Magnetic resonance imaging in patients with progressive myelopathy following spinal surgery.

Thirty one patients with insidious progressive myelopathy 2 to 8 years following surgery of the cervical spine were subjected to magnetic resonance imaging (MRI). In 15 patients operated on for vascular malformations or intramedullary tumours, syringomyelia and cystic lesions of the spinal cord were shown. Seven of these patients also showed a combination of a recurrent tumour and spinal atrophy. Out of 16 patients who had surgery for herniated disc or spinal stenosis of the cervical spine, four had syringomyelia and 12 had spinal cord atrophy. There was no syringomyelia in the 12 patients submitted to MRI prior to surgery.     

Cystic cord lesions and neurological deterioration in spinal cord injury: operative considerations based on magnetic resonance imaging.

In a retrospective review of 94 consecutive patients with past spinal cord injury referred for magnetic resonance imaging (MRI) for the evaluation of new neurological symptoms, 59% were found to have cystic spinal cord lesions. Twelve of these patients underwent surgical cyst drainage, half having presented with increased myelopathy, and half with ascent of the neurological level. All of the operated cysts were greater than 2 cm in diameter (mean 15.8 cm), and 4 had areas of signal void indicating turbulent flow. All 12 patients had clinical improvement following surgery. The future prospective use of MRI in patients with longstanding spinal cord injury may prove valuable in the identification of patients with syrinx formation, at risk of developing neurological deterioration, who may benefit from early cyst drainage. At present, however, the decision to operate on these patients should be based primarily on clinical criteria.     

Increased adherence of T cells to human endothelial cells in patients with human T-cell lymphotropic virus type I-associated myelopathy.

We investigated the adherence of T cells to human umbilical vein endothelial cells in seven patients with human T-lymphotropic virus type I (HTLV-I)-associated myelopathy. The adherence of T cells to endothelial cells increased significantly in all the patients with HTLV-I-associated myelopathy when compared with the adherence in the seronegative controls (1.3- to 2.8-fold) and compared with the adherence in the anti-HTLV-I-seropositive non-HTLV-I-associated myelopathy carriers (1.4- to 2.8-fold). Prior treatment of the endothelial cell monolayer with recombinant interferon gamma (50 IU/mL) enhanced the T cell-endothelial cell adhesion in both the controls and patients with HTLV-I-associated myelopathy. However, values after prior treatment in the patients with HTLV-I-associated myelopathy were significantly higher than those in seronegative controls and carriers. The results suggest that the significantly increased T cell-endothelial cell adherence may be related to the initial stages of lymphocyte migration from the blood to the central nervous system in patients with HTLV-I-associated myelopathy.     

Autoimmune myelopathy associated with collapsin response-mediator protein-5 immunoglobulin G

Several autoimmune myelopathies are recognized clinically. We describe 57 patients in whom serological evaluation for myelopathy of uncertain cause demonstrated collapsin response-mediator protein 5 IgG. Most had spinal imaging and cerebrospinal fluid abnormalities and insidiously progressive presentation; some had acute monophasic or relapsing myelopathy. Initial diagnoses included multiple sclerosis, transverse myelitis, and unspecified neurodegenerative myelopathy. Most were smokers; neoplasia was discovered in 68% (most commonly small-cell lung carcinoma and after collapsin response-mediator protein-5 IgG detection). Collapsin response-mediator protein-5 autoimmune myelopathy and occult neoplasia are important considerations in patients with insidiously progressive myelopathy, especially with known cancer risk.     

Spinal cord and peripheral nerve pathology in AIDS: the roles of cytomegalovirus and human immunodeficiency virus

We examined spinal cords, nerve roots, or peripheral nerves of 27 patients who died with acquired immunodeficiency syndrome (AIDS) for the presence of cytomegalovirus (CMV) and human immunodeficiency virus (HIV) by immunoperoxidase techniques in paraffin-embedded tissue. Vacuolar myelopathy was seen in 8 of 26 spinal cords (31%) and microglial nodules were seen in 13 (50%). All of the patients with lateral column vacuolar myelopathy showed severe brain pathology. HIV antigens had been detected in the brains of 15 (55%) of the 27 patients but were detected in only 3 (11%) of 26 spinal cords and were not localized to regions of vacuolar myelopathy. This suggests that the vacuolar myelopathy may be due to a remote or indirect effect of HIV or other infectious agent. CMV antigens were detected in none of the patients who showed vacuolar myelopathy but were detected in 2 of the 13 with microglial nodules. Focal nerve root or peripheral nerve inflammation was seen in 7 patients; 4 had CMV antigens and none had HIV antigens. CMV appears to be an important cause of inflammatory peripheral neuropathy in AIDS patients.     

