31例脊髓亚急性联合变性临床分析

目的 总结脊髓亚急性联合变性（SCD）的病因、临床特征、诊断及治疗。方法 对31例临床确诊SCD患者的临床资料进行回顾性分析。结果 64.5%（19/31）的SCD患者有贫血,41.9%（13/31）患者血清VitB12水平降低,脊髓MRI检查发现20.0%（4/20）患者可见脊髓后索存在特异性异常信号,84.6%（22/26）患者肌电图有异常改变,体感诱发电位（SEP）检查阳性率100%,经VitB12治疗90.3%（28/31）患者临床症状均有不同程度好转。结论 SCD多由VitB12缺乏引起,试验性VitB12治疗有效,对明确诊断有重要意义;肌电图及SEP等检查可辅助诊断。     

27例脊髓亚急性联合变性临床分析

目的探讨亚急性联合变性(SCD)的病因、临床特征、MRI表现及预后。方法对2004年12月～2008年11月我院收治的27例SCD患者的临床资料进行回顾性分析。结果27例SCD患者主要表现为脊髓后索和侧索受损的症状。51.9%(14/27)伴有巨幼红细胞贫血(MA),平均红细胞体积(MCV)和平均红细胞血红蛋白(MCH)增高者分别为70.4%(19/27)和74.1%(20/27);81.5%(22/27)血清维生素B12(VitB12)水平降低;80.0%(12/15)患者肌电图有异常改变;76.2%(16/21)的患者脊髓MRI发现后索和(或)侧索特异性改变。应用甲钴胺治疗均有不同程度好转,MRI显示病灶消失或明显缩小。结论SCD的诊断除了典型的脊髓侧索、后索损害、贫血、血清VitB12水平降低外,还可借助于电生理、MRI检查;其病因涉及到VitB12吸收、摄取、转运、细胞内代谢及遗传障碍等多种因素。早期及时治疗可改善预后。     

亚急性联合变性的临床研究

回顾分析10例SCD临床表现及辅助检查。结果发现本组患者发病均由VitB(12)缺乏引起的，肢体感觉异常、深感觉减退和共济失调是SCD最常见的症状和体征。血清VitB(12)浓度测定、MR及SEP对诊断有重要作用。尽早明确诊断，尽早VitB(12)治疗，预后良好，治疗早期不主张合用叶酸。     

维生素B12缺乏与亚急性联合变性及巨幼细胞贫血

目的 :探讨维生素B12 (VitB12 )缺乏与亚急性联合变性 (SCD)和贫血的关系 ,讨论以神经系统病变为主要临床表现的VitB12 缺乏症的临床特点 ,以减少该病的误诊。方法 :对 17例SCD临床资料进行回顾性的分析。结果 :所有患者均有SCD常见的神经系统表现 ,多数病例有不同程度的贫血。结论 :SCD在我国并不少见 ;神经系统障碍与贫血之间无相关性 ;血清VitB12 测定、MR、电生理检查有助于本病的诊断 ;早期及时补充VitB12 ,疗效肯定 ,预后良好     

亚急性联合变性的早期诊断

目的 探讨急性联合变性（SCD）的临床及辅助检查特点,拟早期诊断和治疗。方法 对16例SCD的临床特点辅助检查结果进行回顾性分析。结果 16例SCD病人中脊髓后侧索均变性15例（93.57%),伴周围经变性15例（93.75％）,大脑白质变性2例（12.5％）,贫血8例(50%)。头颅CT检查白质低密度2例（2／8,25％）,脊髓MRI异常8例（8／8,100％）。血清维生素B12浓度降低4例（4／9,44.4％）。结论 具有典型临床特点的SCD不难诊断,不典型者脊髓MRI及血清VitB12浓度测定有助于早期诊断及鉴别诊断。     

