肠道病毒71型感染手足口病合并急性弛缓性麻痹的临床及MRI分析

目的：探讨肠道病毒71型感染手足口病（HFMD）合并急性弛缓性麻痹（AFP）的临床及MRI表现。方法搜集2010年05月～2011年010月EV71感染 HFMD合并AFP的患儿13例,分析其临床表现及脊髓MRI特征。结果13例患儿中,表现为单侧下肢瘫痪6例,双下肢瘫痪2例,单侧上肢瘫痪2例,一侧肢体偏瘫1例,双侧上肢瘫痪1例,肢体交叉瘫痪1例,表现为左上肢及右下肢瘫痪。M RI表现为矢状面脊髓内长条形等或长 T1、长 T2信号,横断面显示相应节段脊髓前角圆点状长T2信号;下胸段至腰骶段脊髓前角受累8例,颈段脊髓5例,受累范围3个椎体及以上9例;单侧受累1例,双侧受累12例,其中对称性病变3例,不对称9例。5例颈段脊髓病变中有4例合并脑干脑炎,病变以脑桥、延髓交界部为主,呈长T1长T2信号。M RI表现与临床表现具一致性,脊髓前角病变引起所支配的上肢和（或）下肢肌群的急性弛缓性麻痹。结论 M RI检查是诊断 H FM D合并急性弛缓性麻痹的首选的影像检查方法,损害部位位于脊髓前角,以下胸段至腰骶段脊髓及颈段脊髓常见,影像学表现与临床表现具有一致性。     

Patterns of spinal cord atrophy by metrizamide CT

Forty patients with cervical myelopathy underwent high-resolution computed tomography (CT) with intrathecal administration of metrizamide for evaluation of cervical spinal cord atrophy. Thirty of them showed evidence of either focal atrophic distortion or generalized accentuation of the anatomic surface features of the spinal cord. Patients with a Chiari malformation or syringomyelia were excluded. The characteristic features in cervical spondylosis and canal deformity include flattening of the ventral surface of the cord, central infolding, beaking of the lateral funiculi, and wasting of the dorsal surface of the cord. Patients with motor neuron disease showed a combination of anterolateral and posterolateral atrophy reflecting underlying degeneration of the anterior horn cells and/or corticospinal tracts, respectively. Those with monomelic motor neuron disease had a striking ipsilateral hemiatrophy of the spinal cord. Among those presenting with spastic paraparesis, seven with clinically definite multiple sclerosis showed diffuse atrophy or focal degeneration due to a localized plaque of demyelination. Two cases of cord neoplasm showed atrophy secondary to ascending or descending degeneration of the long tracts.     

Hirayama disease: MR diagnosis.

We report the MR findings in two cases of Hirayama disease, a kind of cervical myelopathy related to flexion movements of the neck. In flexion MR studies, we can see the striking and pathognomonic picture of anterior shifting of posterior dura at the lower cervical spinal canal. In nonflexion studies, we find that asymmetric cord atrophy, especially at the lower cervical cord, though subtle, is highly suggestive of Hirayama disease. When it is seen, a flexion MR study is warranted to prove this diagnosis.     

Acute flaccid paralysis in infants and young children with enterovirus 71 infection: MR imaging findings and clinical correlates

BACKGROUND AND PURPOSE: Enterovirus 71 (EV71) infection is now considered an important cause of childhood acute flaccid paralysis. The purpose of our study was to determine whether EV71-infection-related acute flaccid paralysis in infants and young children has characteristic MR imaging patterns. METHODS: Seven infants and young children with acute paralysis of the upper or lower extremities and positive EV71 cultures underwent spinal MR studies during an outbreak of hand-foot-and-mouth disease in Taiwan in 1998. RESULTS: Acute paralysis was observed in one upper extremity in two patients, in one lower extremity in three patients, and in both lower extremities in two patients. None of the patients had sensory impairment or bulbar palsy. MR studies showed unilateral or bilateral hyperintense lesions in the anterior horn regions of the cord on T2-weighted images in six patients. No abnormal signal was present in one patient. Two of three patients who received intravenous injections of contrast material had ventral root enhancement on T1-weighted images. One of them also had enhancement of the unilateral anterior horn cells. At clinical follow-up, both patients with bilateral anterior horn abnormalities had residual motor weakness, whereas only one of the five patients with unilateral involvement had residual weakness. CONCLUSION: EV71 radiculomyelitis tends to be unilateral and to specifically involve both the anterior horn cells of the cord and the ventral roots. MR imaging allows early detection of spinal cord and root lesions.     

