Progressive ascending myelopathy: atypical forms of multiple sclerosis or what else?

The spinal cord can be affected by multiple heterogeneous disorders often difficult to diagnose. We describe ten patients affected by a progressive ascending myelopathy with a poor prognosis. The patients, during the follow-up period, underwent neurological examinations, cerebrospinal fluid analysis, hematological, microbiological, auto-antibodies screening, brain and spinal cord magnetic resonance imaging (MRI) and electroneurophysiological study. At disease onset spinal cord MRI showed ≥1 myelopathic lesions extended for <2 segments and then evidenced a progressive spinal cord atrophy without any new lesion. All patients showed an increase of the visual evoked potential P100 latency. All of them showed two or more clinical recurrences of myelitis and then, after a period ranging from 3 to 5 years from the disease onset, a progressive course. Five patients became unresponsive to intravenous high-dose steroid treatments and/or intravenous immunoglobulins and to any other therapeutic attempts, developed a progressive ascending myelopathy to tetraplegia and died from respiratory failure. The other five patients are in progressive phase of the disease with an initial involvement of the upper limbs and show mild cervical spinal cord atrophy at MRI, configuring the early stage of an ascending progressive myelopathy. In our opinion, the more suitable diagnosis is an atypical form of MS although is not possible to exclude a new nosological entity that could be included in the expanding range of spinal cord diseases.     

Clinical features of spinal cord sarcoidosis: analysis of 17 neurosarcoidosis patients

The diagnosis of neurosarcoidosis is often difficult; the imaging signs of spinal cord sarcoidosis sometimes mimic those of cervical spondylotic myelopathy, which is common in elderly persons. We examined the characteristics of spinal cord sarcoidosis in Japanese patients with neurosarcoidosis. This case series identified patients with neurosarcoidosis at four general hospitals and one university hospital from April 1998 to September 2010. All diagnoses were based on the diagnostic criteria proposed by Zajicek et al. Seventeen patients (nine men and eight women) were involved: six patients with spinal cord lesions accompanied by cervical spondylosis, five with cerebral lesions, three with cranial nerve lesions, two with meningitis, and one with nerve root lesions. Patients with spinal cord sarcoidosis had a higher onset age, longer duration from onset to diagnosis, reduced leukocytosis in the cerebrospinal fluid (CSF), and lower angiotensin-converting enzyme (ACE) levels in the CSF. The results of this study indicate that diagnosis of spinal cord sarcoidosis requires careful evaluation.     

Myelopathy simulating spinal cord tumor.

Three cases of demyelinating or necrotizing myelopathy are presented in which gradual onset of symptoms, slowly progressive course and widened cord shadow on myelography led to the erroneous diagnosis of intramedullary spinal cord neoplasm. Two of the cases also had inconclusive surgical explorations. We would suggest that patients who seem to have even a typical clinical and myelographic picutre of an intrinsic cord neoplasm have a period of further observation before radiotherapy is begun and that a vigorous search be made for evidence of disseminated disease.     

Angiostrongylus cantonensis infection mimicking a spinal cord tumor

Angiostrongylus cantonensis is the most common cause of eosinophilic meningitis and meningoencephalitis. Almost all cases are self-limiting and are diagnosed by cerebrospinal fluid eosinophilia and enzyme-linked immunosorbent assay; pathology reports are restricted to postmortem samples from lethal cases. We report on what we believe is the first case of A. cantonensis infection diagnosed by biopsy in a living patient. The spinal cord was biopsied because of the unusual clinical presentation of a myelopathy without meningeal symptoms, together with a mass lesion that was clinically and radiologically diagnosed as a spinal cord tumor.     