不典型脊髓亚急性联合变性的临床特征分析

目的探讨不典型脊髓亚急性联合变性（SCD）临床特点和诊断思维。方法对46例SCD患者的临床资料行回顾性分析,并对13例SCD患者首诊误诊的临床资料行重点分析。结果在46例SCD患者中,脊髓后侧索和周围神经、侧索和周围神经、后索和周围神经及后侧索同时受损者分别占52．17％,10．87％,10．87％,10．87％,仅有后索或侧索受损者分别占8．70％,6．52％。血红蛋白（Hb）降低者占43．48％,平均红细胞体积（MCV）升高者占71．74％;血清VitB12降低者占50％（21／42）;脊髓MRI检查发现异常者占28．57％（6／21）。在13例首诊误诊患者中,4例仅有后索和3例仅有侧索受损,侧索和周围神经、后索和周围神经同时受损者各2例,后侧索、后侧索和周围神经同时受损者各1例;5例血清VitB12降低,脊髓MRI检查显示异常1例;首诊误诊率占28．26％（13／46）。结论不典型SCD主要临床特点可为仅累及后索或侧索的非联合性脊髓受损表现,或后索和侧索受损表现不均衡,VitB12等检查可正常。准确识别脊髓后侧索受损的阳性表现、查找体内VitB12缺乏的佐证、排他疾病及VitB12试验性治疗等是避免不典型SCD误诊的有效措施。     

19例亚急性联合变性临床分析

目的 探讨亚急性联合变性（SCD）的病因，临床特征，诊断及治疗。方法 对19例SCD患者的临床资料进行回顾性分析。结果 19例SCD患者主要表现为脊髓后索和侧索受损的症状，血红蛋白正常12例，降低7例，平均红细胞容积（MCV）和平均红细胞血红蛋白（MCH）增高分别为15例和17例，正常分别为4例和2例；血清维生素B12（VitB12)浓度，降低10例，正常8例，增高1例；电生理及MRI检查均有明显的改变。经治疗后临床症状消失12例，血清VitB12浓度恢复正常10例，电生理及MRI也有明显改善。结论 SCD的诊断除了典型的脊髓侧索，后索损害，贫血，血清VitB12含量减少外，还可借助于电生理，MRI检查；其病因涉及到VitB12吸收，摄取，转运，细胞内代谢及遗传障碍等多种因素，早期及时治疗可改善预后。     

MR对脊髓亚急性联合变性的诊疗价值

脊髓亚急性联合变性 (subacutecombineddegenerationofthespinalcord ,SCD)是维生素B1 2 缺乏引起的营养障碍性脊髓病变 ,主要是脊髓后索、侧索和周围神经变性产生相应的临床表现。MR能显示脊髓后索及侧索的变性。现报道 5例SCD的MR表现特点 ,以提高SCD的诊疗水平。1　资料1 1　一般资料 :1996年 4月至 2 0 0 2年 4月经临床、实验室和MR检查证实的 5例SCD病例 ,男 4例 ,女 1例 ,年龄 2 2～ 70岁 ,平均4 4 .6岁。患者均以下肢无力、行走不稳渐进性加重而就医。其中3例有跛行 3～ 6个月 ,3例下肢肌力下降至IV+ 级 ,5例内外踝振动觉消…     

雄生素B12缺乏与亚急性联合变性及巨幼细胞贫血

目的：探讨维生素B12（VitB12)缺乏与亚急性联合变性（SCD）和贫血的关系。讨论以神经系统病变为主要临床表现的VitB12缺乏症的临床特点，以减少该病的误诊。方法：对17例SCD临床资料进行回顾性分析。结果：所有患者均有SCD常见的神经系统表现，多数病例有不同程度的贫血。结论：SCD在我国并不少见；神经系统障碍与贫血之间无相关性，血清VitB12测定，MR，电生理检查有助于本病的诊断；早期及时补充VitB12，疗效肯定，预后良好。     

脊髓亚急性联合变性的临床特征分析

目的总结脊髓亚急性联合变性患者的临床表现及磁共振成像、肌电图的特点,并探索血中有关标志物水平与神经损害程度的相关性。方法回顾性分析2016-12—2018-05于河南省人民医院临床确诊的50例脊髓亚急性联合变性患者的临床特点,用Spearman秩相关分析血标志物水平与神经损害程度的相关性。结果首次发病年龄16~86(58.92±16.52)岁,其中60岁以上首次发病27例(54%),男34例(68%);50例患者中累及周围神经者31例(62%),后索者46例(92%),侧索者27例(54%),大脑白质与视神经损害10例(20%);36例(72%)患者行神经传导速度检查,25例(69%)有异常改变;50例患者全部行常规MRI检查,19例(38%)行常规加增强MRI检查,其中25例(50%)患者发现病灶;血清平均血红蛋白浓度、血清VitB12水平与神经功能损伤程度无显著相关性(P>0.05),血清平均红细胞体积、叶酸、同型半胱氨酸水平与神经功能损伤程度呈正相关(r>0),红细胞计数、血红蛋白、平均血红蛋白量、总蛋白、白蛋白与神经功能损伤程度呈负相关性(r<0)。结论SCD患者临床上以脊髓后索损害较为常见,辅助检查中神经电生理检查检出率高于MRI;且对于院前补充过VitB12或者功能性VitB12缺乏的SCD患者,血清中的其他标志物可以作为间接参考指标。     