Computed tomographic myelography characteristics of spinal cord atrophy in juvenile muscular atrophy of the upper extremity.

Although atrophy of the lower cervical and upper thoracic cord in juvenile muscular atrophy of distal upper extremity has been reported, the atrophic patterns of the cord, especially in the transverse section, have not been studied extensively. The aim of this study is to clarify the atrophic patterns of the cord by CT myelography (CTM) and to discuss the pathogenesis of cord atrophy. Sixteen patients with juvenile muscular atrophy of distal upper extremity were examined by CTM. Atrophy of the lower cervical and upper thoracic cord, consistent with the segmental weakness, was seen in all patients. Flattening of the ventral convexity was a characteristic atrophic pattern of the cord. Bilateral cord atrophy was commonly observed; eight of 12 patients with unilateral clinical form and all four patients with bilateral form showed bilateral cord atrophy with dominance on the clinical side. There was no correlation between the degree of cord atrophy and duration of symptoms. Flattening of the ventral convexity, associated with purely motor disturbances, reflects selective atrophy of the anterior horns in the cord, which is attributable to chronic ischemia. Cord atrophy proved to precede clinical manifestations. The characteristic atrophy of the cord provides useful information to confirm the diagnosis without long-term observation.     

屈颈MRI对青年性上肢远端肌萎缩症的诊断价值

目的评价屈颈MRI对青年性上肢远端肌萎缩症的诊断价值。方法男性患者5例，平均年龄21岁，临床表现为一侧或两侧上肢远端肌萎缩。对照组为健康志愿者，21岁男性8例。2组均行常规及屈颈颈椎MR平扫，矢状、轴面SET1WI、T2WI、液体衰减反转恢复（FLAIR）序列扫描。结果常规颈椎扫描：5例患者下段颈髓变细；屈颈位MR扫描：下颈段颈6以下脊髓前屈、变扁平，矢状径4～6mm，硬膜囊后壁前移，硬膜后间隙明显增宽，可见多发条状、迂曲流空信号影及软组织信号。对照组：常规扫描，下颈段脊髓（颈6～胸2）可见颈膨大，屈颈位脊髓略变细（6～7mm），硬膜囊后壁无前移，硬膜后间隙未见扩张血管影。结论屈颈MRI有助于显示下颈段脊髓及硬膜囊改变，结合临床资料可准确诊断青年性上肢远端肌萎缩症。     

Hirayama disease in unilateral and bilateral forms-3 case reports

Hirayama disease is a benign, nonprogressive motor neuron disease affecting the upper limbs. It is secondary to an abnormal anterior displacement of the posterior dura with secondary compression of the lower cervical spinal cord. It should be suspected in young male patients with a chronic history of weakness and atrophy involving the upper extremities followed by clinical stability in few years. The involvement is usually unilateral but may be bilateral. MR imaging is the best way to make the diagnosis but it necessitates the use of both extension/flexion and post contrast studies.     

