摘要
BACKGROUND： Wilson disease （WD） damages liver, brain, kidney, cornea and nervous system severely. It is manifested in four ways： brain, liver, kidney and bone muscle. Whether or not magnetic resonance imaging （MRI） can clearly display the diseased region and range in brain of patient with WD, which provides imageological evidence for clinical practice, is unclear. OBJECTIVE： To observe the characteristics of MRI of brain in patient with SD, and analyze the correlation of diseased region with clinical symptoms. DESIGN ： Retrospective case-analysis SETTING： Department of Radiology, Second Hospital Affiliated to the General Hospital of Chinese PLA. PARTICIPANTS： Thirty-one patients, including 18 males and 13 females, with WD admitted to the Department of Neurology, Second Hospital Affiliated to the General Hospital of Chinese PLA between January 1999 and December 2005 ware retrieved. The involved patients presented serum copper oxidase （sCP） activity decreasing and/or caruloplasmin level decreasing and /or udnary copper content increasing; typical extrapyramidal symptoms and/or physical sign; abnormality showed by slit-lamp examination, Kayser-Fleischer dog positive. METHODS ： （1） All the involved patients underwent MRI examination. AGE 1.5T imaging equipment was used. Spin-echo sequence was adopted to perform T2 and T1 -weighed image at transverse axis level. Partial cases subjected to head scanning at coronal and/or sagittal level. Gd-DTPA with dosage of 0.1 mmol/kg was the strongest in 4 cases. （2） MRI charactenstics of patients with different clinical symptoms were observed. MAIN OUTCOME MEASURES： MRI detection results of patients with WD and MRI characteristics of patients with different clinical symptoms. RESULTS： Thirty-one patients with WD participated in the result analysis. （1） Imageologicat examination results： WD involved many regions in the brain： dorsal caudate putamen （n =28）, thalamencephalon（n =25）, mesencephalon （n =25）, globus pallidus （n =23）, pons（n =21 ）, posterior limb of internal capsule （n =16）, dentate nucleus（n =16）, caudate nucleus（n =15） and cerebral cortex （n =11）. MRI presented hypo-intensity signal on T2 - weighted image and T1 -weighted image or isointensity signal on T1 -weighted image in 24 patients, characteristic hypo-intensity signal of globus pallidus in 4 patients, mixed signal of hypar- and hypo-intenslty in 2 patients, hypo-intensity signal of globus pallidus, pars anterior pedunculi cerebri and pontine tegmentum on T1 - weighted image in 1 patient. Pathological changes distributed in symmetry and focus of infection mostly presented mottling, lamellar or strip. Different degrees of cerebral cortex atrophy, especially subtentorial cerebellar atrophy, ware found in 20 patients. Four patients subjected to enhancement scanning, but no clear imaging was found. （3）MRI characteristics of patients with different symptoms： Abnormal signal of dorsal caudate putamen was found in 28 patients with dystonia, 21 patients with dysarthda and 16 patients with bradykinesia; Abnormal signal of mesencaphalon was found in 22 patients with trepidation, among which, 18 presented abnormal signal of ports; Abnormal signal of caudate nucleus and lenticular nucleus was found in 15 patients with clysphagia; Abnormal signal of dentate nucleus was found in 16 patients with cerebellar ataxia; Different degrees of changes in cerebral atrophy were found in 14 patients with deteriorating memory and dementia. CONCLUSION ： MRI can clearly display the characteristics of diseased regions in brain of patient with WD. Diseased regions reflected by MRI have obvious differences in patients with different clinical neurosigns.
BACKGROUND： Wilson disease （WD） damages liver, brain, kidney, cornea and nervous system severely. It is manifested in four ways： brain, liver, kidney and bone muscle. Whether or not magnetic resonance imaging （MRI） can clearly display the diseased region and range in brain of patient with WD, which provides imageological evidence for clinical practice, is unclear. OBJECTIVE： To observe the characteristics of MRI of brain in patient with SD, and analyze the correlation of diseased region with clinical symptoms. DESIGN ： Retrospective case-analysis SETTING： Department of Radiology, Second Hospital Affiliated to the General Hospital of Chinese PLA. PARTICIPANTS： Thirty-one patients, including 18 males and 13 females, with WD admitted to the Department of Neurology, Second Hospital Affiliated to the General Hospital of Chinese PLA between January 1999 and December 2005 ware retrieved. The involved patients presented serum copper oxidase （sCP） activity decreasing and/or caruloplasmin level decreasing and /or udnary copper content increasing; typical extrapyramidal symptoms and/or physical sign; abnormality showed by slit-lamp examination, Kayser-Fleischer dog positive. METHODS ： （1） All the involved patients underwent MRI examination. AGE 1.5T imaging equipment was used. Spin-echo sequence was adopted to perform T2 and T1 -weighed image at transverse axis level. Partial cases subjected to head scanning at coronal and/or sagittal level. Gd-DTPA with dosage of 0.1 mmol/kg was the strongest in 4 cases. （2） MRI charactenstics of patients with different clinical symptoms were observed. MAIN OUTCOME MEASURES： MRI detection results of patients with WD and MRI characteristics of patients with different clinical symptoms. RESULTS： Thirty-one patients with WD participated in the result analysis. （1） Imageologicat examination results： WD involved many regions in the brain： dorsal caudate putamen （n =28）, thalamencephalon（n =25）, mesencephalon （n =25）, globus pallidus （n =23）, pons（n =21 ）, posterior limb of internal capsule （n =16）, dentate nucleus（n =16）, caudate nucleus（n =15） and cerebral cortex （n =11）. MRI presented hypo-intensity signal on T2 - weighted image and T1 -weighted image or isointensity signal on T1 -weighted image in 24 patients, characteristic hypo-intensity signal of globus pallidus in 4 patients, mixed signal of hypar- and hypo-intenslty in 2 patients, hypo-intensity signal of globus pallidus, pars anterior pedunculi cerebri and pontine tegmentum on T1 - weighted image in 1 patient. Pathological changes distributed in symmetry and focus of infection mostly presented mottling, lamellar or strip. Different degrees of cerebral cortex atrophy, especially subtentorial cerebellar atrophy, ware found in 20 patients. Four patients subjected to enhancement scanning, but no clear imaging was found. （3）MRI characteristics of patients with different symptoms： Abnormal signal of dorsal caudate putamen was found in 28 patients with dystonia, 21 patients with dysarthda and 16 patients with bradykinesia; Abnormal signal of mesencaphalon was found in 22 patients with trepidation, among which, 18 presented abnormal signal of ports; Abnormal signal of caudate nucleus and lenticular nucleus was found in 15 patients with clysphagia; Abnormal signal of dentate nucleus was found in 16 patients with cerebellar ataxia; Different degrees of changes in cerebral atrophy were found in 14 patients with deteriorating memory and dementia. CONCLUSION ： MRI can clearly display the characteristics of diseased regions in brain of patient with WD. Diseased regions reflected by MRI have obvious differences in patients with different clinical neurosigns.
