儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病首次发作的MRI分析

目的 探讨儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）首次发作的MRI特点。资料与方法 回顾性分析2018年5月—2021年2月湖南省儿童医院临床诊断为MOGAD的40例患儿首次发作时头颅、脊髓及视神经MRI表现,患儿首次发作年龄1.2～13.8岁。结果 40例患儿中,37例（92.5%）头颅MRI可见异常病灶,30例为多脑叶受累,24例皮层、皮层下白质及深部白质均受累,额叶（78.4%,29/37）、顶叶（75.7%,28/37）为最常累及的脑叶,扩散加权成像及磁敏感成像无异常,增强扫描无强化或轻度强化。14例（38.9%）脊髓MRI异常,表现为脊髓长节段、不连续稍长T1稍长T2信号影,主要位于中央灰质部位,增强扫描无强化或轻度强化;其中13例胸髓受累,8例颈髓受累。31.0%(9/29)视神经MRI异常,主要表现为单侧（4例）或双侧（4例）视神经稍增粗,T2WI信号增高,1例累及视交叉,增强扫描以视神经鞘及周围脂肪组织强化为特征。结论 MOGAD患儿头颅、脊髓及视神经MRI表现多样,在头颅多表现为皮层、皮层下白质及深部白质的多发稍长T1稍长T2信号影,在脊髓表现为颈胸髓内长...     

儿童线粒体脑肌病的MRI表现

目的回顾性研究20例线粒体脑肌病患儿(年龄10个月到14岁)的MRI表现.方法20例由肌肉活检及实验室检查证实为线粒体脑肌病的患儿,脑内均有MRI阳性表现,研究其MRI表现的类型.结果20例患儿脑内病灶均表现为T1WI低、T2WI高信号,8例有不同程度的脑萎缩.18例患儿主要为灰质受累,其中10例为只有深部灰质病变;4例为深部灰质和大脑皮质同时受累.4例为深部灰质病变合并脑梗塞而同时累及灰质和白质.2例主要为白质受累,表现侧脑室后角旁白质异常信号.结论儿童线粒体脑肌病的MRI表现是多样性的.当儿童脑MRI表现为灰质(尤其是深部灰质)异常信号、灰质萎缩、不典型梗塞、不典型白质病变且合并临床难以解释的神经、肌肉等多系统的症状时,应考虑到线粒体肌病的可能.     

新生儿低血糖脑损伤的MRI诊断(附7例分析)

目的:探讨新生儿低血糖脑损伤的MRI表现特点及其诊断价值。方法:回顾分析7例新生儿低血糖脑损伤的MRI表现及临床资料,所有患儿均在入院后1~8天行MRI检查。结果:7例患儿血糖水平在0.2~1.6mmol/l,平均约0.86mmol/l。磁共振表现:7例患儿顶枕叶均有斑片状异常信号,DWI上呈高信号,4例在T1加权上内囊后肢高信号消失,2例HIE合并有低血糖患儿除顶枕叶信号异常外,额顶叶皮层、皮层下及脑室旁还可见点状、条状、片状短T1高信号,所有患儿小脑及脑干均未见异常。结论:新生儿低血糖脑损伤多发生在顶枕叶后部脑组织,磁共振弥散加权有助于早期发现病变。     

MRI对先天性肌营养不良的诊断价值

目的研究先天性肌营养不良(congenital muscular dystrophies,CMD)的MRI特点。资料与方法对10例临床诊断为CMD的患儿行常规头部MRI,分析其脑白质及脑发育异常情况。脑干发育是否正常经与对照组比较后进行判断。结果10例中,1例MRI表现基本正常,其余9例均有不同程度的脑白质异常,侧脑室旁白质异常8例,皮层下白质异常9例。1例有新生儿缺氧缺血脑病(HIE)后遗改变。1例有侧脑室扩大。1例小脑发育不良。2例小脑可见多发囊性小病灶。6例脑干发育不良。结论CMD的颅脑MRI具有特征性,MRI能为CMD的诊断提供帮助。     

