Spinal Cord Involvement Research Articles

A Case Report of Abnormal Spina Bifida Cystica

Introduction : Spina bifida is a developmental congenital disorder caused by the incomplete closure of the embryonic neural tube. Some vertebrae overlying the spinal cord are not fully formed and remain unfused and open. If the opening is large enough, this allows a portion of the spinal cord to protrude through the opening in the bones. Aims of Study : To study an abnormal case of Spina Bifida Cystica Material and Method : The present study is a case report of a 11 day old female child who reported to the pediatric surgery department with the chief complaint of a cystic swelling of 53cm in a thoraco lumber region since birth. The swelling was soft but tense and the skin over the swelling was thinned out. The child showed some signs of lower limb involvement which need to confirmed. Result : On anatomical examination it was found to be a case of developmental anomaly of vertebral column in thoraco lumbar region which is against the lumbo-sacral region which is the most common site, with spinal cord involvement (neural tube defect). On test of illumination, it was found to be partially illuminated and X rays and MRI findings showed non fusion of vertebral column with abnormal protrusion of spinal cord through the defect. Conclusion : It is a case of meningomyelocele( spina bifida) caused by abnormal closure of neural folds of IIIrd and IV weeks of developments leading to neural tube defect. It was a spina bifida of cystic variety which mostly accomplish with lower limb involvement.

A cluster of transverse myelitis following dengue virus infection in the brazilian Amazon region.

Background and Purpose: During the last two decades, clinical reports have begun to place increasing emphasis on the possible neurological complications related to dengue. However, reports of cases with myelitis post dengue are rare. This study describes an unprecedented cluster of transverse myelitis following a dengue virus infection.Methods: 51 possible cases of neurological complications related to dengue were identified by the epidemiological surveillance of the State of Rondônia, Brazil and submitted to serial neurological examination, electromyography, vertebral MR and laboratory investigation to confirm the dengue diagnosis and rule out other arboviruses.Results: The diagnosis of acute transverse myelitis post-dengue was established in 26 patients, the majority were women, young and white. Antibodies against virus IgM were present in all cases and DEN 3 virus was isolated by PCR in one patient. Treatment with IV steroids was useful.Conclusions: The cluster of transverse myelitis post-dengue with favorable clinical outcome here reported suggests an immune mediated mechanism for the spinal cord involvement. Whereas dengue epidemics are frequent in tropical and subtropical countries, the dengue fever should be part in the differential diagnosis of the infectious and post-infectious myelitis.

Central Pontine and Extrapontine Myelinolysis: The Great Masquerader—An Autopsy Case Report

Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by rapid correction of chronic hyponatremia. The clinical features vary depending on the extent of involvement. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We herein report a case of myelinolysis involving pons, lateral geniculate bodies, subependymal region, and spinal cord. To the best of our knowledge, this case represents the second case of spinal cord involvement in osmotic demyelination syndrome and the first case of involvement of thoracic region of spinal cord.

Pilomyxoid astrocytoma of the thoracic spinal cord in an adult: A case report and review of literature

We present a case of pilomyxoid astrocytoma (PMA) in a 35-year-old Asian male with history of paraparesis for last 6 months. A contrast-enhanced magnetic resonance imaging of the spine revealed an intramedullary mass lesion occupying most of the thecal sac at the level of 10 th and 11 th dorsal vertebrae, with extensive contrast enhancement. Spinal PMA in an adult is an extremely rare entity, with only two reported cases in the literature, until date. This appears to be the first reported case of spinal PMA in an adult with isolated thoracic spinal cord involvement.

Surgical manifestations of thoracic arachnoid pathology: series of 28 cases

Object Various pathologies involving the thoracic arachnoid mater uniformly manifest as thoracic myelopathy and may present a significant management dilemma. The authors undertook this study to assess outcome in cases of thoracic myelopathy due to thoracic arachnoid pathology. Methods The authors have cared for and followed 28 patients with thoracic myelopathy from thoracic arachnoid pathology over the last 17 years. A chart review and contemporary follow-up of these patients was performed and outcomes were reported. Results Patients with thoracic myelopathy from thoracic arachnoid pathology often have improvement in their condition after surgical decompression/detethering procedures. While not universal, patients in this series had improvement in mJOA scores at 1 year after surgery (p = 0.0001) and at last follow-up (p = 0.04). Results indicated that across a wide variety of pathologies the extent of thoracic spinal cord involvement is a predictor of the disease course and outcome. Comparison of the group of patients with cord involvement limited to 2 vertebral segments (short-segment pathology) versus the group with cord tethering of more than 2 segments (long-segment pathology) showed that patients in the short-segment group more frequently had ventral or dorsal arachnoid bands (p = 0.003), more frequently had signal change in the cord on MRI (p = 0.02), and less frequently presented with a syrinx (p = 0.02), and a smaller percentage of patients in this group underwent reoperation (p = 0.02). While patients with short-segment pathology typically improved after a single operative intervention, patients with long-segment pathology typically improved after multiple operations, frequently for CSF diversion. Conclusions Thoracic arachnoid pathology causing thoracic cord dysfunction and myelopathy is varied, has multiple etiologies, and can be difficult to treat over the long term. Surgical management, when indicated, is case specific. Serial long-term follow-up is essential to document enduring clinical and radiographic success.

