脊柱侧弯合并椎管内病变的一期手术治疗

目的探讨一期手术治疗脊柱侧弯合并椎管内病变的有效性和安全性。方法与结果共6例脊柱侧弯合并椎管内病变患者均完成一期手术治疗,手术成功率为100%。手术时间(470.83±136.20)min,术中出血量1350(625,2150)ml,融合椎体节段(11.00±2.76)个;术后冠状位侧凸角度(19.60±5.94)°,矢状位后凸角度(25.80±10.87)°,均较术前改善(P=0.007,0.005);脊髓拴系行脊髓拴系松解术,表皮样囊肿、节细胞胶质瘤和椎管内脂肪瘤行椎管内病变切除术,脊髓空洞症不予特殊处理;无神经功能缺损恶化;术后下肢肌力增高,肌张力降低;排尿障碍改善。术后住院(8.83±3.31)d,无一例出现感染、脑脊液漏、脊柱内固定失败、椎弓根螺钉和钛棒断钉断棒等手术相关并发症,无一例死亡。术后随访(7.50±1.22)个月,均无神经功能缺损恶化、迟发性感染和假关节形成,均未出现矫形丢失。结论一期手术可以安全、有效地治疗脊柱侧弯合并椎管内病变,术后神经功能改善,通过截骨可以获得良好的矫形效果。     

Case series of two patients with Fibrocartilaginous Embolism mimicking Transverse Myelitis of the Spinal Cord

Fibrocartilaginous Embolism (FCE) refers to the extrusion of some of the fibro-cartilaginous nucleus pulposus material from within the inter-vertebral disc to eventually embolize into one of the spinal cord vessels with resultant spinal cord infarction. According to a 2016 review, AbdelRazek et al. (2106) [1] there are 41 pathologically confirmed and 26 clinically suspected cases in the literature till the end of 2015. We add two more clinically diagnosed cases.     

High resolution nuclear magnetic resonance imaging of the spinal cord in experimental demyelinating disease

Summary Chronic recurrent experimental allergic encephalomyelitis was induced in a strain 13 guinea pig by inoculation of isologous spinal cord homogenate. The spinal cord was obtained after perfusion with 4% paraformaldehyde and examined with nuclear magnetic resonance (NMR) imaging. Proton NMR spin echo images (repetition time: 3 s; echo times: 20 and 60 ms) were obtained from intact, isolated spinal cord in a 4.7 Tesla, 50 mm bore magnet. The slice thickness of the images was 380 m and the inplane resolution was 40×40 m. The images showed superficial areas of low signal intensity in the lateroventral regions of the white matter, in some instances with a seam of higher signal intensity. Neuropathologically, these abnormalities corresponded exactly to areas of demyelination. Control images did not show these abnormalities. The present high resolution imaging allowed a correlation between demyelination and abnormal NMR signals in a small laboratory animal with an inflammatory demyelinating disease.Supported by the Belgian Foundation of Medical Scientific Research (FGWO, grant 3.0096.86 and grant 3.0019.86), by the Institute for the promotion of Scientific Research in Industry and Agriculture (IWONL) and by the Scientic Research Planning Office of the Belgian Government (DPWB), contract no. 87/92-120     

Cervical spinal cord infarction associated with coronavirus infectious disease (COVID)-19

Coronavirus disease (COVID-19) has a number of emerging neurological manifestations in addition to pneumonia and respiratory distress. In what follows, we describe a case of a previously healthy young man with severe COVID-19 who subsequently developed an acute flaccid paralysis. Work up revealed a lesion in his cervical spinal cord concerning for spinal infarction or transverse myelitis. He received empiric pulsed steroids without improvement. Taken together, we felt his presentation was most consistent with spinal cord infarction in the setting of critical illness with COVID-19. We believe this is a rare case of spinal cord stroke associated with COVID-19.     