桥脑中央髓鞘溶解症MRI表现分析

目的 :通过对21例桥脑中央髓鞘溶解症(central pontine myelinolysis,CPM)患者的MRI表现进行分析,探讨CPM的MRI特征性表现。方法 :回顾性分析21例CPM患者的MRI表现。结果 :21例均表现桥脑基底中央部对称性长T1、长T2信号,其中11例可见双侧半卵圆形中心及双侧额顶叶皮层下白质、双侧豆状核、尾状核、双侧桥小脑结合臂、小脑中脚、小脑齿状核对称性长T1、长T2信号;所有患者桥脑基底部病变在T1WI及T2WI上边界清晰。结论 :CPM具有较为特征性的MRI表现,MRI对其具有较大诊断和鉴别诊断价值。     

常染色体显性遗传性脑动脉病伴皮层下梗死和白质脑病的颅脑MRI表现

目的 提高对常染色体显性遗传性脑动脉病伴皮层下梗死和白质脑病(CADASIL)的颅脑MRI表现的认识.方法 对一家系2代5例患者进行头颅常规MR和MR血管成像(MRA)检查.对经Notch3基因检查或皮肤组织活检超微病理检查确诊的3例和经MRI与临床诊断的1例CADASIL的MRI资料进行分析.结果 MR检查的5例中4例CADASIL均获得明确诊断,1例排除诊断.4例CADASIL均见两侧颞叶、额叶和顶叶大致对称性皮层下与侧脑室旁白质病灶,呈长T1、长T2信号,但枕叶累及甚少且皮层不受累;O'Sullivan征阳性4例,皮层下腔隙性损害(SLLs)征阳性2例;3例半卵圆中心可见多发圆形或卵圆形囊性梗死即"黑洞",4例均见多发圆点状血管周间隙即"胡椒罐盖"样征象;4例全部显示胼胝体单发或多发斑片状显著长T1、长T2信号,其中2例伴萎缩;内囊前肢与外囊均受累,呈"人"字征;基底节和脑干可见单发或多发陈旧性腔隙性梗死灶;1例伴右侧小脑小片状梗死灶;4例全部有轻度至中度的脑干、小脑和大脑萎缩;MRA颅内Ⅰ-Ⅲ级较大动脉均未见明显异常.结论 CADASIL的颅脑MRI表现具有一定的特征性,可为CADASIL的初诊和筛选提供重要依据.     

儿童手足口病并发脑干脑炎的MRI表现

目的分析儿童手足口病(hand-foot-and-mouth disease,HFMD)并发脑干脑炎的MRI表现特征,探讨MRI对于早期诊断该并发症的临床价值。资料与方法回顾性分析18例经临床确诊为HFMD并发脑干脑炎的患儿的颅脑MRI资料,所有病例均行头颅MRI平扫及增强扫描。结果影像表现为单发或多灶性的、部分呈对称性分布的脑损害病灶,18例患儿共有病灶25个,其中脑桥-延髓交界处背侧8个,延髓背侧7个,脑桥背侧5个,中脑2个,丘脑1个,脑室旁白质区1个,小脑齿状核1个。MRI信号主要表现为以下几种:(1)T1WI呈低信号,T2WI呈高信号,边界清晰,液体衰减反转恢复序列(FLAIR)、扩散加权成像(DWI)正常或呈低信号,无强化;(2)T1WI呈稍低信号,T2WI呈稍高信号,边界模糊,FLAIR、DWI正常或呈高信号,部分病灶轻度强化;(3)平扫正常,增强扫描可见斑片状轻度强化。结论脑干脑炎为儿童HFMD的严重并发症之一,颅脑MRI对其诊断有较高敏感性和一定特异性,能明确病灶的部位和范围,可为临床诊治提供可靠的影像学依据。     

不同临床分期海洛因海绵状白质脑病患者的头颅MRI特征

目的探讨不同临床分期的海洛因海绵状白质脑病（HSLE）患者的头颅影像特征和演变规律。方法回顾性分析本院收治的行MRI检查的32例HSLE患者资料，其中I期（小脑受累期）6例，Ⅱ期（合并锥体束受累）21例，Ⅲ期（存在意识障碍）5例。按照临床分期对其吸毒史、临床表现、头颅MRI表现进行总结分析。结果全部患者均有烫吸海洛因病史；亚急性起病多见，小脑体征为早期主要表现；头颅MR检查显示均有双侧对称性的小脑半球损害，按照疾病的严重程度，逐步累及胼胝体压部、内囊后肢和枕顶叶深部白质，部分病例累及脑干及额颞叶深部白质；所有病例头颅MRI均未见明显增强病灶。结论烫吸海洛因病史是诊断HSLE的必要条件。头颅MRI提供了HSLE特征性结构性损害的病灶演变特征，对明确诊断具有重要意义，根据受累部位还有助于判断疾病的临床分期。     

