Recidiva traumática de herniación medular idiopática

Idiopathic spinal cord herniation is a rare cause of thoracic myelopathy and its recurrence is even more infrequent. Cord herniation is through an anterior dural defect in thoracic spine with unknown causes. Symptomatic cases must be surgically treated to reduce the hernia and seal the defect to prevent recurrences. We report a patient presenting a Brown-Séquard syndrome secondary to a D5 spinal cord herniation treated successfully and its posterior traumatic recurrence.     

Acute-onset nontraumatic paraplegia in childhood: fibrocartilaginous embolism or acute myelitis?

Fibrocartilaginous embolus causing acute spinal cord infarction is a rare cause of acute-onset paraplegia or quadriplegia. Few cases of survivors have been reported in the neurosurgical literature, with most reports involving post-mortem or biopsy findings. There is little information on MRI findings in such patients. We present the youngest patient ever reported, and discuss the important differences between fibrocartilaginous embolus and acute myelitis of childhood. A 6-year-old girl with a history of back pain presented with sudden-onset nontraumatic paraplegia, with a clinical anterior spinal artery syndrome. Initial MRI scan revealed intervertebral disc disease at L1–2 and an incidental thoracic syrinx, but no cause for her acute-onset paraplegia was identified. Cerebrospinal fluid and other investigations were all negative. Sequential MRI scans revealed development of spinal cord expansion from T10 to the conus medullaris, with increased cord signal in the anterior aspect of the spinal cord. The intervertebral disc disease was unchanged. The imaging and clinical findings were caused by fibrocartilaginous embolus, which meant there was no need for spinal cord biopsy. The report describes the clinical and imaging criteria for diagnosis of fibrocartilaginous embolus, highlighting the case for avoiding an unnecessary biopsy. The clinical pattern in the paediatric group is discussed, with features differentiating it from acute myelitis of childhood. Received: 4 January 2000     

Spinal cord involvement by C4–C5 Vertebral subluxation in rheumatoid arthritis: A description of 2 cases examined at necropsy

In 2 cases of severe, longstanding rheumatoid arthritis involving the cervical spine, a progressive spinal cord syndrome developed in which spastic paraparesis was combined with a lower motoneuron paresis affecting the upper limbs. In both cases necropsy showed the cause to be subluxation between the C4 and C5 vertebral bodies and backward encroachment into the spinal canal of the upper and posterior part of the C5 vertebral body. A feature of the spinal cord abnormality was central infarction, apparently caused by an effect on a patent anterior spinal artery and manifested not only at the site of spinal cord compression but for several spinal cord segments caudal to this level.     

Vitamin B12 extensive thoracic myelopathy: clinical, radiological and prognostic aspects. Two cases report and literature review

The myelopathy caused by vitamin B12 deficiency is known as subacute combined degeneration. It is rare, but a well known cause of demyelination of the dorsal columns of the spinal cord. The magnetic resonance imaging is characterized by an increased signal on T2-weighted images involving the posterior columns of cervical and thoracic cord. There have been few cases in literature with extensive lesions (more than seven levels) of the thoracic spinal cord. The clinical and radiological improvements are possible if the replacement of vitamin B12 is initiated precocious. We present two rare cases of extensive thoracic myelopathy due to vitamin B12 deficiency. The first is a young woman with complete clinical recovery and important radiologic improvement after early treatment. In addition, the second case is an older man with partial response to the treatment. Those cases illustrate the importance of considering vitamin B12 deficiency in any patient, who presents with myelopathy.     

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.     

Pathological examination of spinal lesions in meningeal carcinomatosis

For elucidation of the mechanism of spinal cord disorders associated with meningeal carcinomatosis, we investigated meningeal infiltration and parenchymal, vascular, and radicular damage in spinal cords subjected to pathological autopsy. Spinal cords were collected from 24 patients with a histopathological diagnosis of meningeal carcinomatosis. Infiltration from the subarachnoid space into the spinal cord occurred primarily along the perivascular tissue, and infiltration from the anterior median fissure to the anterior horn was found along the central artery in cases of marked meningeal dissemination. Meningeal dissemination was particularly evident with small cell carcinoma of lung, breast cancer and melanoma, and direct infiltration from the meninges into the spinal cord was found. Direct infiltration into the nerve roots was frequently observed, and infiltration was more evident in the dorsal roots than in the ventral roots, with loss of nerve fibers. Circular necrosis of the white matter in the periphery of the spinal cord was noted in cases of marked meningeal dissemination, which probably resulted from circulatory disturbance secondary to tumor infiltration. In cases of marked dorsal radiculopathy, there was secondary ascending degeneration of the posterior funiculus in cases of marked dorsal radiculopathy. These pathological changes associated with spinal cord disorders are important findings in reviewing spinal cord symptoms and radiographic findings in meningeal carcinomatosis.     

