Freezing of gait in postmortem-confirmed atypical parkinsonism.

The frequency and pathophysiology of freezing of gait (FoG) in atypical parkinsonism is unknown. We analysed the frequency of FoG in postmortem-confirmed atypical parkinsonian disorders (APD) comprising corticobasal degeneration (CBD), dementia with Lewy bodies (DLB), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Sixty-six patients with pathologically confirmed APD (CBD, n = 13; DLB, n = 14; MSA, n = 15; PSP, n = 24) formed the basis for a multicenter clinicopathological study. Clinical features at first and last clinical visit were abstracted from patient records on standardized forms following strict instructions. At the first visit (median 36 months after symptom onset), 24% of APD had FoG (CBD, 8%; DLB, 21%; PSP, 25%; MSA, 40%). Logistic regression analysis showed a significant association of FoG and urinary incontinence (P = 0.04) at first visit. At last visit, 47% of APD had FoG (CBD, 25%; PSP, 53%; DLB, 54%; MSA, 54%). Clinicopathological correlation based on routine postmortem examination failed to identify a consistent neuropathological substrate of FoG. This study demonstrates that (1) FoG is common in APD, and (2) urinary incontinence is significantly associated with FoG in these disorders. Whether FoG and urinary incontinence share similar neuropathological substrates remains to be determined by future studies.     

腰椎管上下缘矢径的测量及腰椎管狭窄的诊断

用２‰游标卡尺对１１４具Ｌ１～Ｌ５段椎骨标本进行测量，包括椎孔上缘矢径及下缘矢径的距离和相互关系。结果：腰椎孔下缘矢径明显大于上缘矢径（Ｐ＜０．０１），腰椎孔下缘矢径明显大于腰椎孔中矢径。为腰椎管狭窄的临床诊断提供解剖学基础。     

Changes in glial fibrillary acidic protein mRNA expression after corticospinal axotomy in the adult hamster

We examined changes in the expression of glial fibrillary acidic protein (GFAP) mRNA during Wallerian degeneration in the corticospinal system of the adult Golden hamster following axotomy. GFAP is the product of a type III intermediate filament (IF) gene that is expressed specifically in mature astrocytes. A well-studied component of a complex response termed reactive astrogliosis that occurs after various types of CNS injury is the increased production of astrocytic processes filled with GFAP-containing IFs. While increased expression of GFAP during reactive astrogliosis has been well established at the protein level, little is known about whether or not changes in GFAP mRNA levels occur after CNS injury. In the present study we used in situ hybridization methods to examine this issue. A 35S-labeled mouse GFAP cDNA probe was used for in situ hybridizations of sections of the brain stem obtained 2, 7, and 14 days after unilateral transections of the corticospinal tract in the caudal medulla. Film as well as emulsion autoradiography showed a dramatic increase in GFAP mRNA labeling associated with the degenerating corticospinal tract. GFAP mRNA levels were already dramatically increased in the injured corticospinal tract by 2 days post axotomy and remained elevated at 14 days. Interestingly, in addition to the robust increase in GFAP mRNA levels specifically associated with the degenerating tract, a diffuse increase in GFAP mRNA labeling was observed throughout the grey matter of the brain stem at 2 days post-axotomy, but not after this time. Immunoblotting and immunocytochemical experiments verified that the increased GFAP mRNA levels in the degenerating corticospinal system were accompanied by an increased expression of the protein. These results demonstrate that an increase in GFAP mRNA levels occurs during Wallerian degeneration in the CNS and suggest that increased expression of the GFAP gene is a major contributor to CNS scarring that results after direct traumatic injury.     