Contribution of magnetic resonance imaging in sclerotic combined degeneration of the spinal cord due to vitamin B12 deficiency

Subacute combined degeneration (SCD) of the spinal cord is known to present histopathologically degenerative lesions in the spinal cord, but few studies on the neuroradiological findings have so far been reported. We present the interest of initial and follow-up MR findings in three cases of SCD. In the three cases, a causal event precipitated the onset of neurological symptoms: general anesthesia for the first and the third one and folic acid treatment for the second one. Clinical evolution was favorable after specific treatment with nearly total recovery. The initial MR study disclosed lesions predominantly involving the posterior columns of the spinal cord: high intensity on T2 weighted image was seen in the initial MR study and disappeared three months after treatment in correlation with good recovery, but with a delay. The recognition of this MR pattern suggests that MRI may be used in conjunction with clinical assessment to confirm the diagnosis and to monitor the efficacity of treatment in SCD.     

大剂量甲钴铵治疗亚急性联合变性的临床、影像及电生理分析

目的：观察亚急性联合变性（SCD）患者的临床特点、影像学（MRI）、电生理表现及甲钻铵治疗前后的变化,评价其诊断价值和疗效。方法：选取15例SCD患者,检测血常规和血清维生素B12、叶酸浓度,进行周围神经传导速度、体感诱发电位、视觉诱发电位、听觉诱发电位及脊髓MRI检查,观察治疗前后血红蛋白、血清维生素B12浓度、MRI及电生理的变化,并进行神经功能评定。结果：经甲钴铵治疗后患者临床症状明显改善,脊髓MRI显示的颈胸髓内后索、侧素长条状等T1长T2异常信号,治疗后多数病灶缩小或消失,电生理改变亦明显好转。结论：MRI诊断SCD准确度高;电生理检查敏感度高,且可发现亚临床病灶。两者结合可有助于SCD的早期明确诊断。大剂量甲钻铵静脉滴注可迅速提高血清维生素B12浓度,吸收利用度好,能有效改善SCD患者的神经功能。     

脊髓亚急性联合变性患者血清维生素B_(12)水平与临床表现的分析

目的探讨脊髓亚急性联合变性(SCD)患者的血清维生素B_(12)水平(正常或降低)与临床表现间的关系。方法回顾性分析18例血清维生素B_(12)水平降低的SCD患者(维生素B_(12)降低组)和12例维生素B_(12)水平正常或升高的SCD患者(维生素B_(12)正常组)的临床资料,比较两组在临床表现、血液学、电生理、影像及维生素B_(12)治疗效果方面的差异。结果 1维生素B_(12)降低组患者肌力较维生素B_(12)正常组显著减退(P=0.002);2维生素B_(12)降低组同型半胱氨酸(Hcy)平均值较正常组高,且Hcy升高比例高于维生素B_(12)正常组(P=0.020);3维生素B_(12)降低组的体感诱发电位异常率高于维生素B_(12)正常组(P=0.044);4两组脊髓MRI阳性发现率差异无显著性;5维生素B_(12)降低组对维生素B_(12)治疗效果好于维生素B_(12)正常组(P=0.006)。结论血清维生素B_(12)水平降低的SCD患者较维生素B_(12)水平正常患者病情更为严重;维生素B_(12)水平降低患者应用维生素B_(12)治疗的效果更好;血清维生素B_(12)降低不是诊断SCD的必要条件;高Hcy并非一项理想的SCD诊断指标;SCD的预后、疗效与病程有关,早期诊断、及时治疗至关重要。     

Diagnosis and clinical manifestations of subacute combined degeneration of the spinal cord： Analysis of 21 cases