Idiopathic dystonia and cervical spondylotic myelopathy.

Cervical myelopathy developed in two patients with idiopathic torsion dystonia. There were marked spondylotic changes in both patients, probably attributable to the incessant dystonic movements of the neck. Previous cervical spine surgery may have exacerbated the myelopathy in one of the patients. Cervical myelopathy complicating idiopathic dystonia must be distinguished from other causes of neurological deterioration, since it may be improved by appropriate neurosurgical treatment.     

Hepatic myelopathy with spastic paraparesis

Progressive myelopathy is a rare neurological complication of chronic liver disease with portal hypertension and there is no special diagnostic tool for hepatic myelopathy. Neuropathological studies of the patients with hepatic myelopathy have demonstrated demyelination of the lateral corticospinal tracts with various degree of axonal loss. Transcranial magnetic stimulation (TMS) is widely utilized as an indicator of changes in exitability and conductivity of the motor pathways. TMS studies are also used for the diagnosis of hereditary spastic paraparesis in the literature. In this study, we described two patients who presented with spastic paraparesis; TMS studies suggested that they had myelopathy and diagnosed as hepatic myelopathy when all the other possible diagnoses were ruled out.     

人类获得性免疫缺陷综合征神经系统并发症临床分析

目的 探讨获得性免疫缺陷综合征（AIDS）患者神经系统并发症的临床特征。方法 对1992年1月至2001年5月间确诊的伴有神经系统并发症的5例AIDS患者进行临床分析。结果 5例中有1例空泡性脊髓病，1例空泡性脊髓病伴痴呆，1例颅内结核瘤伴痴呆，1例脑梗死伴继发性癫痫及三叉神经痛，1例多发性运动感觉神经病。其中2例空泡性脊髓病均为首发症状。结论 AIDS患者容易发生各种神经系统并发症，尤以空泡性脊髓病及痴呆多见，年轻突发痴呆患者尤其伴有机会性感染时需考虑该病的可能。对这些患者，应检查血抗人类免疫缺陷病毒（HIV）抗体。     

Is juvenile spinal muscular atrophy of the distal upper limbs due to cervical flexion myelopathy? A case report

A 22-year-old woman developed a slowly progressive symmetric weakness and muscular atrophy of distal upper limbs at the age of 17. Radiography during anteflexion and retroflexion showed a hypermobile cervical spine with a maximum at the C5/6 disc level. Cervical myelography and postmyelographic computed tomography (CT) of the lower cervical spine demonstrated a remarkable anterior shift of the dural sac during anteflexion resulting in anteroposterior compression of the lower spinal cord. Postmyelographic CT and magnetic resonance imaging (MRI) revealed atrophy of the lower spinal cord with bilateral cystic lesions. We suppose that repetitive straining and compression of the lower cervical cord during neck flexion of the hypermobile cervical spine caused selective necrosis of anterior horn cells with secondary cystic transformation. Mechanically induced flexion myelopathy should be considered in all young patients presenting with muscular atrophy of the distal upper limb. Functional CT myelography or dynamic MRI of the cervical spine are appropriate to demonstrate lower spinal cord compression during flexion.     

副肿瘤性脊髓病的临床和影像特点

目的报道副肿瘤性脊髓病的临床表现、影像和血清学特征。方法检索宣武医院2010年1月至2019年6月期间的副肿瘤性脊髓病住院患者,系统分析其临床表现、血清学和影像学数据。结果10例患者中符合确定的副肿瘤综合征7例,可能的副肿瘤综合征3例。患者中亚急性起病3例,慢性起病7例,脊髓病起病后确诊时间平均为10.1个月。10例患者中7例表现为单纯副肿瘤性脊髓病,2例伴有周围神经病,1例伴有僵人综合征。10例患者中9例患者行腰穿检查脑脊液,其中8例有异常,5例淋巴细胞增多,5例蛋白升高,6例寡克隆区带阳性;7例患者神经肿瘤抗体阳性,分别为抗amphiphysin抗体1例、抗CRMP-5抗体1例、抗Hu抗体3例、抗Yo抗体1例、抗Ri抗体1例;7例患者病理证实为恶性肿瘤。10例患者中,脊髓磁共振异常5例,其中长脊髓病变4例(其中累及椎体节段14个1例,6个1例,3个2例),短脊髓病变1例,病灶均对称性累及传导束或者脊髓灰质。10例患者中9例接受治疗,其中单纯手术治疗3例,单纯糖皮质激素治疗1例,手术+糖皮质激素治疗1例,糖皮质激素+肿瘤化疗治疗2例,手术+糖皮质激素+肿瘤化疗治疗3例,患者临床症状均无明显改善。结论副肿瘤性脊髓病罕见,多表现为慢性进行性脊髓病且多先于肿瘤出现;多数患者脑脊液表现异常,影像学可表现为脊髓长节段病灶。该病治疗上以肿瘤治疗为主,免疫治疗为辅。     