儿童颈椎间盘钙化症的影像诊断

目的 探讨儿童颈椎间盘钙化症的影像表现及诊断价值，并提高对本病的认识。方法 回顾分析16例儿童颈椎钙化性椎间盘病的影像表现。结果 16例患均行颈椎平片检查，5例行CT扫描并作了三维重建，8例行MRI检查。16例平片均显示了椎间盘钙化灶，均为单发，CT平扫加三维重建显示4例钙化呈卵圆形，1例钙化为不规则形。MRI检查不仅显示了椎间盘内改变还清晰显示了病变相邻椎体边缘信号改变，在T1、T2加权像上呈低信号。结论 平片检查可以显示椎间盘钙化；CT平扫加三维重建能清晰显示了钙化灶的大小、形态；MRI检查在显示受累椎体及周围组织的改变方面明显优于CT和平片。     

经皮穿刺髓核成形术治疗腰椎间盘突出症初步临床报道

目的　寻求一种简单、安全、有效、微创的椎间盘突出症治疗方法。方法　对 1 6例腰椎间盘突出症采用经皮穿刺髓核成形术 (Nucleoplasty)治疗 ,并对其疗效进行观察分析。 结果　全部患者经二周至一个月短期随访 ,症状均有不同程度改善 ,疗效优良率为 93 8% ,有效率为 1 0 0 %。未发现明显并发症。结论　髓核成形术是一种先进、安全、有效的椎间盘突出微创手术 ,具有操作简单、安全、微创、疗效佳、恢复快、无需住院等优点。     

非孕期子宫肌瘤红色变性40例分析

目的 了解非孕期子宫肌瘤红色变性的临床特征。方法 本组回顾分析了1993年1月～2001年2月间住院手术治疗40例非孕期子宫肌瘤红色变性的临床病理特征。结果 非孕期子宫肌瘤红色变性发生率为2.3%。生育年龄多见，绝经后也可发生。有典型表现者2例，占5%，非典型下腹隐痛而无发热者7例，占17.5%，肌瘤结节局部压痛者12例，占30%，无任何症状者30例，占75%，白细胞升高占10例，25%。90%的红色变性肌瘤直径在5cm以上，77.5%为壁间肌瘤。68.6%有典型牛肉样改变，54.8%旋涡状结构存在。65%为单纯红色变性，余35%可合并其他类型变性。结论 非孕期子宫肌瘤红色变性临床过程缓和，症状不典型，易漏诊，不作为手术指征，但因子宫肌瘤往往较大，多需手术治疗。     

Percent spinal canal compromise on MRI utilized for predicting the need for surgical treatment in single-level lumbar intervertebral disc herniation

BACKGROUND CONTEXT: There is limited information describing the correlation between the initial quantitative measurements on magnetic resonance imaging (MRI) scans of disc herniation area, canal cross-section areas, percent canal compromise, and disc herniation location to the need for surgery. PURPOSE: Our aim is to determine if the size of disc herniation area, canal cross-section area, percent canal compromise, and disc herniation location taken from MRI images of patients with symptomatic single-level lumbar herniated intervertebral discs upon initial presentation to a spine surgeon, were predictive of the need for surgical treatment. STUDY DESIGN/SETTING: This is a retrospective case matched study of patient MRI images in the senior author's private practice. PATIENT SAMPLE: From a pool of 332 patients with sciatica caused by lumbar intervertebral disc herniations at our institution, 65 patients had surgery, of which MRI images were available and analyzed on 44 patients. Forty-four additional patients were randomly selected from the remaining 267 original group as nonoperative controls. METHODS: The axial MRI image showing the largest canal compromise by the herniated disc was selected for measurements. Using T1- and T2-weighted images, the areas of interest were digitally scanned at high resolution. The canal area and disc herniation area measurement were calculated from the total number of pixels per cross-sectional area, multiplied by a scan correction factor, mm(2) /pixel. Disc herniation locations were classified into either central or paracentral. The percent canal compromise was obtained by disc herniation area divided by canal cross-section area and multiplied by 100. RESULTS: The surgical group's overall mean herniated disc area was 219.6 square millimeter (mm(2)), 179.8 at L4-5, and 267.4 at L5-S1. The nonoperative group's overall mean herniated disc area was 178.4 mm(2), 135.1 at L2-3, 160.3 at L4-5, and 207.4 at L5-S1. The surgical group's overall mean canal cross-sectional area was 471.8 mm(2), 418.6 at L4-5, and 535.6 at L5-S1. The nonoperative group's overall mean canal cross-sectional area was 541.3 mm(2), 518.1 at L2-3, 446.8 at L4-5, and 669.9 at L5-S1. The overall percent canal compromise ratio in the surgery group was 46.7%, 44.1% at L4-5, and 49.8% at L5-S1. The overall percent canal compromise in the nonoperative group was 34.2%, 34.1% at L2-3, 36.1% at L4-5, and 31.8% at L5-S1. The percent canal compromise in central herniations at L4-5 level was 53.0% in the surgical group, and 32.8% in the nonoperative group; at the L5-S1 level surgical group percent canal compromise was 64.1% and in the nonoperative group canal compromise was 27%. L4-L5 level paracentral herniations canal compromise was 36.7% in the surgical group compared with 42.5% canal compromise in the nonoperative group. At the L5-S1 level the canal compromise was 45% in the surgical group and 34.8% in the nonoperative group. CONCLUSIONS: Our findings show a trend for patients treated with surgery to have larger disc herniation areas and smaller canal cross-section areas, corresponding to larger percent canal compromise than the nonoperative group. Centrally located herniations followed this trend closely at all levels studied. However, the paracentral herniation at the L4-5 level does not follow this trend, possibly because paracentral disc herniation clinical course is determined more by herniation location rather than the overall herniation size.     