脑多发性硬化的MRI征象分析

目的　提高对脑多发性硬化 (MS)MRI表现特征的认识。方法　采用 0 .5T超导式磁共振仪 ,对 6 1例临床确诊的脑内MS病人行SE序列扫描 ,分析其MRI表现及特征。结果　 6 1例MS在MRI上均发现病灶。MRI对于大脑半球、小脑和脑干的病灶均能清晰显示 ,对视神经病灶显示不佳。MS病灶T1WI呈中等或低信号 ,T2 WI均为高信号。脑室旁病变其长轴多与侧脑室垂直及胼胝体的信号异常和萎缩对MS确诊起着重要作用。结论　MRI可显示MS的特征性表现 ,能发现许多CT不能发现的病灶 ,是目前诊断MS最好的影像检查方法。     

神经元移行异常的MRI诊断

目的分析各种神经元移行异常的MRI特征。方法分析50例神经元移行异常的MRI表现,总结MRI影像特征。结果灰质异位23例,脑裂畸形12例,多小脑回9例,巨脑回5例,无脑回1例。脑灰质异位MR表现为皮质下,白质区,侧脑室室管膜下结节状、团块状或带状病灶,在所有序列上与正常脑灰质信号相同。脑裂畸形表现为大脑半球的横行裂隙,边缘衬有灰质。多小脑回畸形表现为脑回增多、细小而浅。巨脑回畸形表现为脑回增宽,灰质增厚,白质变薄。无脑回畸形表现为大脑半球呈肾形,正常脑沟和脑回消失,皮质增厚,白质变薄。结论MRI是诊断神经元移行异常的最佳影像方法。     

儿童急性播散性脑脊髓炎与临床孤立综合征的临床及影像学比较研究

目的探讨急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)与临床孤立综合征(clini-cally isolated syndrome,CIS)的鉴别诊断要点。资料与方法搜集39例患儿首发中枢神经系统脱髓鞘病变时的临床及影像学资料。其中CIS 18例,包括女13例,男5例;ADEM共21例,其中女8例,男13例。平均随访时间4.2年,最短随访时间为2年。由一名儿科神经医师对CIS及ADEM的临床表现进行了归类总结。由一名资深神经影像学医师对患者ADEM及CIS头颅MRI表现进行分析,内容包括病灶的位置、大小、形态。对计数资料采用Fisher精确检验,对计量资料进行非参数Mann-Whitney U检验。分别应用几种CIS的诊断标准对患儿进行评价,比较分析每种标准诊断的特异性、敏感性、阳性预测值及阴性预测值。结果 39例儿童ADEM与CIS患者中,ADEM发病年龄小,平均约(6.14±3.41)岁;CIS平均发病年龄(10.05±2.87)岁,两者之间差异有统计学意义(P=0.000);ADEM临床表现以发热和运动障碍为主,分别占76%和71%,高于CIS的33%和22%(P=0.011,0.004)。而CIS中视力障碍及脑脊液寡克隆带阳性出现率分别占50%和61%,与ADEM的0%和24%比较,差异具有统计学意义(P=0.000,0.025)。随访中儿童ADEM的改良的Rankin量表评分不如CIS。影像学特征中,与CIS比较,中央白质病变及脑萎缩在ADEM中表现更为明显,分别占100%和38%(P=0.001,0.023);ADEM病灶分布相对对称。CIS的视神经及脊髓病变出现率分别为22%、17%,ADEM未出现此类病变(P=0.037,P=0.089)。"黑洞"在CIS中具有显著特异性,与ADEM比较差异具有统计学意义(P=0.037)。Callen诊断MS的标准敏感性最高,为87%。KIDMUS(2个均满足)MS诊断标准及Callen的鉴别MS与ADEM标准特异性均较高,分别为92%和90%。KID-MUS敏感性最差,为33%。Barkhof的成人MS标准在儿童中敏感性及特异性均较低,分别为22%和57%。结论临床及影像学特征的结合,可以帮助早期诊断与鉴别ADEM与CIS。在诊断CIS时应该用Callen诊断MS标准为主,而鉴别ADEM和CIS时可以用Callen的鉴别标准。     

