Spontaneous Subarachnoid Haemorrhage in Spinal Hemangioblastoma: Illustrative Case and Discussion of a Pathophysiological Hypothesis.

Spontaneous non-aneurysmal subarachnoid haemorrhage (naSAH) is an unusual finding that could be burdened by significant mortality and morbidity rates. Rare pathologies and delayed diagnosis could be advocated as responsible of unfavourable outcomes. Herein, we describe an exceedingly rare giant lumbar spinal hemangioblastoma (80 × 23 mm) presenting as an intracranial naSAH. Based on our radiological and clinical findings a pathophysiological hypothesis linking intracranial naSAH to venous hypertension was discussed for the first time even among lumbar spinal tumors. Although rare, unusual causes should be investigated in presence of radiological atypical finding as a prompt evaluation and treatment could be needed.

Lentiviral-Induced Spinal Cord Gliomas in Rat Model.

Intramedullary spinal cord tumors are a rare and understudied cancer with poor treatment options and prognosis. Our prior study used a combination of PDGF-B, HRAS, and p53 knockdown to induce the development of high-grade glioma in the spinal cords of minipigs. In this study, we evaluate the ability of each vector alone and combinations of vectors to produce high-grade spinal cord gliomas. Eight groups of rats (n = 8/group) underwent thoracolumbar laminectomy and injection of lentiviral vector in the lateral white matter of the spinal cord. Each group received a different combination of lentiviral vectors expressing PDGF-B, a constitutively active HRAS mutant, or shRNA targeting p53, or a control vector. All animals were monitored once per week for clinical deficits for 98 days. Tissues were harvested and analyzed using hematoxylin and eosin (H&E) and immunohistochemical (IHC) staining. Rats injected with PDGF-B+HRAS+sh-p53 (triple cocktail) exhibited statistically significant declines in all behavioral measures (Basso Beattie Bresnahan scoring, Tarlov scoring, weight, and survival rate) over time when compared to the control. Histologically, all groups except the control and those injected with sh-p53 displayed the development of tumors at the injection site, although there were differences in the rate of tumor growth and the histopathological features of the lesions between groups. Examination of immunohistochemistry revealed rats receiving triple cocktail displayed the largest and most significant increase in the Ki67 proliferation index and GFAP positivity than any other group. PDGF-B+HRAS also displayed a significant increase in the Ki67 proliferation index. Rats receiving PDGF-B alone and PDGF-B+ sh-p53 displayed more a significant increase in SOX2-positive staining than in any other group. We found that different vector combinations produced differing high-grade glioma models in rodents. The combination of all three vectors produced a model of high-grade glioma more efficiently and aggressively with respect to behavioral, physiological, and histological characteristics than the rest of the vector combinations. Thus, the present rat model of spinal cord glioma may potentially be used to evaluate therapeutic strategies in the future.

Intraoperative identification of the corticospinal tract and dorsal column of the spinal cord by electrical stimulation.

OBJECTIVES: Anatomical identification of the corticospinal tract (CT) and the dorsal column (DC) of the exposed spinal cord is difficult when anatomical landmarks are distorted by tumour growth. Neurophysiological identification is complicated by the fact that direct stimulation of the DC may result in muscle motor responses due to the centrally activated H-reflex. This study aims to provide a technique for intraoperative neurophysiological differentiation between CT and DC in the exposed spinal cord. METHODS: Recordings were obtained from 32 consecutive patients undergoing spinal cord tumour surgery from July 2015 to March 2017. A double train stimulation paradigm with an intertrain interval of 60 ms was devised with recording of responses from limb muscles. RESULTS: In non-spastic patients (55% of cohort) an identical second response was noted following the first CT response, but the second response was absent after DC stimulation. In patients with pre-existing spasticity (45%), CT stimulation again resulted in two identical responses, whereas DC stimulation generated a second response that differed substantially from the first one. The recovery times of interneurons in the spinal cord grey matter were much shorter for the CT than those for the DC. Therefore, when a second stimulus train was applied 60 ms after the first, the CT-fibre interneurons had already recovered ready to generate a second response, whereas the DC interneurons were still in the refractory period. CONCLUSIONS: Mapping of the spinal cord using double train stimulation allows neurophysiological distinction of CT from DC pathways during spinal cord surgery in patients with and without pre-existing spasticity.

Placode rotation in transitional lumbosacral lipomas: are there implications for origin and mechanism of deterioration?