A case of Susac syndrome with cervical spinal cord involvement on MRI

We describe a typical case presentation of Susac syndrome with a novel MRI finding of cervical spinal cord involvement. A 25-year-old, 14-week gestation white woman presented with two episodes of encephalopathy, responsive to steroids, with abnormal brain magnetic resonance imaging (MRI) concerning for Susac syndrome. Further studies confirmed the clinical triad of encephalopathy, branch retinal artery occlusions and hearing loss pathognomonic for Susac syndrome. Cervical spine MRI demonstrated two focal areas of high-signal abnormality at C2 and C3. We provide a brief review of Susac syndrome, data regarding pregnancy in this rare syndrome, and discuss how this unique observation may assist in the medical management of such cases and contribute to the understanding of the underlying pathophysiology.

Efficacy of infliximab in neuro-Behçet’s disease presenting with isolated longitudinally extensive transverse myelitis

Efficacy of infliximab in neuro-Behçet’s disease presenting with isolated longitudinally extensive transverse myelitis

Diagnostic use of dermatomal somatosensory-evoked potentials in spinal disorders: Case series

Objective/ContextDermatomal somatosensory-evoked potentials (dSEPs) may be valuable for diagnostic purposes in selected cases with spinal disorders.DesignReports on cases with successful use of dSEPs.FindingsCases 1 and 2 had lesions causing multiple root involvement (upper to middle lumbar region in Case 1 and lower sacral region in Case 2). Cystic lesions in both cases seemed to compress more than one nerve root, and stimulation at the center of the involved dermatomes in dSEPs helped to reveal the functional abnormality. Cases 3 and 4 had lesions involving the spinal cord with or without nerve root impairment. In Case 3, an magnetic resonance imaging (MRI)-verified lesion seemed to occupy a considerable volume of the lower spinal cord, causing only very restricted clinical sensory and motor signs. In Case 4, a cervical MRI showed a small well-circumscribed intramedullary lesion at right C2 level. All neurophysiological investigations were normal in the latter two patients (motor, tibial, and median somatosensory-evoked potentials in Case 3, and electromyography in both) except for the dSEPs.ConclusionsObjectifying the presence and degree of sensory involvement in spinal disorders may be helpful for establishing diagnoses and in therapeutic decision-making. Valuable information could be provided by dSEPs in selected patients with multiple root or spinal cord involvement.

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.

129. Heart rate variability in patients with spinal cord injuries

In spinal cord injury both the level and the extension of the lesions have been related to different response to tilting in Low Frequency (LF) and High Frequency (HF) oscillations of heart rate. To understand the role of sympatho–vagal interaction in such patients, we tried to evaluate, using the LF/HF ratio, the residual possibility in maintaining the cardiovascular homeostasis. 9 subjects with different outcome of traumatic (4) and inflammatory (5) spinal cord involvement, with lesion between C6 and T10, were investigated with power spectral analysis of the blood pressure and heart rate variability in resting condition and during a period of head – up tilt test. The difference between the two conditions was tested using the Wilcoxon signed rank test. In patients with complete ASIA A lesion, the relatively preserved LF activity in resting condition did not increase during tilting, with a parallel decrease of LF/HF ratio. In patients with incomplete lesion (ASIA B, C, D), the LF power of R–R interval showed an inverse relation with the level of the lesion. The LF/HF ratio was partially maintained due to the concomitant adapting HF activity permitting an efficient sympatho–vagal balance.