MRI of the brain in the Kearns- Sayre syndrome: report of four cases and a review

We report brain MRI findings in four patients with typical Kearns-Sayre syndrome (KSS) and correlate them with clinical manifestations. MRI was interpreted as normal in two patients; cerebral and cerebellar atrophy was seen in the other two. On T2-weighted spin-echo images, two patients had high-signal lesions bilaterally in subcortical white matter, thalamus and brain stem. In one patient, the white matter lesion extended into the deep cerebral white matter and the cerebellum was also affected. The other also had bilateral high-signal lesions in the globus pallidus. There was little correlation between neurological deficits and MRI findings. A review of the literature revealed that 10 of the 13 patients with typical KSS previously studied had bilateral subcortical white-matter lesions on T2-weighted images; at least 7 also had high-signal lesions in the brain stem, globus pallidus, thalamus or cerebellum. Although MRI may be normal or show atrophy, the characteristic finding in KSS is a combination of the high-signal foci in subcortical cerebral white matter and in the brain stem, globus pallidus or thalamus. Received: 23 October 1998 Accepted: 8 February 1999     

Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet's disease from other central nervous system

Two neuroradiologists reviewed MRI studies of 34 patients with neuro-Behçet's disease (NBD), 22 with multiple sclerosis (MS) and 7 with systemic lupus erythematosus (SLE) with central nervous system involvement, masked to the clinical diagnosis, age and sex of the patients. Of the patients with NBD 12 were in an acute attack; the others had chronic disease. MRI was assessed using a set of criteria, looking at atrophy, the site of discrete parenchymal lesions, regions of predominant involvement and the extent of the lesion(s). The observers also made a guess at the clinical diagnosis. The brain stem and/or basal ganglia were the most predominantly involved sites in all patients with acute NBD; 75 % of these lesions were large and confluent, mainly extending from the brain stem to the diencephalon and basal ganglia. However, in chronic cases, the predominant involvement was in the brain stem and/or basal ganglia in only 36 %, and in cerebral hemisphere white matter in another 36 %; 27 % of these patients showed no parenchymal lesion. Hemisphere white-matter lesions were equally distributed between periventricular and other areas in NBD, while in MS more were periventricular, and in SLE more were nonperiventricular. Brain-stem atrophy was seen in 21 % of patients with NBD, with a specificity of 96.5 %. In the absence of cortical atrophy, its specificity was 100 %. The attempt at making a radiological diagnosis was successful in all cases of acute NBD and 95.5 % of patients with MS, but in only 40 % of patients with chronic NBD. Most of this latter groups MRI studies were interpreted as MS. An extensive lesion involving the brain stem and basal ganglia seemed to be diagnostic of acute NBD. However, hemisphere white-matter lesions could not be differentiated from those in MS.     

老年人皮质下动脉硬化性脑病的病理学基础和CT,MRI对照研究

本文就２６例皮质下动脉硬化性脑病患者的ＣＴ、ＭＲＩ检查结合病理学基础进行了分析。ＭＲＩ检查，当ＴＲ＝２０００ｍｓｅｅ，ＴＥ＝３０、６０ｍｓｅｃ时，皮质动脉硬化性脑病损害均为明显的高信号，Ｔ１加权像为低信号。ＣＴ像为低密度改变。Ｔ２加权像上半卵圆中心的白质表现为不均匀弥漫的高信号区，可累及基底节、丘脑、脑干及小脑的白质，并有不同程度的侧脑室扩大、脑室边缘呈斑片状改变可有脑萎缩。病理学特征是弥漫不完全     

Akute disseminierte Enzephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is an acute widespread autoimmune demyelinating condition, which principally affects the white matter of the brain and spinal cord. It usually follows an infection or vaccination. The typical presentation is that of multifocal neurologic disturbances accompanied by change in mental status. CSF analysis reveals lymphocytic pleocytosis and elevated protein content, but may also yield normal results. MRI is regarded as the diagnostic imaging modality of choice and typically demonstrates involvement of deep cerebral hemispheric and subcortical white matter as well as lesions in the basal ganglia, gray-white junction, diencephalon, brainstem, cerebellum and spinal cord. Unlike multiple sclerosis (MS), ADEM has a monophasic course and a favorable long-term prognosis.     