PURPOSE: Rotation of the lipoma-neural placode has been noted in transitional lumbosacral lipomas. The purpose of this study was to confirm this rotation; that this rotation occurs with a preference to the left, and correlates with clinical symptoms. In addition, this study tests the hypothesis that this rotation occurs through local mechanical forces rather than intrinsic congenital malformation. METHODS: Lipomas were classified as per the Chapman classification. Degree of rotation of the placode from the coronal plane was recorded along with the presence of herniation outside of the vertebral canal. Abnormalities on urodynamic testing were recorded, along with neuro-orthopaedic signs picked up on formal neuro-physiotherapy assessment. RESULTS: Placode rotation occurs more frequently in the transitional group. Regardless of lipoma classification, rotation was much more common to the left. Furthermore, when lateralisation of symptoms was present, this strongly correlated with the direct of rotation. There was no difference in rotation of the placode whether it was within (lipomyelocoele) or without the vertebral canal (lipomyelomeningocoele). CONCLUSIONS: Placode rotation is a feature of transitional lumbosacral lipomas and may account for the increase in symptoms amongst this subgroup. Herniation of the placode outside the vertebral canal does not increase the risk of rotation suggesting a congenital cause for this finding rather than a purely mechanical explanation.

Diffusion tensor tractography of pyramidal tracts in patients with brainstem and intramedullary spinal cord tumors: Relationship with motor deficits and intraoperative MEP changes.

PURPOSE: To evaluate whether pyramidal tracts course alterations observed in diffusion tensor tractography (DTT) in cases of brainstem and intramedullary spinal cord tumors reflect patient clinical status and prognosis. MATERIALS AND METHODS: For this purpose, we assessed in 17 patients relationships between pyramidal tracts course alterations observed in DTT (classified into four categories: unaffected; displaced or interspaced; partially disintegrated and completely disintegrated) performed on a 1.5 Tesla scanner and the presence of preoperative motor deficits, changes observed in motor evoked potentials (MEPs) records at the beginning of the operation, deterioration of the MEPs records during the operation, and perioperative deterioration of muscle strength. RESULTS: We found that, if the picture of pyramidal tracts in DTT was worse, motor deficit was more common (P = 0.062). This observation was even more evident (P = 0.027), when cases with at least partially destroyed pyramidal tracts were compared with cases with normal or at most displaced or interspaced by tumor but still preserved pyramidal tracts. Significant relationships were also found between changes in DTT and abnormal MEP records at the beginning of the operation (P = 0.032) and perioperative deterioration of muscle strength (P = 0.0058). CONCLUSION: A close relationship was found between pyramidal tracts course alterations in DTT imaging and preoperative motor status and especially with changes in the MEP records at the beginning of the operation. DTT may be a method that allows the better planning of brainstem and intramedullary spinal cord tumors operations and may help in the risk assessment of postoperative motor deficits. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 4 J. MAGN. RESON. IMAGING 2017;46:715-723.

Natural history of intramedullary spinal cord ependymoma in patients preferring nonoperative treatment.

Surgical resection of intramedullary spinal cord ependymoma still remains the standard of care but is challenging and occasionally associated with poor outcome. The aim of this study is therefore to provide additional information regarding the natural history of conservatively treated symptomatic intramedullary spinal cord ependymoma. Retrospective, single center review of all patients with intramedullary spinal cord ependymoma treated conservatively (wait and see) between 1980 and 2016. The neurological outcomes at first presentation, as well as in long-term follow-up, were assessed using the modified McCormick Disability Scale and modified Rankin Scale. Thirteen of 41 patients were managed conservatively and were included in the study. Mean age at the admission was 49 years. There were seven women and six men. All patients were symptomatic at the time of presentation. The mean follow-up from admission to the last neurological examination was 47.9 months. The mean modified McCormick score in conservatively treated patients was 1.3 at admission and 1.6 (p = 0.3) at last follow-up. There was no significant neurological detoriation over time in conservatively managed patients as assessed by the modified Rankin Scale at first presentation and last follow-up (mRS scores of 0-2, 100 vs 92%; p = 0.9). This cohort of conservatively managed patients with symptomatic intramedullary spinal cord ependymoma was clinically stable throughout the follow-up period. Our data provide additional information for counseling patients with intramedullary spinal cord tumors who chose a nonoperative treatment.

Monitoring rate and predictability of intraoperative monitoring in patients with intradural extramedullary and epidural metastatic spinal tumors.