肠道病毒71型感染手足口病合并急性弛缓性麻痹的脊髓MRI特征

目的 探讨肠道病毒71型(EV71)感染手足口病合并急性弛缓性麻痹(AFP)的脊髓MRI特点.方法 回顾性分析8例EV71感染手足口病合并AFP患儿临床及影像资料,总结其脊髓MRJ特点.结果 8例患儿中,单侧下肢无力4例,四肢无力2例,单侧上肢无力和双侧下肢无力各1例,合并脑干脑炎2例.MRI表现为脊髓前角T2WI高信号,T1WI低信号,其中C3～7水平1例,T10至圆锥、T11至圆锥水平5例,两处均受累2例.单侧肢体无力5例,其中2例为单侧脊髓前角受累,3例表现为双侧受累,且患侧病变范围大,信号强度高.1例行增强扫描,表现为受累脊髓前角轻度强化,同侧前根明显强化.双侧肢体无力3例,均表现为双侧脊髓前角受累.结论 MR平扫及增强检查是诊断EV71相关AFP所致的脊髓神经根病变的首选的影像检查方法.     

MR imaging in radiation myelopathy.

PURPOSE: Using MR imaging, we assessed the signal, size, and enhancing characteristics of the cervical cord in patients in whom radiation myelopathy developed after radiotherapy for nasopharyngeal carcinoma. PATIENTS AND METHODS: Ten patients, 3 men and 7 women, aged from 32 to 77 years, were included. MR imaging was performed 1 to 53 months after clinical manifestations of myelopathy. RESULTS: Two cases showed atrophy of the cervical cord without abnormal signal intensity; in the others, a long segment of the cervical cord demonstrated low signal intensity on T1-weighted images and high signal intensity on T2- or T2*-weighted images. Some of these cases also showed swelling of the cord. Focal enhancement at C1-C2 area after intravenous administration of Gd-DTPA was seen in four cases. CONCLUSIONS: There is a correlation between the time of MR imaging after onset of symptoms and MR findings. When MR scans were obtained more than 3 years after onset of symptoms, atrophy of the cervical cord was noted without abnormal signal intensity. When MR was performed less than 8 months after onset of symptoms, a long segment of the cervical cord demonstrated abnormal signal intensity with or without associated swelling of the cord and focal enhancement.     

平山病的MRI诊断价值

目的:探讨平山病的MR表现和价值,认识平山病的临床特征和可能的发病机制.方法:5例经临床确诊的平山病患者均隐匿起病,病变局限于上肢远端肌肉,4例单侧上肢前臂受累,1例双侧不对称性受累.5例患者均行颈椎X线平片检查,及颈髓常规自然体位MR平扫及屈颈位平扫 增强扫描.所有患者均进行常规生化检查,脑脊液检查,肌电图检查和肌肉活检.结果:5例患者普通X线检查4例可见颈椎生理曲度变直,自然体位MR平扫3例有低位脊髓萎缩变扁(萎缩水平在颈5-胸1,最明显在颈6水平),2例萎缩的脊髓内信号异常.曲颈MR检查时,除了可见自然体位MR的表现外,5例患者均可见特征性的颈髓前移、变平,硬脊膜向前移位,硬脊膜外腔增宽,4例患者在增宽的硬膜外腔均可见流空血管信号,增强扫描5例患者在增宽的硬脊膜外腔均可见强化的静脉丛.结论:平山病的MR表现存在一定的特征性,MR屈曲位平扫 增强扫描对平山病的诊断及其发病机制的理解有重要的价值.     

Magnetic resonance imaging in a juvenile type of distal and segmental muscular atrophy of the upper extremities

Eight patients with a juvenile type of distal and segmental muscular atrophy of the upper extremities (DSMA), a type of cervical flexion myelophathy, were evaluated using MR imaging. In the neutral position there was no spinal cord compression, but in flexion the spinal cord was displaced anteriorly and was compressed by the posterior surfaces or margins of the vertebrae and/or any herniated disks in all cases. In flexion, compression of the cord was exaggerated in seven patients by the anterior displacement of the posterior margin of the thecal sac, which was accompanied by dilated posterior internal vertebral veins. In patients suspected of having DSMA, MR images made in flexion are regarded essential for verifying the diagnosis. Correspondence to: K. Hasuo     

Subacute necrotizing myelopathy: MR imaging in four pathologically proved cases.