BACKGROUND： Subacute combined degeneration of the spinal cord is caused by vitamin B12 deficiency and is a kind of degenerative disease owing the characteristics of nervous system diseases. In addition, different patients have variously clinical manifestations and various prognoses after vitamin B12 therapy. OBJECTIVE： To investigate and analyze diagnosis, clinical manifestations and prognosis of subacute combined degeneration of the spinal cord. DESIGN： Case analysis. SETTING： Department of Neurology, the Third Hospital of Peking University. PARTICIPANTS： A total of 21 subacute combined degeneration of the spinal cord patients including 14 males and 7 females aged from 33 to 82 years were selected from Department of Neurology, the Third Hospital of Peking University from January 1999 to December 2005. Duration from onset to final diagnosis lasted for 1.5 - 108 months. All patients had typically clinical manifestations; meanwhile, level of serum vitamin B12 was decreased and/or vitamin B12 therapy was effective. All patients provided the confirmed consent. METHODS： Clinical data of 21 subacute combined degeneration of the spinal cord patients were retrospectively analyzed, while general data and clinical characteristics were recorded at the same time. Levels of blood routine, serum vitamin B12 and homocysteine were measured at the phase of hospitalization. Normal value of serum vitamin B12 was 187 - 1 059 ng/L and normal value of serum homocysteine was 5 - 15μ mol/L. All patients received neuroelectrophysiological examination and 15 patients received MRI examinations of spinal cord. After final diagnosis, patients were given vitamin B12 therapy. And follow-up was performed to investigate the prognosis. MAIN OUTCOME MEASURES： （1） Levels of blood routine, serum vitamin B12 and homocysteine; （2） results of neuroelectrophysiological examination; （3） results of MRI examination of spinal cord; （4） prognosis. RESULTS： Clinical data of 21 patients and follow-up data of 20 patients were involved in the final analysis and 1 patient was lost because of living in the other province. （1） Clinical manifestations： All 21 patients had typically clinical manifestations. The original symptoms included numbness of lower and/or upper limbs （5 cases）, unstable gait （3 cases）, limb asthenia （4 cases）, limb numbness combined with light asthenia （5 cases）, limb numbness combined with unskillful activity （3 cases）, and limb numbness combined with unstable gait （1 case）. （2） Experimental results： Eight subacute combined degeneration of the spinal cord patients accompanied with mild-severe anemia and mean corpuscular volume of 13 patients were increased. Among 13 subacute combined degeneration of the spinal cord patients not administrating vitamin B12 before hospitalization, the levels of serum vitamin B12 of 2 patients were not measured but those of other patients were decreased. After vitamin B12 therapy,the levels of serum vitamin B12 of 8 patients were normal or increased. In addition, the levels of serum homocysteine of 6 patients were not measured but those of 7 patients were increased. While, the levels of homocysteine of 5 following-up patients were normal. The levels of serum vitamin B12 of 8 patients who received with vitamin B12 therapy before hospitalization were normal or increased. Among them,the levels of bomocysteine were not measured in 4 patients, those of 3 patients were increased, and that of 1 patient was normal. （3） Results of neuroelectrophysiological examination： Among all patients, 95% （20/21） patients had abnormal sensory-evoked potential, 89% （8/9） patients had abnormal motor evoked potential, 67% （10/15） patients had abnormal nerve conduction, 13% （2/15） patients had neurogenic muscle injury showed by electromyography （EMG）, 70% （7/10） patients had abnormal brain-stem auditory evoked potential, and 40% （4/10） patients had abnormal visual evoked potential. （4） Results of MRI examination of spinal cord： MRI examination demonstrated that 40% （6/15） patients had spinal cord lesion, but spinal cord lesion disappeared in 2 patients during follow up. In addition, clinical manifestations of patients were improved after standard vitamin B I2 therapy. CONCLUSION： Nervous system lesion caused by vitamin B 12 deficiency is not only involved in spinal cord, also in peripheral nerve, optic nerve, auditory pathway, etc. Diagnosis of the lesion depends on clinical characteristics and level of serum vitamin BI2. Especially, neuroelectrophysiological examination, measurement of homocysteine and MRI examination of spinal cord are beneficial for diagnosis and evaluation of therapeutic effects.     