STUDY DESIGN: Single-center retrospective study. OBJECTIVES: To evaluate the monitoring rate, sensitivity and specificity of intraoperative monitoring (IOM) during removal of intradural extramedullary (IDEM) or epidural metastatic spinal tumors. Also, to assess the efficacy of monitoring somatosensory-evoked potentials (SSEP) when motor-evoked potentials (MEP) are not measurable. SETTING: The Neuro-Oncology Clinic, National Cancer Center, Korea. METHODS: Patients (n=101) with IDEM or epidural metastatic spinal tumors at the cord level underwent surgeries monitored with SSEP and/or MEP. The monitoring rate was defined as negative when MEP or SSEP could not be measured after reversal of the neuromuscular block under general anesthesia. Positive IOM changes included more than a 50% change in the MEP or SSEP amplitude and more than a 10% delay in SSEP latency. RESULTS: MEP was measurable in 73% of patients. The MEP monitoring rate in patients with motor power grades of 3 or less was 39%, which was lower than that of SSEP (83%). The sensitivity, specificity and predictability of MEP for motor changes were 93, 90 and 91%, respectively. Conversely, the sensitivity, specificity and predictability of SSEP were 62, 97 and 89%, respectively. In patients in whom MEP was not measurable (n=24), SSEP was monitored with a predictability of 83%. CONCLUSION: In cases of extramedullary spinal tumors, MEP shows a higher sensitivity than SSEP does. However, the monitoring rate of MEP in non-ambulatory patients was lower than that of SSEP. In those cases, SSEP can be useful to monitor for postoperative neurological deficits.

Is intraoperative neurophysiological monitoring valuable predicting postoperative neurological recovery?

OBJECTIVES: We investigated the ability of intraoperative neurophysiological monitoring to predict postoperative neurological recovery in intradural-extramedullary spinal cord tumors. METHODS: From 2010 to 2014, we operated on 173 intradural-extramedullary spinal cord tumor patients with intraoperative neurophysiological monitoring. We retrospectively compared preoperative and postoperative clinical status using a modified McCormick grading scale and correlated with intraoperative neurophysiological monitoring. We followed patients for at least 1 year and correlated neurological outcomes with intraoperative changes in intraoperative neurophysiological monitoring. We then compared the degree of intraoperative neurophysiological monitoring change with the duration of the neurological deficit. RESULTS: Monitorability was 92% and 57% with transcranial motor-evoked potential and somatosensory-evoked potential modalities, respectively. Waveform attenuation on transcranial motor-evoked potentials was detected in 8.17% of cases. For somatosensory-evoked potentials, waveform attenuation was detected in 7% of the patients. A multimodality approach incorporating any transcranial motor-evoked potential changes had a sensitivity of 0.91 and a specificity of 0.98. The McCormick grade scale increased until 1 month in patients with alarm criteria on transcranial motor-evoked potentials (P<0.05). CONCLUSIONS: Patients suffered neurological deterioration in case of abolishment or >50% irreversible attenuation of the waveform in transcranial motor-evoked potentials. All patients gradually recovered after 1 postoperative month with alarm criteria from 50% to 80% irreversible amplitude drop on transcranial motor-evoked potentials.

Intraoperative neurophysiological mapping and monitoring in spinal tumor surgery: sirens or indispensable tools?

Spinal tumor (ST) surgery carries the risk of new neurological deficits in the postoperative period. Intraoperative neurophysiological monitoring and mapping (IONM) represents an effective method of identifying and monitoring in real time the functional integrity of both the spinal cord (SC) and the nerve roots (NRs). Despite consensus favoring the use of IONM in ST surgery, in this era of evidence-based medicine, there is still a need to demonstrate the effective role of IONM in ST surgery in achieving an oncological cure, optimizing patient safety, and considering medicolegal aspects. Thus, neurosurgeons are asked to establish which techniques are considered indispensable. In the present study, the authors focused on the rationale for and the accuracy (sensitivity, specificity, and positive and negative predictive values) of IONM in ST surgery in light of more recent evidence in the literature, with specific emphasis on the role of IONM in reducing the incidence of postoperative neurological deficits. This review confirms the role of IONM as a useful tool in the workup for ST surgery. Individual monitoring and mapping techniques are clearly not sufficient to account for the complex function of the SC and NRs. Conversely, multimodal IONM is highly sensitive and specific for anticipating neurological injury during ST surgery and represents an important tool for preserving neuronal structures and achieving an optimal postoperative functional outcome.

Dominant neurologic symptomatology in intravascular large B-cell lymphoma.