This report describes the MR and correlative imaging findings of four histologically proved cases of subacute necrotizing myelopathy in which there was no evidence of a spinal dural arteriovenous fistula. Subacute necrotizing myelopathy is characterized clinically by progressive motor and sensory deterioration, and pathologically by necrosis in the spinal cord. Initial MR imaging showed focal enlargement of the spinal cord and nonspecific T1 and T2 lengthening. Rimlike enhancement was demonstrated in one case. Clinically, steroid therapy failed in all four patients. Follow-up MR scans showed two slightly enlarged lesions, one stable thoracolumbar lesion, and atrophy of a cervical lesion. Open spinal cord biopsies revealed foci of necrosis and abnormal parenchymal vessels with thickened hyalinized walls. A prolonged course distinguishes subacute necrotizing myelopathy from acute transverse myelitis, but the clinical course and imaging appearance are similar to those of intramedullary tumor. Rimlike rather than solid contrast enhancement may be a distinguishing feature. In the absence of a demonstrable spinal dural arteriovenous fistula, the radiologic differentiation of subacute necrotizing myelopathy from tumor is probably impossible, and biopsy establishes the correct diagnosis.     

1H-Magnetic Resonance Spectroscopy in diffuse and focal cervical cord lesions in Multiple Sclerosis

Objectives

To investigate differences between focal and diffuse cervical lesions in multiple sclerosis (MS) by proton magnetic resonance spectroscopy (¹H-MRS) at 1.5 T in comparison to quantitative MR imaging of the upper cervical cord area and T2 relaxometry at baseline and follow-up.

Methods

Including 22 MS patients with persistent spinal cord symptoms by either diffuse or focal lesions and 17 controls, we acquired MRS, the mean cord area and the water T2 relaxation time and disability at baseline and follow-up. Cross-sectional analyses included group-level comparisons and correlation studies. Follow-up studies covered assessment of reproducibility and progression of the baseline results.

Results

Compared with focal lesions, diffuse lesions were attended by more cord atrophy, longer T2, elevated levels of creatine (Cre) and reduced N-acetyl aspartate (NAA)/Cre (focal/diffuse: 83?±?9/73?±?15 mm², 121?±?21/104?±?13 ms, 3.6?±?1.1/5.1?±?2.4 mM, 2.4?±?1.1/2.0?±?0.9). NAA/Cre at baseline was associated significantly with cord atrophy and with clinical progression during follow-up. Baseline MRS results were not significantly correlated to the clinical disability parameters. The reproducibility of MRS was 0.17-0.30. Longitudinal changes of the MRS results were not statistically significant.

Conclusions

MRS indicated differences in demyelination and gliosis between diffuse and focal cervical lesions in MS. Although longitudinal spectral and clinical changes were sparse, NAA/Cre turned out to be the most sensitive spectral parameter.

Key Points

? MR spectroscopy shows differences between focal and diffuse spinal cord MS lesions. ? MRS indicates axonal degeneration and gliosis in MS compared with controls. ? MRS results are associated with cervical cord atrophy. ? MRS may help clinicians to monitor spinal cord damage in MS.     

肠道病毒71感染手足口病合并急性弛缓性麻痹的脊髓MRI表现及肌力的2年随访研究

目的探讨肠道病毒71型(EV71)感染手足口病合并急性弛缓性麻痹(AFP)的起病及恢复期的脊髓MRI特点,并观察影像学和肌力变化之间的相互关系。资料与方法搜集2008年6至10月EV71感染手足口病合并AFP患儿8例,对其脊髓MRI表现及肌力进行2年随访观察。结果本组8例,单侧下肢无力4例,双侧下肢无力2例,单侧上肢无力和双侧上肢无力各1例,合并脑干脑炎4例。MRI病变特异性累及脊髓前角,上肢无力累及颈膨大,下肢无力累及腰膨大,T2WI高信号。单侧肢体无力5例,其中2例为单侧脊髓前角受累,3例表现为双侧受累,患侧病变范围大,信号强度高。双侧肢体无力3例,脊髓前角病变范围大的一侧肌力下降明显。2年后复查,8例均表现脊髓前角病变范围减小,肌力提高者10肢,肌力下降1肢,新出现肌力下降者1肢。T2WI呈高信号及稍高信号6例,肌力均达4级以上,呈脑脊液信号2例,肌力未达4级。结论脊髓MRI是诊断和随诊EV71感染手足口病合并AFP的最佳影像学检查方法。起病时病变累及脊髓前角区,单侧或双侧受累,以一侧为主多见。恢复期病变范围均有不同程度吸收,多数病变T2WI信号减低。起病时病变范围与肌力下降程度相一致,恢复期T2WI病变区仍呈脑脊液信号者肌力恢复较差。脊髓MRI对EV71感染手足口病合并AFP的临床预后评估具有一定的价值。     