Natural history and management of spinal cord arteriovenous shunts in pregnancy: A monocentric series of 10 consecutive cases with emphasis on endovascular treatment

PurposeIntradural spinal cord arteriovenous shunts (IDSCAVS) are rare and constitute a challenging situation if symptoms occur during pregnancy. We present a series of ten such cases referred to our center: five cervical, four thoracic and one lumbar.MethodsWe retrospectively reviewed our global series of 215 IDSCAVSs between 2002 and March 2020 and found ten patients who had presented during pregnancy. Clinical, radiological and therapeutic data were studied.ResultsSeven shunts were AVM type niduses and three were micro AV-fistulae. All were associated with pial venous reflux and six hemorrhagic cases had pseudo aneurysms. Symptoms occurred mainly during the third trimester, 80% of patients presented with hemorrhage and spinal cord dysfunction. We embolized seven patients and proposed surgery in one, always after delivery: all recovered well. One woman declined treatment; one other was operated in emergency but did not improve. Mean follow-up was 3.9 years (0.5...19 years).ConclusionsDespite this small group of patients, our initial experience of IDSCAVSs diagnosed during pregnancy indicates that embolization is an effective management strategy if performed after delivery and a recovery period. Results indicate that IDSCAVSs seem to have a low risk of early rebleedings after the ictal event and may be closely followed up until delivery. The results obtained show good clinical outcome without long-term rebleeds. Women with known IDSCAVSs should not be discouraged from becoming pregnant, however it seems wise to embolize them before pregnancy in order to offer protection against risks during pregnancy.     

Distribution of sodium channels in chronically demyelinated spinal cord axons: immuno-ultrastructural localization and electrophysiological observations

The immuno-ultrastructural localization of voltage-sensitive sodium channels was demonstrated within a central demyelinating lesion induced in the rat spinal cord by ethidium bromide/irradiation using polyclonal antibody 7493. Antibody 7493 has previously been shown to immunostain intensely axon membrane at nodes of Ranvier, and also perinodal astrocyte processes. At 25–35 days post injection/irradiation, the central portion of the demyelinating lesion is populated with chronically demyelinated axons and there is an absence of glial processes. Sodum channel immunoreactivity was not observed on the chronically demyelinated axolemma within this central portion of the lesion. Within the peripheral portion of the lesion demyelinated axons were occasionally abutted by astrocyte and Schwann cell processes. At these focal sites of apposition, the axon membrane displayed intense sodium channel immunoreactivity, while the abutting astrocyte and Schwann cell processes did not exhibit immunostaining. Also in the periphery of the lesion, some axons become ensheathed and myelinated by oligodendrocytes and Schwann cells. The axon membrane of circumferentially ensheathed axons displayed antibody 7493 immunostaining, and this immunoreactivity persisted on the axolemma until the ensheathing cytoplasmic processes compacted into myelin. Internodal axon membrane beneath the myelin sheath did not display sodium channel immunoreactivity, though (putative) developing nodal axon membrane adjacent to terminal paranodal loops exhibited robust sodium channel staining. Electrophysiological recordings within the ethidium bromide/irradiation lesion demonstrated that at least some axons conducted action potentials within the lesion, while others exhibited conduction block. These results indicate that there is a reorganization of sodium channels within the axon membrane of chronically demyelinated central axons.     

Altered levels of circulating insulin and other neuroendocrine hormones associated with the onset of schizophrenia

Recently, we showed that the circulating levels of insulin-related peptides and the secretory granule protein chromogranin A were increased in small cohorts of first onset schizophrenia patients. Assuming that this effect was associated with impaired insulin signalling, we investigated the possibility that secretion of other hormones is also affected in schizophrenia. Multiplex immunoassay analysis of 21 hormones and hormone-related molecules was carried out using sera from 236 first and recent onset schizophrenia patients and 230 matched controls. Serum concentrations of insulin and chromogranin A were increased in schizophrenia subjects, consistent with our previous study. In addition, we found elevated concentrations of pancreatic polypeptide, prolactin, progesterone and cortisol, and decreased levels of growth hormone. We also found that growth hormone levels were decreased in post-mortem pituitaries obtained from chronic schizophrenia patients. It will be important to determine whether any of these molecules are involved in the pathosphysiology of schizophrenia or if they reflect the associated insulin resistance. We conclude that function of multiple components of the hypothalamic-pituitary-adrenal-gonadal axis may be affected in schizophrenia. This could have important implications for future biomarker discovery efforts and personalized medicine strategies based on patient stratification for the treatment of this debilitating disorder.     