Preferential neurodegeneration in the cervical spinal cord of progressive supranuclear palsy

Spinal cord lesions have seldom been described in cases with progressive supranuclear palsy (PSP). We thus decided to analyze spinal cord lesions by microtubule-associated protein 2 (MAP2) immunohistochemistry in six cases of PSP, five cases of Parkinson’s disease (PD) and two cases of corticobasal degeneration (CBD), all of which cause parkinsonism, while six patients without any neurological disease served as controls. In the PSP cases, the MAP2 expression in the cervical spinal cords significantly decreased in the medial division of the anterior gray horn, intermediate gray and posterior gray horn, but showed no significant change in the substantia gelatinosa and lateral division of the anterior gray horn. The thoracic and lumbar spinal cords were well preserved for MAP2 immunoreactivity. In addition, the globose type neurofibrillary tangles and glial fibrillary tangles were more conspicuous in the cervical than in the thoracic and lumbar spinal cord in PSP cases. On the other hand, the PD and CBD cases showed no significant decrease of MAP2 immunoreactivity in the spinal cords. The small neurons, which are located rather selectively in the intermediate zone of the spinal cord, are considered to be mostly present in the interneurons, and are also thought to play a role in various types of focal dystonia, such as neck dystonia. We therefore consider the distinct decrease in the MAP2-positive neuronal processes in the cervical spinal cord may partly reflect the loss of interneurons and may, thereby, possibly cause nuchal dystonia. Received: 17 August 1998 / Revised, accepted: 9 November 1998     

Central nervous system of a thoracopagus

Summary Postmortem examination of the central nervous system (CNS) of a dicephalus thoracopagus tetrapus revealed two separate brains and spinal cords. On the conjoined side the spinal cord showed hypoplasia of the anterior horns and dorsal funiculi at the lower cervical and upper thoracic levels. Hypoplasia of the peripheral nerves, anterior horn cells, and dorsal funiculi is a consequence of a reduction in tissue mass due to a loss of inductive influence.     

Sensory ataxic neuropathy presenting anti-a-fodrin antibody associated with rheumatoid arthritis]

We reported a 41-year-old woman with severe rheumatoid arthritis who developed chronic sensory ataxic neuropathy and anti-a-fodrin antibody. Neurological examinations, electrophysiological studies and sural nerve biopsy findings suggested dorsal root ganglioneuronopathy. T2*-weighted MRIs showed high signal intensities in the posterior column of the cervical and thoracic spinal cord, suggesting the degeneration of the posterior column of the spinal cord caused by ganglioneuronopathy. She showed xerophthalmia but no other manifestations indicating Sj?gren's syndrome including sialography and salivary gland biopsy findings. In serological studies, although anti-SS-A/SS-B antibodies and anti-ganglioside antibodies were negative, her serum reacted to a-fodrin, a 120kD protein degraded from a chain of fodrin. As a-fodrin has been recently regarded as an autoantigen highly specific to Sj?gren's syndrome and its antibody is likely to appear preclinically, she may develop Sj?gren's syndrome in the future. It is essential to accumulate clinical and pathological findings of cases like ours in order to elucidate an etiological relationship between anti-a-fodrin antibody and dorsal root ganglioneuronopathy.     

