Pick’s disease with Pick bodies combined with progressive supranuclear palsy without tuft‐shaped astrocytes: A clinical,neuroradiologic and pathological study of an autopsied case

We report clinical, neuroradiologic features, and neuropathologic findings of a 76‐year‐old man with coexistent Pick’s disease and progressive supranuclear palsy. The patient presented with loss of recent memory, abnormal behavior and change in personality at the age of 60. The symptoms were progressive. Three years later, repetitive or compulsive behavior became prominent. About 9 years after onset, he had difficulty moving and became bed‐ridden because of a fracture of his left leg. His condition gradually deteriorated and he developed mutism and became vegetative. The patient died from pneumonia 16 years after the onset of symptoms. Serial MRI scans showed progressive cortex atrophy, especially in the bilateral frontal and temporal lobes. Macroscopic inspection showed severe atrophy of the whole brain, including cerebrum, brainstem and cerebellum. Microscopic observations showed extensive superficial spongiosis and severe neuronal loss with gliosis in the second and third cortical layers in the frontal, temporal and parietal cortex. There were Pick cells and argyrophilic Pick bodies, which were tau‐ and ubiquitin‐positive in neurons of layers II–III of the above‐mentioned cortex. Numerous argyrophilic Pick bodies were observed in the hippocampus, especially in the dentate fascia. In addition, moderate to severe loss of neurons was found with gliosis and a lot of Gallyas/tau‐positive globus neurofibrillary tangles in the caudate nucleus, globus pallidus, thalamus, substantia nigra, locus coeruleus and dentate nucleus. Numerous thorned‐astrocytes and coiled bodies but no‐tuft shaped astrocytes were noted in the basal ganglion, brainstem and cerebellar white matter. In conclusion, these histopathological features were compatible with classical Pick’s disease and coexistence with progressive supranuclear palsy without tuft‐shaped astrocytes.     

经皮激光椎间盘减压术治疗椎间盘源性腰痛

目的探讨经皮激光椎间盘减压术（percutaneous laser disc decompression，PLDD）治疗椎间盘源性腰痛的疗效。方法2002年6月～2004年12月我院对36例椎间盘源性腰痛，采用英国DIOMED公司半导体激光仪，激光功率15W，每个激光脉冲持续1s，间隔1s，照射能量800～1200J。VAS评分评价治疗效果。结果手术时间15～60min，平均30min。32例出现“疼痛复制效应”。36例随访6～36个月，平均11个月，32例有效（术后VAS评分改善≥3分18例，≥分14例），4例无效，有效率88．9％（32／36）。结论PLDD治疗椎间盘源性腰痛安全、有效、微创。     

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.     

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。     

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。     

经皮穿刺颈椎髓核成形术治疗多间隙颈椎间盘突出症

目的:探讨经皮穿刺颈椎髓核成形术(percutaneous cervical nucleoplasty,PCN)治疗多间隙颈椎间盘突出症的临床疗效及其对颈椎稳定性的影响。方法:对我院2003年7月～2005年6月因颈椎间盘突出症住院行PCN治疗的64例患者进行回顾性研究,根据手术间隙分为两组:单间隙组(A组)34例,多间隙组(B组)30例。临床效果评价采用JOA评分标准(17分制并计算改善率),颈椎稳定性评价采用Katsumi标准。比较两组的临床效果及颈椎稳定性。结果:随访6～24个月,A组平均14.1±3.7个月,B组平均13.9±3.8个月,A组术前JOA评分为9.19±2.64分,术后为14.37±1.42分,改善率为66.3%;B组术前JOA评分为8.89±2.31分,术后为14.33±1.28分,改善率为67.1%,与术前比较均有显著性差异(P<0.001),两组患者的JOA评分改善率无显著性差异(P>0.05)。两组患者术后颈椎稳定性与术前比较均无显著性差异(P>0.05)。结论:PCN治疗多间隙颈椎间盘突出症效果优良,对颈椎稳定性影响小,不会造成颈椎失稳。     

CT scan and threshold vibrometry in the diagnosis of spinocerebellar degenerations

45 patients with spinocerebellar degeneration (SCD) underwent through quantitative investigation of the ventricular and cisternal systems by CT scanning and threshold vibrometry in the limbs to find out whether these parameters could be used for distinguishing mainly spinal from cerebellar forms or from olivopontoce-rebellar atrophy. The increase in the vibration sense threshold and the mild atrophy in the posterior cranial fossa proved typical of spinal forms and the reverse for cerebellar forms. In olivopontocerebellar atrophy enlargement of the cisterns and dilatation of the ventricles always exceeded 85% of normal values.

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Sommario 45 pazienti affetti da degenerazione spinocerebellare (SCD) sono stati sottoposti ad uno studio approfondito di esplorazione quantitativa del sistema ventricolare e cisternale alla TAC e della soglia di percezione vibratoria agli arti per valutare la possibilità di distinguere con questi parametri le forme prevalentemente spinali da quelle cerebellari o delle atrofie olivopontocerebellari. L'aumento della soglia vibratoria e la modesta atrofia in fossa posteriore è risultata tipica delle forme spinali, mentre l'inverso si verifica per le forme cerebellari. Nelle atrofie olivopontocerebellari l'allargamento delle cisterne e la dilatazione dei ventricoli supera quasi sempre l'85% rispetto ai casi normali.

     

扩大的椎板间开窗治疗腰椎间盘突出伴侧隐窝狭窄（附140例报告）

本组采用的扩大的椎板开窗术治疗腰椎间盘突出合并侧隐窝狭窄140例。结果全部优良，与适应症严格选择和手术损伤小有关。强调切除卡压神经根的侧隐窝后壁外侧，即上关节突的冠状面．不失其脊椎三柱系统的三角结构。俯卧位头低足高，可减少硬膜囊张力避免负损伤。切除棘突下椎板时，用神经拉勾代替神经剥离器易于分离黄韧带，用90度的椎板咬骨钳斜向棘突方向，可满意地切除该部份椎板黄韧带，并可刮切椎板内层。神经根周围滴入醋酸炎舒松A2-3ml，术后疼痛锐减。减压窗口置盖明胶海绵有其争议，作者实践体会放置害处不大，要求病人有“护腰”意识。