A controlled study of reliability and validity of MRI findings in neuro-Behçet's disease

Our aim was to test the reliability of interpreting MRI studies in neuro-Behçet's disease (NBD) and to determine the sensitivity and specificity of different MRI findings. We prospectively studied 50 patients: 24 had chronic NBD, 12 multiple sclerosis, 5 vasculitis other than Behçet's disease (BD) and 9 patients had BD without neurological involvement. MRI studies were performed according to a standard protocol with a 0.2 T imager. Two neuroradiologists, blinded to the diagnosis, age and sex of the subjects, reviewed the films independently, twice. Separate assessments were made for a set of items: dural sinus pathology, widening of ventricles and sulci, brain stem atrophy, lesions of the cerebral cortex, discrete lesions of deep white matter, basal ganglia, brain stem and cerebellum and the presence of smooth periventricular high-signal foci. Intraobserver agreement was substantial or better, and interobserver agreement moderate to substantial for most items. In these patients with chronic NBD we found low sensitivity on all assessed items. Dural sinus pathology or brain stem atrophy were highly specific, but parenchymal lesions in different sites had uniformly low specificity.     

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL): Assessment of the involved white matter tracts by MRI

Background and purpose

Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL) is a recently identified autosomal recessive disorder with early onset of symptoms and slowly progressive pyramidal, cerebellar and dorsal column dysfunction. LBSL is characterized by distinct white matter abnormalities and selective involvement of brainstem and spinal cord tracts. The purpose of this study is to assess the imaging features of the involved white matter tracts in cases of LBSL by MRI.

Patients and methods

We retrospectively reviewed the imaging features of the selectively involved white matter tracts in sixteen genetically proven cases of leukoencephalopathy with brainstem and spinal cord involvement and elevated brain lactate (LBSL). All patients presented with slowly progressive cerebellar sensory ataxia with spasticity and dorsal column dysfunction. MRI of the brain and spine using 1.5 T machine and proton magnetic resonance spectroscopy (¹H MRS) on the abnormal white matter were done to all patients. The MRI and MRS data sets were analyzed according to lesion location, extent, distribution and signal pattern as well as metabolite values and ratios in MRS. Laboratory examinations ruled out classic leukodystrophies.

Results

In all cases, MRI showed high signal intensity in T2-weighted and FLAIR images within the cerebral subcortical, periventricular and deep white matter, posterior limbs of internal capsules, centrum semiovale, medulla oblongata, intraparenchymal trajectory of trigeminal nerves and deep cerebellar white matter. In the spine, the signal intensity of the dorsal column and lateral cortico-spinal tracts were altered in all patients. The subcortical U fibers, globi pallidi, thalami, midbrain and transverse pontine fibers were spared in all cases. In 11 cases (68.8%), the signal changes were inhomogeneous and confluent whereas in 5 patients (31.2%), the signal abnormalities were spotty. MRI also showed variable signal abnormalities in the sensory and pyramidal tracts in addition to the brainstem and cerebellar connections. Proton MRS showed consistent elevation of the lactate within the abnormal white matter.

Conclusion

Distinct MRI findings in the form of selective affection of subcortical and deep white matter tracts of the brain (involving the posterior limb of internal capsules and sparing the subcortical U fibers), dorsal column and lateral cortico-spinal tracts of the spinal cord should lead to the diagnosis of LBSL supported by the presence of lactate peak in ¹H MRS. The disease can be confirmed by the analysis of the disease gene DARS2.     