肝硬化的脊髓病变：肝硬化与非肝硬化尸体胸腰髓形态及神经元功能蛋白表达比较

依据国内外现有文献报道的肝硬化存在脊髓外侧索损伤的现象,实验对肝硬化与非肝硬化尸体的胸、腰髓形态、功能蛋白表达及其相关血管变化等进行了解剖观察与比较分析。结果显示,与非肝硬化尸体相比,肝硬化患者尸体的肝门静脉管径增粗,全胃肠管腔阶段性积血;椎管内静脉丛和腰髓明显淤血;胸、腰髓前角神经元胞体变小;少数神经元固缩;Nissel体密集细小,呈脱颗粒样改变,胞核不清晰;前角神经元胞体和白质侧索纤维束神经丝蛋白和突触素含量减少。提示肝硬化门静脉高压血流动力学改变导致的椎管和脊髓血循环异常可能是肝性脊髓病的最重要因素。     

Role of diffusion MRI in diagnosis of spinal cord infarction in children

Anterior spinal artery (ASA) infarction is a rare but well-described cause of flaccid paraparesis in adults, presenting with a high thoracic spinothalamic sensory level and preservation of dorsal column function. Careful sensory examination, demonstrating loss of spinothalamic modalities with preservation of dorsal column modalities, supports a clinical diagnosis of ASA infarction. Findings on conventional MRI of the spinal cord are often non-specific, and diffusion-weighted imaging (DWI) is not routinely performed. We describe four children with ASA infarction after minor trauma. DWI was performed in all cases and confirmed the clinical diagnosis.     

Effects of selective spinal cord lesions on hind limb locomotion in birds

In birds, a variety of subtotal thoracic spinal cord lesions were made to determine the spinal cord pathways essential for avian hind limb locomotion (bipedal walking). The various surgical disruptions included: section of the dorsal half of the cord, a hemisection, section of the dorsal cord and ventromedial funiculi, section of the dorsal cord ventrolateral funiculi, section of the entire thoracic cord except for one ventrolateral quadrant, section of the ventral half of the cord, and complete transection of the thoracic cord. The study compared the locomotion following these lesions in both chronic surviving and acutely decerebrated, brain stem-stimulated birds. Behavioral assessments and electromyographic recording techniques were used to evaluate locomotor activity. Our results showed that transectioning pathways within the dorsal cord did not hinder the activation and maintenance of self-supported walking in either preparation. However, sparing the spinal cord pathways within either the ventromedial or ventrolateral funiculi of the thoracic spinal cord was essential for the activation of self-supported walking in both preparations. When our findings are integrated with previous studies, medullary reticulospinal pathways (projecting through the ventral funiculi) are strongly implicated as a common descending projection for the activation of spinal cord locomotor networks and the initiation of locomotion. Similar findings have been found in quadrapedal mammals and, as a complement, birds may make an excellent model for the study of bipedal locomotion.     

Pernicious azotaemia? A case series of subacute combined degeneration of the cord secondary to nitrous oxide abuse

Nitrous oxide abuse is a rare cause of vitamin B12 deficiency and consequent subacute combined degeneration of the spinal cord. Worldwide and Australian statistics indicate that recreational use of nitrous oxide is increasing. We report four cases of females aged 18–24 years presenting with clinical symptoms of subacute combined degeneration of the spinal cord. MRI during admission demonstrated the classic findings of T2 hyperintensity, predominantly within the dorsal columns of the spinal cord, with variable involvement of the lateral corticospinal tracts. These cases highlight the ready availability of nitrous oxide and the fact that heavy prolonged recreational use is occurring in the community. It is important that clinicians in emergency and community settings are alerted to this unusual cause of subacute combined degeneration of the spinal cord because early aggressive vitamin B12 replacement together with behavioural change can reverse this disabling neurological syndrome.     

NADPH diaphorase in the spinal cord of rats.

To identify spinal neurons that may synthesize nitric oxide, cells and fibers histochemically stained for NADPH diaphorase (a nitric oxide synthase) were studied in the spinal cord of rats. The histochemical reaction gave an image similar to the best Golgi impregnations, staining cells down to their finest processes. Transverse, horizontal, and parasagittal 50 and 100 microns sections were used to follow dendritic and axonal arborizations of stained neurons. Major cell groups were identified in the superficial dorsal horn and around the central canal (at all spinal levels), and in the intermediolateral cell column (at thoracic and sacral levels). Scattered positive cells were also found in deeper dorsal horn, ventral horn, and white matter. In some cases, axons of cells in the dorsal horn could be traced into the white matter; many of these cells resembled neurons projecting to various supraspinal targets. Stained cells in the intermediolateral column, which sent their axons into the ventral root, were presumed to be preganglionic autonomic neurons. Dense plexes of fibers were stained in laminae I and II and in the intermediolateral column. A large number of NADPH diaphorase-positive neurons in the spinal cord appear to be involved in visceral regulation. Fibers of the intermediolateral system had a special relationship with vasculature, suggesting that nitric oxide may help to couple neural activity with regional blood flow in the spinal cord. The abundance of NADPH diaphorase-positive neurons and fibers in the superficial dorsal horn suggests that nitric oxide may also be involved in spinal sensory processing.     