NEUROPHYSIOLOGICAL DETERMINATION OF THE CARPAL TUNNEL SYNDROME IN PATIENTS WITH CHRONIC DEMYELINATING POLYNEUROPATHY

Clerici R., De Riz M., Corrà B., Baron P.L., Scarpini E., Conti G., Scarlato G.
Department of Neurological Sciences, Univ. of Milan, IRCCS Ospedale Maggiore, Milano
The specific spinal cord lesion caused by vitamin B12 deficiency is known as subacute combined degeneration (SCD). This is a rare cause of demyelination of the dorsal and lateral columns of spinal cord and even more rarely of peripheral nerves, optic nerve and brain.
We report a case of SCD in a vegetarian 55-year-old fe- male who presented with 6 weeks history of gradually progressive paresthesia involving thoracoabdominal skin below C5 dermatome and both lower limbs and postural instability due to a mild impairment of deep sensation. Hematological tests revealed abnormal Medium Corpuscular Volume (MCV) without anaemia and low vitamin B12 levels. The cause of this deficiency was secondary to atrophic gastritis. A spinal cord MRI demonstrated two T2-weighted hyperintense signal alterations (C1-C3 and C3-C4). She was treated with parenteral vitamin B12 supplements and experienced gradual improvement in her clinical symptoms. Repeat MRI of cervical spinal cord after 5 months showed a relevant decrease in the areas of abnormal signal. In other documented cases reported in literature, there was a precocious clinical improvement, while the MRI lesions recovered with a delay. However, it is essential to recognize SCD among the different demyelinating diseases and treat it as soon as possible.     

磁共振T2加权成像的扫描方位和层厚对亚急性联合变性脊髓内病变显示的影响

目的探讨不同方位及不同层厚横断面磁共振成像(MRI)T2加权像(T2WI)对亚急性联合变性(SCD)病变显示的影响。方法 24例SCD患者接受了颈髓及胸髓的MRI平扫及增强扫描,T2WI序列包括3mm层厚矢状面、3mm和7mm层厚横断面,经3名放射科医师分别阅读了所有MRI图像。结果 24例患者3mm层厚横断面T2WI像均可见脊髓后索高信号〔显示率为100%(24/24)〕,13例显示侧索病变〔显示率100%(13/13)〕;17例在矢状面T2WI像显示后索病变(显示率为70.8%);7mm层厚的横断面T2WI像显示19例后索病变〔显示率为79.2%(19/24)〕和6例侧索病变〔显示率46.2%(6/13)〕。结论对于SCD脊髓内病变的显示,横断面T2WI优于矢状面T2WI,3mm层厚的横断面T2WI优于7mm层厚的横断面T2WI。     

自发性硬脊膜外血肿的诊断和治疗策略

目的探讨自发性硬脊膜外血肿的临床特征、治疗及影响预后的因素。方法对16例自发性硬脊膜外血肿患者的临床特征、手术治疗时机以及手术后神经功能恢复情况进行回顾总结,并结合文献分析影响预后的因素。所有自发性硬脊膜外血肿患者均行MRI检查。结果16例患者的硬脊膜外血肿分别位于下颈段（2例）、颈胸段（6例）、胸段（7例）及胸腰段（1例）。MRI检查T1WI表现为等信号或略高信号,T2WI以高信号为主,其中可见混杂低信号。12例施行手术治疗的患者中10例预后良好;4例保守治疗者中3例神经功能完全恢复。结论脊髓MRI检查是诊断自发性硬脊膜外血肿的首选方法,早期诊断和外科手术治疗是恢复神经功能、提高疗效的关键。手术疗效主要与自发性硬脊膜外血肿患者手术前的神经功能缺损程度和手术间隔时间有关;症状较轻者在密切观察下可予以保守治疗,其神经功能的恢复主要取决于神经功能缺损程度。目的探讨自发性硬脊膜外血肿的临床特征、治疗及影响预后的因素。方法对16例自发性硬脊膜外血肿患者的临床特征、手术治疗时机以及手术后神经功能恢复情况进行回顾总结,并结合文献分析影响预后的因素。所有自发性硬脊膜外血肿患者均行MRI检查。结果16例患者的硬脊膜外血肿分别位于下颈段（2例）、颈胸段（6例）、胸段（7例）及胸腰段（1例）。MRI检查T1WI表现为等信号或略高信号,T2WI以高信号为主,其中可见混杂低信号。12例施行手术治疗的患者中10例预后良好;4例保守治疗者中3例神经功能完全恢复。结论脊髓MRI检查是诊断自发性硬脊膜外血肿的首选方法,早期诊断和外科手术治疗是恢复神经功能、提高疗效的关键。手术疗效主要与自发性硬脊膜外血肿患者手术前的神经功能缺损程度和手术间隔时间有关;症状较轻者在密切观察下可予以保守治疗,其神经功能的恢复主要取决于神经功能缺损程度。