肝豆状核变性的治疗进展

阐述肝豆状核变性（HLD）在药物、饮食、外科和分子生物学方面的治疗进展，介绍了HLD的药物治疗、外科治疗和分子生物学治疗中的新方法或新技术：以DMPS等为主的药物治疗仍是治疗HLD的主体方法，肝移植等是治疗HLD中的暴发性肝功能衰竭的首选方法，基因等治疗为HLD的彻底治疗提供了可能。     

腰椎节段形态对峡部、小关节应力及小关节接触力影响的有限元分析

目的:探讨腰椎形态结构变化对峡部裂性滑脱及小关节退变的作用和意义。方法:采用改良的“非种子区域分割方法”及非平行“最佳切割平面”等一系列新型计算机辅助设计(CAD)方法精确建立包括椎间盘高度、腰椎小关节角、椎间盘前凸角改变的L4~L5活动节段有限元模型;在2700N轴向压缩载荷条件下,分别对各有限元模型的峡部、小关节应力、小关节接触力以及椎间盘负载进行测试。结果:压缩载荷下,腰椎活动节段峡部、小关节等效应力及小关节接触力随椎间盘高度的减小而减小,随小关节角的增大而增加,随椎间盘前凸角的增加而减小。结论:椎间盘高度、腰椎小关节角、椎间盘前凸角等形态结构变化对腰椎节段有限元模型的峡部、小关节应力及小关节接触力有明显的影响。提示腰椎峡部应力性骨折及小关节退变的发生与椎间盘高度、腰椎小关节角、椎间盘前凸角等解剖形态因素有关。     

腰椎间盘突出症合并椎管狭窄23 例治疗分析

目的探讨经后路椎间盘镜手术治疗合并腰椎管狭窄症的椎间盘突出症的临床应用。方法采用后路椎间盘镜进行单侧开窗减压术。通过术前标记腰椎正侧位片定位，于定位棘突间隙后正中偏患侧作长约1.5 cm小切口，逐级扩张后置入工作通道管，钻除部分椎板，置入内窥镜，于电视监视器下显露椎板、增生内聚的关节突、肥厚的黄韧带及突出的椎间盘髓核组织，彻底解除其对硬脊膜、神经根的压迫。结果本组共治疗合并腰椎管狭窄症的腰椎间盘突出症23例，平均随访7个月，按Prolo标准评定，治愈20例，有效2例，无效1例。结论本术式在严格掌握适应证前提下对合并腰椎管狭窄症的腰椎间盘突出症患者效果明显。