A case of benign postpartum anterior spinal artery syndrome with thrombocytosis and high fibrolytic activity

A 35-year-old woman was admitted to our hospital with acute onset paraparesis, sensory disturbance in her lower body, and urinary disturbance. She had given birth by normal vaginal delivery 6 days before the onset of these symptoms. On admission, paresis of her left leg was more severe than that of her right leg. Deep tendon reflexes of her lower limbs were hyperactive, and bilateral Babinski reflexes were observed. The patient's thermal and pain sensations below the lumbus were impaired. She lost bladder-filling sensation and bladder control. She showed an anterior spinal artery syndrome. Blood analysis revealed thrombocytosis and high fibrolytic activity. MRI of the brain and spinal cord did not show any lesions. Cerebrospinal fluid measurements showed that parameters were within the normal range and that the protein levels were not increased. We considered that a spinal cord infarction had occurred. After administration of antithrombotic agents and methylpredonisolone, her symptoms gradually improved. The coagulation disorders during the peripartum period were assumed to be a cause of anterior spinal artery syndrome in this case. It is very rare for perinatal women to have a spinal cord infarction. Only 3 cases of spinal cord infarction during the perinatal period have been reported thus far.     

A case of spinal dural arteriovenous fistula draining to the ventral coronal venous plexus]

Here we report a case of spinal dural areteriovenous fistula (AVF) draining to the anterior spinal vein. An 80-year-old female presented with progressive weakness of lower extremities. MRI showed spinal enlargement at the Th10 to L1 with high intensity signals on T2-weighted image and multiple flow voids on the dorsal and ventral surface of the spinal cord. Angiogram of the left L2 lumbar artery demonstrated a hairpin-shaped vessel with ascending and descending limbs, mimicking radiculomedullary artery. Oblique view angiogram of the left L2 lumbar artery showed that radiculomedullary vein drained to the dilated anterior spinal vein, which then drained cranially and caudally on the anterior and posterior surface of the spinal cord. The patient underwent T9-L2 laminectomy. Several large tortuous dilated veins in the subarachnoid space were found. Examination of the inner surface of the dura revealed an arterialized vein that began at the level of L2 and coursed superiorly. The arterialized vein was coagulated and interrupted. The postoperative angiogram demonstrated the obliteration of the fistula. Postoperative MRI returned to normal with complete disappearance of T2 high signal, cord enlargement. In most spinal dural AVF, the venous drainage is predominantly upward on the posterior surface of the spinal cord. The spinal dural AVF draining to the anterior spinal vein is atypical, and cause difficulty in differentiating the anterior spinal artery from the anterior spinal vein. Oblique view angiogram may be helpful to differentiate the anterior spinal vein from anterior spinal artery.     

Alpha-2-adrenergic receptors in avian spinal cord: increases in apparent density associated with the sympathetic preganglionic cell column

Vertebrate spinal cord receives a dense and diversified catecholaminergic innervation from brainstem and diencephalon. Within the spinal gray, the densest terminations appear to be within the neuropil surrounding sympathetic preganglionic neurons (SPNs) in thoracic spinal cord. Results of recent iontophoresis investigations showed that several catecholamines and clonidine, an alpha-2 agonist, uniformly inhibited the maintained discharge activity of SPNs [19]. These experiments raised the possibility that the inhibitory effects might be mediated by activation of an alpha-2-adrenergic receptor. The present series of ligand binding studies provide biochemical evidence suggesting the presence of alpha-2-adrenergic receptors in the SPN cell column. Total specific binding (Bmax) of the radiolabeled agonists clonidine (CLO) and para-amino-clonidine (PAC) (at concentrations above and below apparent KDS) was significantly greater in thoracic spinal cord in comparison with cervical spinal cord (P less than 0.001). The elevated levels in thoracic spinal cord were entirely accounted for by increases in apparent receptor density in dorsal horn and the SPN cell column (inclusive of the adjoining intermediate spinal laminae) (P less than 0.005). Adrenergic receptor subtype specificity of [3H]PAC was tested in competitive inhibition experiments. The results confirmed that [3H]PAC is a preferential alpha-2 agonist in thoracic and cervical spinal cord, and indicated the following rank order of potency for its displacement: norepinephrine = yohimbine much greater than prazosin greater than propranolol.     