多发性硬化脊髓受累临床与MRI对照研究

目的分析多发性硬化脊髓受累患者临床表现及MRI影像学特点,并探讨其临床诊断意义。方法回顾分析2006年1月-2009年12月住院治疗的46例多发性硬化脊髓受累患者的临床资料及影像学表现。结果起病形式以急性（58.70%,27/46）或亚急性（34.78%,16/46）为主,临床主要表现为肢体瘫痪（95.65%,44/46）、感觉障碍（84.78%,39/46）和尿潴留（67.39%,31/46）。MRI受累部位以颈髓最常见（45.65%21/46）,其次为胸髓（28.26%,13/46）,呈脊髓内单一或散在多发长T₁、长T₂斑片状异常信号影,病灶长度一般不超过2个椎体节段（84.78%,39/46）,个别患者（15.22%,7/46）病灶长度超过2个椎体节段;增强扫描可有不同程度强化（78.26%,36/46）。结论多发性硬化脊髓受累患者临床表现复杂多样,MRI脊髓受累可呈现单一或散在多发病灶,病灶长度较少超过2个椎体节段,但病灶节段延长不能排除多发性硬化。MRI是诊断多发性硬化脊髓受累最敏感和最特异的影像学检查方法。     

Primary germinoma of the spinal cord: a case report with 28-year follow-up and review of the literature

Germ cell neoplasms occur in extra-gonadal midline locations of the retroperitoneum, mediastinum, pineal gland, areas of the suprasellar cistern, and rarely in the spinal cord. We recently reviewed a case of an unresectable lumbar spinal cord tumor in a 16-year-old female previously diagnosed as metastatic poorly differentiated carcinoma. An extensive evaluation for a primary neoplasm at that time was unsuccessful and the patient was treated with local radiation therapy. Recently, additional histochemical and immunocytochemical studies were performed on the archival formalin-fixed paraffin-embedded material and the clinical history was reviewed. These ancillary studies (including positive immunohistochemical staining for placental alkaline phosphatase) support a diagnosis of intramedullary germinoma of the conus medullaris. This patient has enjoyed 28 years of disease free survival which reflects the radiosensitive nature of this neoplasm. These data lend support to the existence of a primary germinoma in the spinal cord and illustrate the utility of using histochemical stains and immunohistochemistry to assist in diagnosing this treatable neoplasm.     

多发性硬化脊髓受累临床与MRI对照研究

目的分析多发性硬化脊髓受累患者临床表现及MRI影像学特点,并探讨其临床诊断意义。方法回顾分析2006年1月-2009年12月住院治疗的46例多发性硬化脊髓受累患者的临床资料及影像学表现。结果起病形式以急性（58．70％,27／46）或亚急性（34．78％,16／46）为主,临床主要表现为肢体瘫痪（95．65％,44／46）、感觉障碍（84．78％,39／46）和尿潴留（67．39％,31／46）。MRI受累部位以颈髓最常见（45．65％,21／46）,其次为胸髓（28．26％,13／46）,呈脊髓内单一或散在多发长T。、长T：斑片状异常信号影,病灶长度一般不超过2个椎体节段（84．78％,39／46）,个别患者（15．22％,7／46）病灶长度超过2个椎体节段;增强扫描可有不同程度强化（78．26％,36／46）。结论多发性硬化脊髓受累患者临床表现复杂多样,MRI脊髓受累可呈现单一或散在多发病灶,病灶长度较少超过2个椎体节段,但病灶节段延长不能排除多发性硬化。MRI是诊断多发性硬化脊髓受累最敏感和最特异的影像学检查方法。     