Electroencephalographic abnormalities in human T-cell lymphotropic virus type I-associated myelopathy

Thirty-six patients with human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy were studied by electroencephalogram. Twenty-two of 36 patients showed mild to moderate electroencephalographic abnormalities, ranging from poor organization or slowing of the background activity to theta bursts and/or spikes. None of these abnormalities were considered specific for HTLV-I-associated myelopathy. These electroencephalographic abnormalities had no apparent relationship to duration or severity of illness, nor to HTLV-I antibody titers in the cerebrospinal fluid. We document electroencephalographic changes in HTLV-I-associated myelopathy. Our data are consistent with previous reports describing the fact that involvement of regions above the spinal cord may exist in HTLV-I-associated myelopathy.     

Activated T cells in HTLV-I-associated myelopathy: autologous mixed lymphocyte reaction

We examined the autologous mixed lymphocyte reaction in human T-cell lymphotropic virus type I-associated myelopathy to elucidate the known increase in spontaneous proliferation of peripheral blood lymphocytes. Proliferative responses in the autologous mixed lymphocyte reaction test were significantly increased (34,205 cpm) in cells from 9 patients with the myelopathy, as compared with findings (18,695 cpm) in the control subjects. When non-T-cell fractions were depleted from autologous mixed lymphocyte reaction cultures, proliferative responses were completely suppressed in the control subjects, while proliferation of the peripheral blood lymphocytes from the groups with the myelopathy were not affected by depletion of non-T-cell fractions. The increase in spontaneous proliferation of peripheral blood lymphocytes in patients with the myelopathy is probably not related to an increase in autologous mixed lymphocyte reactions.     

Somatosensory conduction times and peripheral, cervical and cortical evoked potentials in patients with cervical spondylosis.

Peripheral, cervical and cortical somatosensory evoked potentials after median or ulnar nerve stimulation were recorded in 21 patients with cervical spondylosis with radiculopathy or myelopathy. The test was normal when pain and paraesthesias were the only symptoms, while pathological in radiculopathy with objective neurological signs. The results varied in patients with cervical myelopathy.     

Absence of antibody to HTLV I and III in sera of Canadian patients with multiple sclerosis and chronic myelopathy

Recently, elevated titers of antibody to HTLV I have been demonstrated in patients with tropical spastic paraparesis and multiple sclerosis. We evaluated the possible role of human retroviruses HTLV I and III in Canadian patients with multiple sclerosis and chronic idiopathic myelopathy. Using sensitive enzyme immunoassays, we were unable to find antibody to either HTLV I or III in 201 patients with multiple sclerosis, 29 patients with chronic myelopathy, 51 patients with other neurological disorders, or 29 normal subjects. These data do not support a role for these viruses in the cause of sporadic multiple sclerosis and chronic myelopathy in a regionally based Canadian population, but do not exclude a role for other antigenically distinct retroviruses.     

Multiple lesions in cerebral white matter in two young adults with thoracic extramedullary tumours.

Cranial MRI showed multiple lesions in white matter that were thought to be consistent with multiple sclerosis in two young adults presenting with symptoms of progressive myelopathy. MRI of the cervicothoracic spine around one and two years after onset showed the myelopathy to be due to mid-thoracic tumours. The tumours (an extradural meningioma and intradural neuroma) were resected with complete resolution of myelopathy in one patient but no recovery in the other. Spinal MRI (or myelography) should be performed in young patients presenting with signs of progressive myelopathy even when cranial MRI shows a picture typical of multiple sclerosis.     

脊髓型颈椎病合并胸椎管狭窄症的诊断与治疗

目的：探讨脊髓型颈椎病合并胸椎管狭窄症的诊断与治疗。方法：回顾性分析4例脊髓型颈椎病合并胸椎管狭窄症的诊断与治疗。其中2例先行颈椎减压和稳定手术，后行胸椎板切除减压术；1例颈、胸椎同时行减压；1例先行胸椎板切除减压术，后行颈椎板成形术。结果：神经病学损害完全恢复2例，明显改善2例。结论：脊髓型颈椎病合并胸椎管狭窄症手术治疗可取得良好的效果。     

Chronic progressive myelopathy: its relation to the spinal progressive form of multiple sclerosis