Hirayama flexion myelopathy: neutral-position MR imaging findings--importance of loss of attachment

PURPOSE: To investigate the sensitivity and specificity of various neutral-position magnetic resonance (MR) imaging findings in the diagnosis of Hirayama flexion myelopathy. MATERIALS AND METHODS: The neutral-position cervical MR images of 46 patients and 51 control subjects were evaluated for the following findings: localized lower cervical cord atrophy, asymmetric cord flattening, abnormal cervical curvature, loss of attachment (LOA) between the posterior dural sac and subjacent lamina, and noncompressed intramedullary high signal intensity on T2-weighted MR images. The difference in frequency of these findings between the control and patient groups was examined by means of the chi(2) test. The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of these MR imaging findings in the diagnosis Hirayama disease were calculated. Multivariate analysis of these findings was also performed. RESULTS: There was a significant difference in the frequency of these MR imaging findings between the control and patient groups (all comparisons, P 相似文献   

脊髓亚急性联合变性的MRI诊断要点分析

目的分析脊髓亚急性联合变性(SCD)的MRI诊断要点。资料与方法回顾性分析15例临床确诊SCD的病人临床及MRI资料,分析其脊髓MRI表现特点,统计分析SCD病人受累脊髓节段数与血清VB12水平、发病时间的相关性。结果 15例SCD病人均于横断面影像上显示颈胸段脊髓对称性等或长T1、长T2信号,13例病变主要位于颈胸段水平脊髓后索,2例同时累及后索、侧索及前索。但于横断面T2WI上颈段与胸段脊髓异常信号形态各具特点。颈段脊髓受累时于横断面T2WI上呈脊髓内"倒V"形或"反兔耳"形的对称性高信号。胸段脊髓受累时于横断面T2WI上呈脊髓内"哑铃"形的对称性高信号。统计学分析显示15例SCD病人脊髓受累节段数与血清VB12水平呈负相关,而与病程长短无相关性。结论脊髓MR成像对于SCD脊髓疾病的诊断有重要价值。     

MR imaging of the spinal cord in 23 subjects with ALD-AMN complex.

Twenty-three subjects from two family groups with the adrenoleukodystrophy (ALD)-adrenomyeloneuropathy (AMN) complex were examined with MR imaging at 1.5 T to determine the presence and extent of brain and spinal cord abnormalities. Nineteen individuals were identified as having ALD or AMN, or as having carrier status on the basis of pedigree analysis and/or evaluation of serum very-long-chain fatty acids. In addition to the expected intracranial white matter changes for this disorder, decreased spinal cord diameter was found in seven (30%) of the 23 subjects. In three of these cases, atrophy was limited to the thoracic spinal cord, while atrophy of both the cervical and thoracic cord was identified in four patients. Two patients who did not have MR imaging of the spine were found to have spinal cord atrophy at autopsy. The finding of decreased spinal cord diameter on MR examinations in individuals who are heterozygous for ALD-AMN, in patients with ALD or AMN, and in asymptomatic ALD-AMN patients may represent a new anatomic marker for the variable clinical presentations of this condition. In addition to cranial MR examination, MR imaging of the spine may be indicated in patients with suspected ALD or AMN, or in women with carrier status.     