Spinal cord sarcoidosis--diagnostic value of Gd-enhanced MRI]

A 50-year-old man was admitted because of slowly progressive myelopathy for the last ten months without a correct diagnosis. The CSF showed mild pleocytosis (predominantly mononuclear cells) together with elevated protein content. MRI findings after administration of Gd-DTPA were characteristic: diffuse swelling of the lower cervical and upper thoracic cord, multiple patchy enhanced areas in the cord parenchyma adjacent to meninges, and linear meningeal enhancement. Based on these observations, a scalene node biopsy was performed, yielding a pathological diagnosis of sarcoidosis. The clinical neurological signs as well as the abnormal MRI findings of myelopathy were considerably improved after prednisolone therapy. An early diagnosis of spinal cord involvement in sarcoidosis should be established, because it is treatable in the earlier stage. Mild inflammatory evidence in CSF and Gd-enhanced MRI findings may be the cardinal clues to the diagnosis.     

A case report of the compression syndrome due to hypertrophic neuropathy

We reported a case of hypertrophic neuropathy of adult onset. The pathological change in the sural nerve was decreased axonal population with onion-bulb formation. On examination, there were enlarged nerve on palpation and she was found to have distal muscle atrophy, weakness and sensory loss. The deep tendon reflexes of extremities were weak. The first clinical feature of this patient was mainly polyneuropathy. The lower limbs were slightly spastic and plantar responses were extensor bilaterally. There was also sensory level at C6 level. After 1.5 years from first examination, she had shown myelopathy. Magnetic resonance imaging (MRI) of the spine showed marked thickening of the nerve roots and it revealed the compression of the spinal cord by enlarged nerve roots from C2 to C6 level. The compression syndrome of the patients with hypertrophic neuropathy was unclear at the onset in this case. MRI study of the spinal cord would be very beneficial to disclose subclinical myelopathy associated with hypertrophic neuropathy, as indicated in this report.     

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.     

Pathological and molecular biological features of a myelopathy associated with HTLV-1 infection

We report the pathological and molecular biological findings of human T-cell lymphotropic virus type 1 (HTLV-1) infection of the spinal cord in a patient with a chronic progressive myelopathy. Light microscopy disclosed loss of myelin and axons, thickening of blood vessels and a lymphocytic cell infiltrate in the spinal cord especially at the cervical and thoracic levels. Electron microscopy confirmed the vascular appearance seen with light microscopy but virus particles were not observed. The HTLV-1 gag gene could be amplified (by polymerase chain reaction) from cervical spinal cord tissue while not from elsewhere in the neuroaxis. The presence of HTLV-1 genomic material in spinal cord tissue has not been previously reported.     

A tentorial dural arteriovenous fistula presenting progressive myelopathy: a case report

Abstract We report a case of a dural arteriovenous fistula (DAVF) at the tentorium cerebelli, which presented progressive myelopathy. A 68-year-old man with neurological deterioration of the cervical myelopathy visited our hospital. T₂ weighted magnetic resonance (MR) imaging showed high signal area and edema from the medulla to the upper thoracic spinal cord with flow voids on the dorsal surface of the cord. Angiography showed right tentorial DAVF, which was supplied by the right meningohypophyseal trunk, the middle meningeal artery, the accessory meningeal artery, and was drained into the posterior spinal veins. The patient underwent right retrosigmoid suboccipital craniotomy, then disruption of the fistula was performed by using micro Doppler sonography following endovascular obliteration of the main feeders. Postoperative angiography showed complete obliteration of the fistula. His daily functioning gradually improved up to 6 months after the surgery. Tentorial DAVFs with clinical manifestation of myelopathy are rare. Considering its aggressive nature, early surgical treatment could be necessary. (Received: November 17, 2010, Accepted: December 18, 2010).     

Delayed diagnosis of spinal dural arteriovenous fistula in the absence of pathological vessels on MRI

The authors report on a 69-year-old man presenting with progressive leg weakness and gait ataxia over two years. A central intramedullary cord lesion ranging from T8-12 on MR imaging was misdiagnosed as a low-grade glioma and a biopsy was attempted followed by temporary clinical deterioration. Selective spinal angiography revealed a spinal dural arteriovenous (AV) fistula on the left L3 nerve root sheath despite the absence of pathological vessels on MR imaging. The fistula was successfully treated by microsurgical interruption of the arterialized intradural vein. The present case should remind us to include selective spinal angiography in our diagnostic work-up in patients predisposed for spinal dural AV fistula by male sex, advanced age and clinical presentation of slowly progressive sensorimotor symptoms with myelopathy on MR imaging, even in the absence of any pathological vascular structures.     