The causes and clinical features of chronic progressive myelopathy (CPM) were evaluated in a retrospective study of 107 patients. A special emphasis was put on those in whom no underlying cause for the myelopathy could be determined. Of 76 such, 39 (51%) had oligoclonal immunoglobulins (Ig) in the CSF and were therefore considered as possible MS, while the remainder, without oligoclonal Ig, were designated "myelopathy of unknown origin" (MUO). Our "possible MS" group was similar clinically to reported series of proven spinal MS, and it seems therefore, that the presence of oligoclonal Ig permits the recognition of a group of patients with myelopathy who might be at a greater risk for MS. Patients with MUO differed from possible MS patients in several clinical characteristics, but most significantly in disease course and levels of functional disability which were more benign in the former. Myelopathy in possible MS patients was also of a primary pyramidal and asymmetrical nature. It is therefore suggested that the segregation of patients with CPM of undetermined origin into 2 separate groups based on the presence or absence of oligoclonal Ig might be of prognostic significance.     

Acute- and insidious-onset myelopathy of undetermined aetiology: contribution of paraclinical tests to the diagnosis of multiple sclerosis

Summary Brain magnetic resonance imaging (MRI), multimodality evoked potentials (EPs) and cerebrospinal fluid examination were performed in 42 patients with myelopathy of undetermined aetiology in order to detect abnormalities usually related to multiple sclerosis (MS). Patients were divided into three groups: insidious-onset myelopathy with only motor signs (group A; 11 patients), with both motor and sensory signs (group B; 18 patients) and acute-onset myelopathy (group C; 13 patients). Multiple brain MRI lesions were found in 18 patients (2 of group A, 13 of group B and 3 of group C). Another 7 patients had a single white-matter lesion. Visual EPs were abnormal in 21 and brain-stem auditory EPs in 12 patients. Paraclinical tests supported the diagnosis of MS in 25 patients (60%) by showing subclinical brain abnormalities. Oligoclonal bands were found in 16 of these 25 patients. The findings strongly suggest a diagnosis of MS in the patients of group B.     

An autopsy case with adult onset type II citrullinemia showing myelopathy

Hepatic myelopathy is a rare neurological complication in patients with chronic liver failure and most patients who suffered from this disorder were demonstrated to have portal-systemic shunt. A 31-year-old man who was diagnosed as having adult-onset type II citrullinemia (CTLN2) and had a six-year history of recurrent hepatic encephalopathy showed progressive spastic paraparesis with no involvement of sensation and sphincter function. Examinations of cerebrospinal fluid and spinal MRI were normal. He suddenly died of acute exacerbation of hepatic encephalopathy with severe brain edema. The pathology of the spinal cord disclosed a localized degeneration of both lateral columns, the lesion being more remarkable in the lower segments of the cord. These clinical and pathological findings of hepatic myelopathy have not been noted in the many patients with CTLN2 previously reported, and our patient is unique in developing hepatic myelopathy without porto-caval shunting. Thus, repeated attacks of encephalopathy with hyperammonemia might secondarily have induced the myelopathy in this patient.     

The role of somatosensory evoked potentials in the diagnosis of AIDS-associated myelopathy

BACKGROUND: Although AIDS-associated vacuolar myelopathy is detected in >50% of autopsy cases, it is often unrecognized during life. The clinical assessment is often difficult because of concurrent peripheral neuropathy and lack of specific diagnostic markers. Somatosensory evoked potentials (SEPs) have been successfully used to evaluate central conduction in a number of diseases involving the spinal cord. OBJECTIVES: To assess the diagnostic yield of SEPs in AIDS-associated myelopathy. METHODS: We recorded tibial and median nerve SEPs in 69 HIV-infected subjects referred for evaluation of lower extremity neurologic abnormalities. Stimulation of the peroneal nerve at the popliteal fossa was performed in patients with absent response to ankle stimulation. RESULTS: HIV-infected subjects had significantly delayed latencies of both peripheral and central potentials, suggesting a combination of peripheral and CNS abnormalities. Analysis of peripheral and central latencies allowed us to discriminate between neuropathy and myelopathy in individual patients. Abnormalities of tibial central conduction time (CCT) correlated with clinical diagnosis of myelopathy. There was no significant difference in median CCTs between patients and controls, suggesting that conduction abnormalities were restricted to the thoracolumbar spinal cord. A derived spinal conduction time was a sensitive indicator of central conduction abnormalities in AIDS patients with myelopathy. CONCLUSIONS: The combination of median, posterior tibial, and peroneal SEPs is a valuable tool in the diagnosis of AIDS-associated myelopathy, particularly when myelopathy and peripheral neuropathy coexist. The use of a derived spinal conduction time improves the diagnostic yield of SEPs in AIDS-associated myelopathy.