多发性硬化患者颈髓扩散张量成像研究

目的 通过扩散张量成像研究多发性硬化患者常规MRI表现正常脊髓(NASC)的改变,并探讨其临床应用价值.方法 采用平面回波成像技术对13 例多发性硬化患者和13 例健康志愿者施行颈髓扩散张量成像检查,分别测量第2 ~ 5 颈椎(C2 ~ 5)水平前索、后索、侧索及灰质兴趣区的部分各向异性(FA)值及平均扩散率(MD)值,比较两组之间所存在的差异性;相关分析检验多发性硬化组患者FA值和MD 值与扩展残疾状态量表(EDSS)评分之间的关系.结果 与正常对照组相比,多发性硬化组患者C2 ~ 5 前索、侧索、后索和灰质NASC 的FA 值降低、MD 值升高(均P < 0.05);相关分析显示,FA 值与EDSS 评分呈负相关(r = - 0.328 ~ - 0.207,P = 0.001 ~ 0.035),各兴趣区MD 值与EDSS 评分呈正相关(r =0.234 ~ 0.409,P = 0.000 ~ 0.018).结论 多发性硬化患者常规MRI 表现比正常脊髓的FA 值降低,提示存在隐匿性病变.脊髓扩散张量成像对多发性硬化患者临床评价和疗效判断有一定应用价值.     

A case of split cord malformation associated with myeloschisis

Introduction Split cord malformation (SCM) associated with myeloschisis is a very rare form of spinal dysraphism. We encountered a case of SCM associated with myeloschisis showing split neural placodes (hemicords) in the upper lumbar region. Results Radiological examinations, including prenatal MRI and postnatal CT scan, clearly demonstrated a bony spur between the two hemicords as well as myeloschisis, which facilitated a precise preoperative diagnosis of this complex anomaly. Resection of the spur, closure of the myeloschisis, and untethering of the thickened filum terminale were successfully accomplished in one stage. The presence of SCM associated with myeloschisis is consistent with the hypothesis of an ontogenic basis of neural development, and we emphasize the importance of early imaging including prenatal MRI for diagnosing this complex anomaly.     

Neuropathology of the acid sphingomyelinase knockout mouse model of Niemann-Pick A disease including structure-function studies associated with cerebellar Purkinje cell degeneration

Niemann-Pick A (NP-A) is an inherited metabolic (lysosomal storage) disease characterized by neurovisceral accumulation of sphingomyelin due to deficiency of acid sphingomyelinase (ASM). An ASM knockout (ASMKO) mouse model of NP-A is available through targeted disruption of the parent gene. This study presents the pattern and time course of lysosomal pathology and neurodegeneration in the ASMKO mouse nervous system. Cells throughout the nervous system developed the classic foamy appearance associated with lysosomal storage disorders. Despite this, neurons were capable of retrogradely transporting dyes within established brain pathways comparable to control animals. A silver degeneration staining method demonstrated widespread damage in the form of ‘classic’ impregnation of cells, fibers and synaptic terminals. Of particular interest was the degeneration of Purkinje cells (PC) within the cerebellum, beginning by 7 weeks of age in parasagittal bands and culminating with near complete degeneration of this cell type by 20 weeks. In parallel, ASMKO mice had progressively deteriorating motor performance on two versions of the rotating rod test (accelerating and rocking). ASMKO mice at 5–7 weeks of age performed similarly to controls on both rotating rod tests, but performance sharply deteriorated between 7 and 20 weeks of age. This study further characterized the neuropathology associated with ASM deficiency, and identifies quantitative histological and behavioral endpoints for evaluation of therapeutic intervention in this authentic NP-A mouse model.     