Intracranial dural arteriovenous fistula with spinal venous drainage]

Two cases of intracranial dural arteriovenous fistulae draining into the medullary veins are reported. One patient was a 68-year old man who experienced brief and repeated episodes of paraplegia, followed by a permanent spinal cord syndrome. The other patient was a 74-year-old woman who rapidly developed a syndrome of the medulla and spinal cord. In both cases spinal cord angiography failed to show vascular malformations, but myelography revealed venous imprints and magnetic resonance imaging of the cervical spinal cord disclosed a pre-medullary signal. Cerebral angiography showed an intracranial arteriovenous fistula fed by the external carotid artery and draining into the veins of the posterior fossa and the spinal venous network. Complete occlusion of the fistula was achieved by embolization with microparticles. Intracranial dural arteriovenous fistulae draining into the medullary veins are rare: to our knowledge, only 4 cases have been reported. Such lesions must be considered whenever a spinal cord syndrome has no detectable cause.     

Cervical intradural ventral arachnoid cyst resected via anterior corpectomy with reconstruction: a case report

Intradural arachnoid cysts are rare lesions that frequently arise posterior to the spinal cord in the thoracic spine region. Those located at the cervical spine level, anterior to the spinal cord are even rarer. The usual treatment of symptomatic intradural spinal cysts involves surgical removal through a posterior approach using a laminectomy or laminotomy. However, ventrally located intradural cysts are frequently not amenable to complete resection without undue manipulation of the cord and aggressive removal through a posterior approach may result in spinal cord injury. The authors present a 29-year-old male harbouring an intradural ventral cervical arachnoid cyst which was successfully resected via an anterior approach with corpectomy and reconstruction. CONCLUSION: For purely ventral cervical intradural arachnoid cysts, which compress the spinal cord dorsally, an anterior approach can allow access to the lesion without any need for intraoperative manipulation of the spinal cord. For such cases, the anterior approach prevents the consequent risk of neurological injury due to posterior approaches.     

Trajectory of spinocerebellar fibers passing through the inferior and superior cerebellar peduncles in the rat spinal cord: a study using horseradish peroxidase with pedunculotomy

The course of spinocerebellar fibers in the rat spinal cord was investigated by injecting horseradish peroxidase into the cerebellar anterior vermis after complete transection of the left inferior and right superior cerebellar peduncles. By this procedure, fibers passing via the inferior cerebellar peduncles (icp-fibers) were labeled retrogradely on the right side of the spinal cord, whereas fibers passing via the superior cerebellar peduncles (scp-fibers) were labeled on the left side. Crossed icp-fibers were located diffusely in the anterior and lateral funiculi in the sacral to lower lumbar segments. They gradually migrated laterally and dorsally in these funiculi and received many uncrossed icp-fibers moving laterally in the lateral funiculus from the gray substance in the upper lumbar to lower thoracic segments. These mixed fibers shifted more dorsally and laterally in the anterior and lateral funiculi to aggregate in the narrow peripheral zone of the lateral funiculus in the upper thoracic and lower cervical segments, and received many crossed fibers in the upper cervical segments. There were more icp-fibers than scp-fibers through the spinal cord. However, the extent of scp-fibers in the anterior and lateral funiculi was essentially the same as that for icp-fibers, except that a few scp-fibers were found in the dorsolateral marginal zone of the lateral funiculus. It has been generally accepted that the dorsal spinocerebellar tract ascends in the dorsal half of the lateral funiculus and enters the cerebellum via the inferior cerebellar peduncle, whereas the ventral spinocerebellar tract ascends in the ventral half of it and takes the superior cerebellar peduncle route. The results of this study suggest that it is necessary to revise this concept.