Spinal cord infarction: MR imaging and clinical features in 16 cases

Spinal cord infarctions are rare and due to heterogeneous etiologies. The aim of the study was to analyze the MR imaging findings and evaluate their correlations with clinical symptoms in ischemic spinal cord lesions. MR images and clinical features of 16 patients (11 male, 5 female) with typical sudden onset of neurological deficits caused by spinal cord ischemia were evaluated. MR imaging was performed within 2 h to 14 days after the initial neurological symptoms. Eight patients had follow-up examinations including contrast-enhanced MR imaging. MR abnormalities were best demonstrated on sagittal T2-weighted images, with "pencil-like" hyperintensities (16/16) and cord enlargement (9/16). Axial T2-weighted images showed bilateral (13/16) and unilateral (3/16) hyperintensities according, in 15 patients, to anterior spinal artery (ASA) territory, with three of them located particularly in the spinal sulcal artery territory. In one patient only the posterior spinal artery (PSA) territory was involved. Spinal cord was affected at the cervical level (especially C2-C3) in seven patients, at the upper thoracic level (T3-T5) in two patients and at the thoracolumbar region including the conus medullaris (T10-L1) in seven patients. Presumed etiologies were vascular surgery (3 patients), infrarenal aortic aneurysm (1 patient), bilateral vertebral artery dissection (1 patient), hypotension (1 patient), spine operation (1 patient), excessive cocaine misuse (1 patient) and cardioembolic vertebral artery occlusion (1 patient); six of seven patients with unclear etiologies had vascular risk factors such as hypertension, diabetes and cigarette smoking. MR imaging is therefore useful in detecting spinal cord infarction, with axial T2-weighted images showing hyperintensities in the ASA territory in 15 of 16 patients. Contrary to the presumed spinal cord watershed at the lower cervical and upper thoracic level, and despite numerous central arteries in the cervical cord, our data suggest a high ischemic vulnerability of the cervical spinal cord at level C2-C3.     

Venous infarction of the spinal cord resulting from dural arteriovenous fistula: MR imaging findings

Three patients with spinal dural arteriovenous fistula presented with acute and/or progressive myelopathy. The thoracic cord was focally enlarged and poorly defined on MR images in two of the patients. One individual showed focal cord atrophy, and one demonstrated abnormal intrathecal vessels. In all patients MR studies revealed cord enhancement after IV administration of gadopentetate dimeglumine. The MR findings are believed to represent disruption of the blood-cord barrier associated with cord ischemia and/or infarction, which, in turn, is caused by venous stasis resulting from the fistula. The diagnosis in each case was confirmed by the combined results of myelography, spinal arteriography, and surgery. Surgical excision or embolization of the fistula produced a poor return of lost function but an arrest in the progression of paresis. One of the patients had constant severe back and leg pain postoperatively, and a follow-up MR study 5 months after surgery showed focal atrophy and persistent enhancement of the thoracic cord. The patient with preoperative focal cord atrophy had an MR examination 1 year prior to surgery, which revealed enhancement of the cord similar to that seen on the immediate preoperative MR study. This patient also had severe pain in the back and lower extremities preoperatively, which accompanied her progressive paraparesis. It is believed that long-standing enhancement of the spinal cord in patients with dural arteriovenous fistula probably results from chronic progressive venous ischemia, which may be irreversible and cause pain of a central type.     

亚急性联合变性颈髓的磁共振成像表现

目的分析亚急性联合变性颈髓的磁共振成像表现特点,提高对该病的认识.方法收集临床确诊亚急性联合变性累及颈髓的患者5例,回顾性分析其磁共振成像表现,并复习相关临床及文献资料.结果5例患者磁共振成像均显示颈髓不同程度的后、侧索长T2信号,其颈髓正中矢状位T2加权图像均呈连续束带状异常信号;4例行钆喷替酸葡甲胺增强扫描,其中2例可见条片状强化.结论亚急性联合变性的颈髓矢状位束带状改变有比较典型的特点,磁共振成像是诊断该病的重要手段.