Brainstem manifestations in neuromyelitis optica: a multicenter study of 258 patients

Background: Neuromyelitis optica (NMO) is a severe autoimmune disease of the central nervous system characterized by spinal cord and optic nerve involvement. Brainstem manifestations have recently been described. Objective: To evaluate the time of occurrence, the frequency and the characteristics of brainstem symptoms in a cohort of patients with NMO according to the ethnic background and the serologic status for anti-aquaporin-4 antibodies (AQP4-abs). Methods: We performed a multicenter study of 258 patients with NMO according to the 2006 Wingerchuk criteria and we evaluated prospectively the frequency, the date of onset and the duration of various brainstem signs in this population. Results: Brainstem signs were observed in 81 patients (31.4%). The most frequently observed signs were vomiting (33.1%), hiccups (22.3%), oculomotor dysfunction (19.8%), pruritus (12.4%), followed by hearing loss (2.5%), facial palsy (2.5%), vertigo or vestibular ataxia (1.7%), trigeminal neuralgia (2.5%) and other cranial nerve signs (3.3%). They were inaugural in 44 patients (54.3%). The prevalence was higher in the non-Caucasian population (36.6%) than in the Caucasian population (26%) (p<0.05) and was higher in AQP4-ab-seropositive patients (32.7%) than in seronegative patients (26%) (not significant). Conclusions: This study confirms the high frequency of brainstem symptoms in NMO with a majority of vomiting and hiccups. The prevalence of these manifestations was higher in the non Caucasian population.

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL): Assessment of the involved white matter tracts by MRI

Background and purposeLeukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL) is a recently identified autosomal recessive disorder with early onset of symptoms and slowly progressive pyramidal, cerebellar and dorsal column dysfunction. LBSL is characterized by distinct white matter abnormalities and selective involvement of brainstem and spinal cord tracts. The purpose of this study is to assess the imaging features of the involved white matter tracts in cases of LBSL by MRI. Patients and methodsWe retrospectively reviewed the imaging features of the selectively involved white matter tracts in sixteen genetically proven cases of leukoencephalopathy with brainstem and spinal cord involvement and elevated brain lactate (LBSL). All patients presented with slowly progressive cerebellar sensory ataxia with spasticity and dorsal column dysfunction. MRI of the brain and spine using 1.5T machine and proton magnetic resonance spectroscopy (1H MRS) on the abnormal white matter were done to all patients. The MRI and MRS data sets were analyzed according to lesion location, extent, distribution and signal pattern as well as metabolite values and ratios in MRS. Laboratory examinations ruled out classic leukodystrophies. ResultsIn all cases, MRI showed high signal intensity in T2-weighted and FLAIR images within the cerebral subcortical, periventricular and deep white matter, posterior limbs of internal capsules, centrum semiovale, medulla oblongata, intraparenchymal trajectory of trigeminal nerves and deep cerebellar white matter. In the spine, the signal intensity of the dorsal column and lateral cortico-spinal tracts were altered in all patients. The subcortical U fibers, globi pallidi, thalami, midbrain and transverse pontine fibers were spared in all cases. In 11 cases (68.8%), the signal changes were inhomogeneous and confluent whereas in 5 patients (31.2%), the signal abnormalities were spotty. MRI also showed variable signal abnormalities in the sensory and pyramidal tracts in addition to the brainstem and cerebellar connections. Proton MRS showed consistent elevation of the lactate within the abnormal white matter. ConclusionDistinct MRI findings in the form of selective affection of subcortical and deep white matter tracts of the brain (involving the posterior limb of internal capsules and sparing the subcortical U fibers), dorsal column and lateral cortico-spinal tracts of the spinal cord should lead to the diagnosis of LBSL supported by the presence of lactate peak in 1H MRS. The disease can be confirmed by the analysis of the disease gene DARS2.

Gorham's disease of the spine

Gorham's disease of the spine is very rare and usually associated with poor prognosis. Till date very few cases have been reported in the literature. It is characterized by osseous invasion by angiomatous vascular mass without skip areas, eventually causing lysis of affected bone. Morbidity and mortality are high in those with spinal and/or visceral involvement. Neurological complications increase the mortality to 33% whereas with chylothorax, mortality increases to more than 50%. To describe the clinical course and the challenges in planning and implementing effective rehabilitation services for management of patients with Gorham's disease of the spine. We report two patients with Gorham's disease who had neurological deficits secondary to spinal cord involvement, and their rehabilitation perspectives. Both patients achieved satisfactory ambulation and functional outcomes. Multiple revision surgeries may be required to ensure spinal stability. When working with these patients, one must remain vigilant about spinal stability as well as about possible serious pulmonary complications, and be prepared to make appropriate management decisions when necessary.