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。     

Venous congestive myelopathy: Three autopsy cases showing a variety of clinicopathologic features

We describe three patients with progressive myelopathy, in whom autopsy revealed spinal cord pathology compatible with that of venous congestive myelopathy (VCM) associated with dural arteriovenous fistula (AVF), formerly known as angiodysgenetic necrotizing myelopathy (Foix–Alajournine syndrome). In these three patients, common symptoms were gait disturbance and sensory disturbance of the extremities, and these symptoms slowly worsened. The clinical diagnoses varied and included spinal cord intramedullary tumor, cervical spondylosis and multiple sclerosis. At autopsy, all the patients showed enlarged, tortuous venous vessels on the dorsal surfaces of the spinal cord at the affected levels. In the affected spinal cord parenchyma, necrotic lesions manifested by various degrees of neuronal loss and gliosis, with increased numbers of hyalinized vessels, were evident. The presence or absence of associated spinal dural AVF could not be identified histopathologically. Even with the help of modern neurological examination methods, early and accurate clinical diagnosis of VCM is sometimes difficult. When encountering patients with progressive myelopathy, VCM, although recognized as rare, should be considered as an important differential diagnosis.     

Cervical Compressive Myelopathy due to Anomalous Bilateral Vertebral Artery

We report a very rare case of cervical compressive myelopathy by an anomalous bilateral vertebral artery (VA) entering the spinal canal at the C1 level and compressing the spinal cord. A 70-year-old woman had been suffering from progressive gait disturbance. Magnetic resonance imaging revealed that a bilateral VA at the V4 segment had abnormal courses and caused compression to the high cervical cord. VA repositioning was performed by anchoring a suture between the artery and around the arachnoid membrane and dentate ligament, and then, microvascular decompression using a Teflon sponge was done between the VA and the spinal cord. The weakness in the patient improved in the lower extremity after the operation. Anomalous VA could be one of the rare causes of cervical compressive myelopathy. Additionally, an anchoring suture and microvascular decompression around the VA could be a sufficient and safe method to indirectly decompress the spinal canal.     

Spinal angiolipoma

BACKGROUND: Spinal epidural angiolipoma is a rare cause of spinal cord compression. We present a case and review the clinical presentation, radiological appearance, pathological aspects and treatment of this distinct clinico-pathological entity. METHODS: A case of a 46-year-old woman with a five-month history of progressive myelopathy affecting her lower extremities is presented. CT and MRI revealed a large epidural fat-containing mass compressing the spinal cord dorsally at the T7-T8 level. A laminectomy was performed with gross total resection of the lesion. RESULTS: The patient's neurologic symptoms improved postoperatively. A two-year follow-up period has revealed no signs of tumor recurrence and no neurological deficit. CONCLUSION: The diagnosis of spinal angiolipoma should be considered in the differential diagnosis of spinal cord compression. Magnetic resonance imaging is the investigation of choice. The surgical objective is complete excision but, for anterior lesions involving bone, an overly aggressive approach should be tempered by an awareness of the overall indolent natural history of so-called "infiltrating" spinal angiolipomas that are only partially excised.     

Myelopathy in mucopolysaccharidosis type II (Hunter syndrome)

A 24-Year-old man with Hunter syndrome had spastic quadriparesis due to impingement of thickened meninges upon the cervical spinal cord. Tracheal narrowing due to submucosal deposits (presumably mucopolysaccharide) produced serious ventilatory complications during induction of anesthesia and necessitated tracheostomy before surgical decompression of the spinal cord could be attempted. Recognition of compressive myelopathy and tracheal compromise as late complications of Hunter syndrome may promote early therapy and prevent respiratory catastrophe.     

Acute myelopathy. Retrospective clinical, laboratory, MRI and outcome analysis of 49 cases.

