh classification among the 3 groups (P < .001). Following copper chelating therapy, the changes on follow-up MR imaging were strongly correlated with clinical response to treatment (P < .001). CONCLUSION: Brain MR imaging in children with Wilson disease can be categorized into distinct groups and demonstrated a significant correlation with clinical findings. Interval changes on follow-up MR imaging were also closely correlated with clinical findings and helpful in assessing the clinical response.     

有机磷中毒性脑病恢复期MRI表现(附4例报告)

目的:探讨有机磷中毒性脑病恢复期的MRI特征.方法:回顾性分析4例重度有机磷中毒慢性期脑结构变化.结果:双侧巴宾斯基征阳性4例,脑电图轻度异常4例.1例右侧小脑半球和中脑长T1长T2信号.1例双侧尾状核和壳核对称性长T2信号,T1WI高信号.1例双侧尾状核、壳核和苍白球对称性长T1长T2信号,侧脑室扩大,小脑萎缩.1例双侧尾状核、壳核和苍白球对称性异常信号,T1WI部分萎缩的小脑回呈高信号;脑室扩大,双侧枕叶内侧回、脑干和小脑萎缩.结论:有机磷中毒性脑病恢复期MRI表现主要累及尾状核、苍白球、丘脑、中脑和小脑;严重者皮质出现软化灶,伴有脑萎缩.     

Sequential change of MR signal intensity of the brain after manganese administration in rabbits. Correlation with manganese concentration and histopathologic findings.

RATIONALE AND OBJECTIVES: High signal intensity in the basal ganglia on T1-weighted MR imaging has been reported in chronic manganese (Mn) poisoning. However, the exact meaning of the high signal intensity remains unclear: does it result from Mn itself, secondary pathologic changes of the brain tissue, or both? The goal of this study was to evaluate the sequential change of MR signal intensity and to correlate the MR intensity of the globus pallidus and the hypothalamus with the Mn concentration in the blood and the brain tissue, and with the histopathologic findings. METHODS: Ten milligrams per kilogram of Mn was administered once a week for 4 weeks to 14 rabbits. The rabbits in the control group (n = 2) were killed without Mn administration; those in group I (n = 4) were killed 1 day after the completion of Mn administration, those in group II (n = 4) were killed at 4 weeks, and those in group III (n = 6) were killed at 8 weeks. Sequential MR imaging, blood and tissue concentration measurement, and pathologic examination were performed. Sequential changes of the percent contrasts, contrast-to-noise ratios, and T1 relaxation times were analyzed with blood and tissue concentrations and histopathologic findings. RESULTS: The signal intensity of the basal ganglia on T1-weighted imaging was highest 1 day after cessation of Mn administration and sequentially washed out. The contrast, contrast-to-noise ratio, and T1 relaxation time showed significant correlations with blood concentration. Only the T1 relaxation time of the globus pallidus showed a significant correlation with tissue concentration. Histopathologic examination disclosed mild abnormalities in the globus pallidus, thalamus, and hypothalamus. CONCLUSIONS: The high signal intensity on T1-weighted MR imaging presumably indicates mainly the exposure marker of Mn, although mild pathologic findings were observed.     

胎儿基底核区发育的高场强MRI研究

目的 应用高场强MRI探讨胎儿基底核区的发育规律.方法 选取131例14～40孕 周胎儿标本,先行3.0 T MR扫描,从中选取11例14～27孕周标本再行7.0 T MR扫描.在上述MR图像上分析基底核区各结构的出现时间及信号变化.结果 在3.0 T MR图像上,14孕周时基底核区仅背侧丘脑能够辨别,15孕周时能辨识生发基质、尾状核与壳,18孕周时能显示苍白球,20孕周时内囊与外囊显示清晰.尾状核在15～30孕周时与脑白质的信号强度相比,在T1WI上呈低信号,T2WI上呈高信号,31～40孕周,在T1WI聊上呈高信号,T2WI上呈低信号.壳在15～17孕周时,在T1WI上呈高信号,T2WI上呈低信号,18～25孕周呈现斑块状外观,26～30孕周在T1WI上呈低信号,T2WI上呈高信号,31～40孕周在T1WI上呈高信号,T2WI上呈低信号.苍白球20孕周后T1WI上呈高信号,T2WI上呈低信号.与3.0 TMRI相比,7.0TT2WI更加清晰,16孕周时生发基质、尾状核、豆状核、背侧丘脑、内囊与外囊均显示清晰,18孕周时能辨别出屏状核.结论 3.0 T MR/可以较好地显示发育中的胎儿基底核区的结构.在14～21孕周,7.O T T2WI比3.0 T MR图像更好地显示胎儿基底核区的结构.     

与帕金森有关的亚临床期肝性脑病的MRI表现

目的:研究亚临床期肝性脑病患者锥体外系症状及MRI表现。方法:选择经神经心理测试诊断为亚临床期肝性脑病的患者12例,其中10例有明确的帕金森症状和其他锥体外系的症状,使用UPDRS(unified parkinson disease ratingscale)量表评价帕金森症状,所有患者行头部MRI检查。结果:10人有明确的帕金森症状,包括运动迟缓、肌强直、姿势性震颤等。所有患者MRI扫描都显示T1WI像苍白球对称的高信号。结论:亚临床期肝性脑病患者常表现为T1WI像高信号,并伴有帕金森症状,它与锰异常沉积于基底节区导致基底节功能受损有关。     

获得性肝性脑部变性的MRI表现

目的探讨获得性肝性脑部变性的MRI影像学特征及原因。方法对23例有精神神经症状的慢性肝硬化患者进行头颅MRI扫描,其中1例并行增强扫描,4例同时进行腹部扫描,分析其MRI征象。结果23例患者,22例脑部MRI有阳性发现,1例MRI表现阴性。双侧苍白球(22/22),豆状核(13/22),红核(9/22),垂体前叶(7/22),大脑脚盖(2/22)在自旋回波(SE)序列T1WI表现为高信号,快速自旋回波(FSE)T2WI信号正常,病灶区脑结构的体积、形态无变化。结论双侧基底节等部位出现T1WI高信号是获得性肝性脑部变性较为特征性的影像学表现,结合临床慢性肝硬化病史可确定诊断。