Enfermedades menos conocidas de la mielina: lesiones desmielinizantes focales seudotumorales

IntroductionFocal tumour-like demyelinating lesions are defined as solitary demyelinating lesions with a diameter greater than 2cm. In imaging studies, these lesions may mimic a neoplasm or brain abscess; as a result, invasive diagnostic and therapeutic measures may be performed that will in some cases increase morbidity. Our aim was to analyse and characterise these lesions according to their clinical, radiological, and pathological characteristics, and this data in addition to our literature review will contribute to a better understanding of these lesions. MethodsThis descriptive study includes 5 cases with pathological diagnoses. We provide subject characteristics gathered through reviewing their clinical, radiology, and pathology reports. ResultsPatients’ ages ranged from 12 to 60 years; 3 patients were female. The time delay between symptom onset and hospital admission was 3 to 120 days. Clinical manifestations were diverse and dependent on the location of the lesion, pyramidal signs were found in 80% of patients, there were no clinical or radiological signs of spinal cord involvement, and follow-up times ranged from 1 to 15 years. ConclusionBrain biopsy is the gold standard for the diagnosis of demyelinating tumour-like lesions; however, their clinical features, along with several magnetic resonance imaging features such as open ring enhancement, venular enhancement, the presence of glutamate in spectroscopy, and others, may be sufficient to differentiate neoplastic lesions from focal tumour-like demyelinating lesions.

Autoimmune diseases go well together — Multiple autoimmune syndrome type 3

CO RR EC TE D PR OO F ataxia; extensor plantar reflexes;and hypoesthesia from D5–D9. Cerebrospinal fluid examination revealed a normal cytochemical analysis but multiple type 2 oligoclonal bands — bacteriological and mycobacterium cultures, Borrelia spp, neurotropic virus, and immunological exam were negative and neoplastic cells absent. Magnetic Resonance Imaging (MRI) of the spine showed multiple intra-axial lesions with hypersignal in T2 and contrast enhancement— suggestive of an inflammatory process. Blood work revealed the presence of antibody antiSSA (RO52), with the rest of autoimmunity tests normal – Reumathoid Arthritis, Lupus and other Autoimmune Diseases excluded – and thyroid function, vitamine B12, and folic acid were also normal. The Schirmer test was under normal values, and salivary gland biopsy confirmed the diagnosis of Sjogren Syndrome. Steroid therapy was started with Prednisone with improvement. Three months after discharge she referred weakness in the lower extremities, with no other changes in the neurological exam. MRI showed great improvement of the former lesions. An electromyogram was performed — compatible with Myopathy. Muscle biopsy revealed steroid induced myopathy, and the patient suspended Prednisone and started Azatyoprin, achieving symptom regression. Conclusion: Neurological involvement in PSS occurs in about 20% of patients, from which spinal cord involvement is by far themost frequent, aswell as themost severe. Despite being the first line of treatment, steroid therapy has many collateral effects, and isn't always tolerated.

A Case with Leukoencephalopathy with Brainstem and Spinal Cord Involvement and Elevated Lactate (LBSL) with Its Characteristic Clinical and Neuroimaging Findings

A Case with Leukoencephalopathy with Brainstem and Spinal Cord Involvement and Elevated Lactate (LBSL) with Its Characteristic Clinical and Neuroimaging Findings

O22 – 1585 New insights into the spectrum of phenotypes and genotypes in Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL)

O22 – 1585 New insights into the spectrum of phenotypes and genotypes in Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL)

O14 – 1917 Hypomyelination with brain stem and spinal cord involvement and severe leg spasticity (HBSL): mutations in DARS are responsible

O14 – 1917 Hypomyelination with brain stem and spinal cord involvement and severe leg spasticity (HBSL): mutations in DARS are responsible

Changes in normal appearing spinal cord in multiple sclerosis: another brick in the wall