Forty nine consecutive cases of acute myelopathy were related to the following pathologies: 31 MS, four spinal cord infarction, four parainfectious, one antiphospolipid syndrome and nine of unknown origin. Sensory deficits were most frequent in MS and in myelopathy of unknown origin. In spinal cord infarction motor deficits and sphincter dysfunction were present in all cases. In parainfectious myelopathy sensory-motor deficits and sphincter dysfunction were most frequent. Myelopathy extended over less than two vertebral segments in MS and in myelopathy of unknown origin. Myelopathy extended over more than two vertebral segments in spinal cord infarction and in parainfectious myelopathy. The clinical outcome was good in MS, parainfectious myelopathy and myelopathy of unknown origin, but poor in spinal cord infarction. Our findings suggest that various aetiologies of acute myelopathy may be differentiated on the basis of distinct clinical, MRI, laboratory and outcome data.     

Cerebral amyloid angiopathy as a cause of an extensive brain hemorrhage in adult patient with Down's syndrome - a case report

A case of 54-year old woman who deceased due to consequence of extensive brain hemorrhage is presented. The patient was admitted to our Department of Neurology due to progressive quadriparesis as a complication of the cervical spinal cord compressive myelopathy. On the third day after neurosurgical decompression of the spinal cord sudden worsening of neurological and general condition was observed, finally caused death. An autopsy study revealed an extensive brain lobar hemorrhage and a dorsal-ventral compression of the cervical spinal cord. Alzheimer's disease-type degenerative changes with concomitant CAA were seen in light microscope examination. Extensive foci of demyelination were found especially in dorsal funiculi of the cervical spinal cord. Smaller foci of demyelination were present in anterior funiculi due to the stenosis of vertebral canal.     

Rapidly progressive quadriparesis heralding disseminated coccidioidomycosis in an immunocompetent patient

Coccidioides species are dimorphic fungi endemic to southwestern USA and northern Mexico. Disseminated coccidioidomycosis is rare with an estimated incidence of 1% in affected individuals and usually presents as meningitis when the central nervous system is involved. Spinal involvement with coccidioidomycosis, though not uncommon, predominantly manifests as osseous involvement leading to osteomyelitis and epidural abscess formation. Progressive quadriparesis as a presenting symptom secondary to intramedullary spinal cord coccidioidomycosis is very unusual and to our knowledge has not been described. We report a patient with disseminated coccidioidomycosis who presented with rapidly progressive quadriparesis due to cervical intramedullary spinal cord involvement. The absence of known coccidioidomycosis with atypical clinical presentation made the diagnosis elusive, requiring emergent cervical laminectomies with dural biopsy for decompression of the spinal cord and confirmation of the diagnosis. The patient eventually succumbed to the progressive course of the disease. Although rare, disseminated coccidioidomycosis can present as new, rapidly progressing quadriparesis in patients who have traveled to endemic areas. A high index of suspicion in such patients with appropriately directed laboratory investigations and consideration of early biopsy might unravel the diagnosis facilitating early antifungal treatment with the potential to minimize morbidity and mortality associated with disseminated coccidioidomycosis.     

Syringomyelia as a complication of tuberculous meningitis.

Tuberculous meningitis may rarely be followed by the development of syringomyelia despite appropriate chemotherapy. In the present paper, we report a case of tuberculous meningitis in a 23-year-old Vietnamese male complicated by a rapidly progressive myelopathy due to granulomatous arachnoiditis which culminated in the development of a syrinx. The relevant literature is reviewed. The present case supports the hypothesis that vasculitic thrombosis of spinal cord vessels leading to ischemic myelomalacia is the mechanism causing postinflammatory syringomyelia.     

HTLV-I-associated myelopathy: clinicopathologic correlation with localization of provirus to spinal cord.

A 49-year-old South African man developed a rapidly progressive myelopathy 14 months after blood transfusion and died 1 year after the onset of symptoms. Detailed pathologic examination of the spinal cord was consistent with the diagnosis of HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Although no HTLV-I viral particles, antigens, or nucleic acids were detected in situ, polymerase chain reaction assays revealed HTLV-I proviral DNA in cervical, thoracic, and lumbar levels of the spinal cord, with the greatest amount being detected at the thoracic level. These findings suggest that the pathogenesis of HAM/TSP depends on direct infection of neural or immune elements within the spinal cord.