鼻咽癌放疗后放射性脑病MRI评价

本文分析12例鼻咽癌放疗所致放射性脑病的CT和MRI表现。MRI检查采用SE序列(TR=1800ms,TE=99、132ms)和RARE序列,T_2加权成像。结果表明:MRI显示病变较CT敏感,以表现侧脑室旁白质内爪样高强度信号病灶为特征,颞叶病变最重,向上逐渐减轻,严重者累及颞叶灰质。作者认为MRI是放射性脑病的有效诊断手段。     

视神经脊髓炎脑部异常的MRI表现及相关危险因素分析

目的 分析视神经脊髓炎(NMO)脑部异常的MRI表现及特征,探讨脑内病灶发生的相关危险因素.方法 对符合2006年Wingerchuk诊断标准的54例NMO患者行脑部MR检查,分析脑部MRI异常患者病灶的分布及信号特点.采用Logistic回归分析评估脑内病灶发生的相关危险因素.结果 54例NMO患者脑部MRI正常24例(44.4%),MRI表现异常30例(55.6%),以多发的皮层下和皮髓质交界区白质小病灶最常见(13/30,43.3%).典型的NMO脑内病灶位于胼胝体、脑室室管膜下、下丘脑及脑干等部位,表现为斑点、斑片及线状异常信号.16例脑部增强检查均未见异常强化病灶.Logistic回归分析显示病程以及合并自身免疫病或前驱感染史与脑部MRI异常有关联(OR=3.519,P＜0.05).结论 NMO患者脑部MRI出现异常信号较常见并有较特异的好发部位.合并系统性自身免疫病或有前驱感染史的NMO患者容易出现脑部MRI异常.

Abstract：

Objective To investigate the MRI features of the brain in patients with neuromyelitis optica (NMO), and to evaluate the correlation between the brain abnormalities and related risk factors.Methods Fifty-four patients with definite NMO according to 2006 Wingerchuk diagnosis criteria were enrolled in this study. MRI scanning of the brain was performed in these patients. Distribution and signalfeatures of all the lesions were analyzed. A Logistic regression analysis was used to evaluate the risk factors of brain abnormalities. Results Twenty-four NMO patients (44. 4%) showed unremarkable findings and thirty (55.6%) showed abnormalities on brain MRI. Multiple and non-specific small lesions in the subcortical white matter and grey-white matter junction were the most frequent abnormalities on brain MRI (13/30, 43. 3%). Typical lesion locations included corpus callosum, subependyma of ventricles,hypothalamus and brain stem. The lesions showed punctate, patchy and linear abnormal signals. Postcontrast MRI showed no abnormal enhancement in 16 cases. Logistic regression analysis showed that coexisting anto-immune disease or infection history had correlations with abnormalities of the brain on MRI (OR=3.519,P ＜0.05). Conclusions There was a high incidence of brain abnormalities in NMO.Subependymal white matter, corpus callosum, hypothalamus and brain stem were often involved in NMO.NMO patients with coexisting anto-immune disease and infection history had higher risk of brain abnormalities.     

MRI findings in neuro-beh?et's disease.

AIM: To evaluate the pattern and site of involvement in neuro-Beh?et's disease (NBD). MATERIALS AND METHODS: Twenty-one patients with NBD were evaluated. Using 1.5T magnetic resonance imaging (MRI), T1-weighted axial and sagittal images, gadolinium enhanced axial and coronal images and T2-weighted axial images were obtained. RESULTS: The brainstem, basal ganglia, cerebral white matter, internal capsule, thalamus and spinal cord were involved in eighteen, nine, nine, seven, six and two patients, respectively. In nine patients with cerebral white matter involvement, four had subcortical involvement and three had periventricular involvement, in addition to two patients with focal deep white matter lesions. Among the brainstem lesions, pons involvement was seen in fourteen patients, all had ventrally located lesions, and nine had tegmental involvement. Midbrain involvement was seen in fourteen patients; the cerebral peduncle was involved in 11 of these. Five patients had brainstem atrophy: two cases were demonstrated at initial MRI, the other three cases were seen on follow-up MRI. Pyramidal signs, the most common neurological signs, were demonstrated in fourteen patients. Follow-up MRI was obtained 10 days to 20 months after the initial MRI in eight cases; all showed changes in size, shape and site of involvement. After gadolinium enhancement, thirteen patients demonstrated mottled non-confluent enhancement in the brainstem (eight patients), posterior limb of the internal capsule (three patients), pachymeninges (two patients) and spinal cord (two patients). CONCLUSION: NBD manifests a reversible course, but chronic NBD may result in brainstem atrophy. Characteristic involvement along the corticospinal tract is well correlated with neurological signs.