It is needless to say how magnetic resonance (MR) has changed drastically the clinical management of multiple sclerosis (MS). Research in this field has also been much influenced by advances in MR techniques. Conventional MR is invaluable for the detection of MS plaques, using T2, protondensity and FLAIR (fluid attenuated inversion recovery) images. Inflammatory activity can be observed by means of postcontrast T1-weighted images. Together, they are the basis of the present diagnostic criteria for dissemination in space and time that characterizes MS1. However, the discrepancy between clinical manifestations of the disease and conventional MR metrics for lesion burden is well known, the so-called clinico-radiological paradox2. Some of the reasons listed to explain this dissociation include: inappropriate clinical rating, lack of histopathological specificity, neglecting spinal cord involvement, underestimating damage to the normal appearing brain tissue; and masking cortical adaptation effects2. MR has once again contributed enormously in this regard. Quantitative techniques (such as magnetization transfer and diffusion-weighted MR) disclose damage not only in conventional MR-visible lesions, but also in normal appearing tissues; MR spectroscopy adds metabolic information and functional MR demonstrates how cortical activation is affected during the course of the disease3. A robust body of evidence has emerged from all these data, pointing out to our current understanding of MS as being not only a chronic inflammatory / demyelinating disease, but also a degenerative condition of the central nervous system. Another paradigm shift is related to the pathophysiology of MS: it is not only a white matter disease, characterized by focal inflammatory lesions, but it also involves more subtle and diffuse damage throughout white and gray matter3. Specifically focusing on the spinal cord, it is a highly organized and clinically eloquent structure that is often affected by MS. Due to its peculiar anatomic arrangement, the cord is a useful model to assess the relations between inflammation, demyelination, and axonal pathology4. Diffusion tensor imaging (DTI) is one of the most important quantitative techniques. It is a refinement of the conventional diffusion-weighted imaging, able to measure the random diffusional motion of water molecules and provide quantitative indices of structural and orientational features of tissues5,6. Among the most used DTI indices we can mention mean diffusivity (MD), which is a directionally averaged measure of the apparent diffusion coefficient, and fractional anisotropy (FA), which summarizes the orientational dependence of diffusivity7. More recently, new DTI metrics were introduced, probing new aspects of tissue structure and disease-related changes, such as axial (AD) and radial diffusivity (RD). AD is considered to represent the water diffusion in parallel to the main structure of the white matter fibers, while RD represents the water diffusion perpendicular to them. It has been postulated, firstly in studies involving animals, that decreased AD and increased RD could represent surrogate markers of axonal damage and demyelination, respectively8. Performing DTI of the spinal cord is much more challenging than of the brain, due to the small cross section of the cord, pulsatile cord motion, and field inhomogeneities caused by variations in susceptibility from nearby vertebrae9. Nevertheless, in spite of these limitations, Neuroradiologist, Instituto de Radiologia do Hospital das Clinicas da Faculdade de Medicina da USP. CDB – Centro de Diagnosticos Brasil, Sao Paulo SP, Brazil. Correspondence Leandro Tavares Lucato Rua Prof Pedreira de Freitas 372 / apto 101 / bloco 2 03312-052 Sao Paulo SP Brasil E-mail: ltlucato@uol.com.br Conflict of interest There is no conflict of interest to declare.

Letter to the editor concerning “hepatic myelopathy with spastic paraparesis: report of two cases and review of the literature” by S. Ben Amor et al. (Eur Spine J. 2013, Jun 1)

To the Editor, We read with great interest the article by Ben Amor et al. [1] published in the European Spine Journal entitled “Hepatic myelopathy with spastic paraparesis: report of two cases and review of the literature”. The authors state in their paper that there is no special diagnostic tool for hepatic myelopathy (HM), and that the diagnosis has to be established on clinical grounds after exclusion of other possible entities. However, in our opinion, there is a noticeable omission in this important article. Our previous study [2] revealed that motor evoked potentials (MEP) elicited by transcranial magnetic stimulation (TMS) may be very helpful in the diagnosis of this rare complication of chronic liver diseases that is usually associated with a portosystemic shunt and likely to be overlooked. The authors demonstrated that central motor conduction studies by means of TMS are sensitive, accurate and easily applicable methods for detecting even a subclinical damage of the corticospinal tract. The threshold for corticospinal electrophysiological abnormalities was below that for clinical and/or radiological changes. Spinal cord involvement was evident even at the preclinical stage and became more overt as the disease progressed. Most of the examined patients did not have clinical symptoms and presented only physiological subclinical abnormalities. MEP studies may thus disclose an impairment of the corticospinal pathways even before HM is clinically manifest and enable an early diagnosis of HM. Since, liver transplantation (the first therapeutic option) should be performed before spinal cord damage becomes irreversible [3–5], the diagnosis of HM must be established as early as possible to enhance the chance of a complete recovery of the spinal cord. Therefore, we believe that such an important examination, which is at present widely and easily performed in the most neurophysiology laboratories, cannot be neglected. We found important to highlight that, especially in a review article on HM, this important diagnostic tool should be included and discussed.