An autopsied case of the Crow-Fukase syndrome: a neuropathological study with emphasis on spinal roots

Summary An autopsied case of the Crow-Fukase syndrome is reported. Neuropathological findings were as follows: (1) in the sural nerve, there was marked decrease of large and small myelinated fibers. Myelinated fibers showing axonal degeneration and segmental demyelination and remyelination were moderately increased. (2) In the lumbar spinal roots, myelinated fibers showing segmental demyelination and remyelination were frequently observed. The density of myelinated fibers of the ventral root was less at the dural site than the spinal site, while that of the dorsal roots was less at the spinal site than the dural site. (3) In the dorsal root ganglion, there were Nageotte's residual nodules and satellitosis; (4) in the lumbar and thoracic spinal cord, there was pallor of the dorsal column; and (5) nerve cells showing central chromatolysis were frequently observed in the spinal anterior horn cells. Segmental demyelination and remyelination in the spinal roots and loss of myelinated fibers with axonal degeneration in the sural nerve are fibers with axonal degeneration in the sural nerve are main neuropathological features of this syndrome.     

Autosomal dominant Emery-Dreifuss syndrome: Evidence of a neurogenic variant of the disease

Summary The first German family with autosomal dominant Emery-Dreifuss syndrome (EDS) is described, with electrophysiologic and myopathologic results providing evidence of a primary neurogenic disease. According to classification of the scapulo peroneal syndrome without cardiomyopathy, we conclude that there are two variants of EDS: one myopathic, the other neurogenic in origin. Therefore, the term Emery-Dreifuss muscular dystrophy should be avoided. Instead, each case of EDS should be classified as myopathic or neurogenic with X chromosome recessive or autosomal dominant inheritance.The results of this study were presented in part at the 8th international congress of electromyography and related neurophysiology, Sorrento, Italy 1987     

Infantile neurodegenerative disease with neuronal accumulation of phosphorylated neurofilaments

Summary A caucasian male with a history of mental retardation and intractable epilepsy since birth, developed progressive wasting and weakness of skeletal muscles, leading to death at 4 years of age. A biopsy of gastrocnemius muscle at 2 years of age revealed severe neurogenic atrophy. Sural nerve biopsies at 2 and 3 years showed progressive atrophy and loss of large myelinated nerve fibers with a paucity of neurofilaments in remaining nerve fibers. Postmortem immunohistochemical and ultrastructural examination showed that neurons were markedly distended by phosphorylated neurofilaments. Whereas large lower motor neurons were most severely involved, dorsal root ganglia and neurons in the cerebral cortex and deep gray nuclei were also affected. It is suggested that this disease is caused by a disorder of neurofilament phosphorylation and transport.Supported by Aging Grant AG 04342 and by a Public Health Service International Research fellowship TW 03560 (SL). C. A. Wiley is a TIDA recipient NS 00928-01     

Biogenic amines and metabolites in spinal cord of patients with Parkinson's disease and amyotrophic lateral sclerosis

Summary In four human controls, four cases of Parkinson's disease and three cases of amyotrophic lateral sclerosis analysis of dopamine, noradrenaline, serotonin and the metabolites 3,4-dihydroxyphenylacetic acid, homovanillic acid and 5-hydroxyindoleacetic acid was performed in various segments of postmortem spinal cord. In controls the concentrations of dopamine are about 1/3 to 1/4 that of noradrenaline; the significantly highest content of noradrenaline was found in the lumbar, and dopamine in thoracic, lumbar and sacral segments of the spinal cord. Intersegmental distribution of monoamines was only present in spinal cord of controls, while in the spinal cord of parkinsonian patients such a difference was not found.Otherwise, biogenic amine and metabolite concentrations in spinal cord segments of parkinsonian patients did not differ significantly from those in the control subjects. However, it cannot be excluded that these segments are sensitive to drugs including neuroleptics and combined L-DOPA treatment.In subjects with amyotrophic lateral sclerosis significantly lower concentrations of noradrenaline in the cervical and thoracic, and of dopamine and homovanillic acid in the thoracic and lumbar segments were found in comparison with controls. The concentrations of serotonin and 5-hydroxyindoleacetic acid in the thoracic segments of amyotrophic lateral sclerosis were significantly lower than that of controls. Differences in the inter-segmental distribution of noradrenaline in lumbar, lumbar-sacral, and serotonin in lumbar segments of spinal cord were found in this group.