Intravascular large B-cell lymphoma (IVLBCL) is a rare variant of extranodal large B-cell lymphoma and it is characterized by selective intravascular proliferation of malignant cells. Typical features of the disease include aggressive behavior, rapid and frequently fatal course. Clinical picture is non-specific and heterogeneous, depending on the affected organ. It is not uncommon that this unique type of lymphoma is diagnosed post mortem. Herein, we report two cases of IVLBCL with neurologic symptomatology. In our clinical study patient 1 was an 80-year-old male with mixed paraparesis of lower extremities and difficulties with sphincter control. Patient 2 (56-year-old male) had vision malfunction, mental status changes and defect in phatic and motor functions. In both cases definite diagnosis was established by histological examination of necroptic material. We propose to include IVLBCL in differential diagnostic considerations in patients presenting with gradually impairing neurological status and spinal cord damage of unknown etiology (Fig. 2, Ref. 9).

[Intramedullary melanocytoma: a clinical case report and literature review]. / Intramedullyarnaya melanotsitoma: opisanie klinicheskogo nablyudeniya i obzor literatury.

The paper describes a rare clinical case of intramedullary melanocytoma, provides a detailed description of a pathomorphological study, and addresses the issues of differential diagnosis and surgical treatment.

[PREDICTING, POSSIBLE WAYS TO REDUCE AND CORRECTION OF MASSIVE INTRAOPERATIVE BLOOD LOSS IN SPINAL TUMOR SURGERY].

The paper discusses the problem ofpredicting, prevention and therapy of massive intraoperative blood loss in patients with metastasis in spine and spinal cord. We analyze 60 surgical cases in last 14 years in our clinic. Amount of blood loss was more that 80% of total blood volume in each case (from 2.5 to 17 liters). Preoperative selective angiography data on intensity of tumor blood supply were essential for blood loss prediction. Simultaneous embolization oftumor during angiography dramatically reduced intraoperative blood loss. Combination of blood saving techniques (preoperative autodonation, acute normovolemic hemodilution and intraoperative cell salvage) led to effective compensation of blood volume deficit and minimizing of allogenic blood transfusion. Plasma-derived and recombinant factors were effective in management of hemostatic disorders associated with massive blood loss.

Clinical characteristics and surgical outcomes of primary spinal paragangliomas.

Spinal paragangliomas are extremely rare tumors, most frequently involving the cauda equina and the filum terminale. We aimed to investigate the clinical manifestations, radiological features, management, and follow-up data of primary spinal paraganglioma. We present the clinical data and long-term outcomes from a consecutive surgical series of 19 patients with pathologically diagnosed spinal paragangliomas. All of the patients had undergone surgical resection. Pre- and postoperative magnetic resonance imaging was performed and follow-up data and neurological functional assessment are presented and discussed. The mean age at diagnosis was 47.7 years, with a significant male predominance. The primary clinical symptoms were low back pain and sciatica. Magnetic resonance images (MRI) showed characteristic signs that help differentiate paragangliomas from other spinal tumors, including a "salt & pepper" sign, serpiginous flow void, and a peripheral hypointense rim. Also, a well-encapsulated appearance can be found intraoperatively. During a mean follow-up period of 62.1 months, remnant tumor progression was noted on MRI in three patients with incomplete resection. Pain symptoms were relieved immediately after surgical intervention, while motor and sphincter dysfunction were much slower to improve. Differential diagnosis of paraganglioma based on MR images alone is challenging, but the presence of specific characteristic features provides suggestive clues; however, accurate diagnosis depends on pathological criteria. Despite the benign course, gross total resection is ideal, given an increased risk of recurrence in situ. Timely recognition and surgical treatment should be emphasized to avoid progressive neurological deficits.

Primary intramedullary non-Hodgkin's lymphoma in an immunocompetent child.

STUDY DESIGN: Case report. OBJECTIVES: Primary intramedullary spinal cord lymphoma is a rare entity. Studies have shown that there is a recent increase in the number of patients regardless of the status of the immunity. High index of suspicion should be kept in all patients with intramedullary tumors. Multidisciplinary approach at the earliest is required for best outcomes. SETTING: Department of Neurosurgery, Medical Oncology, Pathology and Radiation oncology. Nizam's Institute of Medical Sciences, Hyderabad, India. METHODS: We describe the case of an 11-year-old boy who presented with paraparesis and sensory loss below T10 level. On imaging, the dorsal spine showed intramedullary lesion mimicking an astrocytoma. RESULTS: Surgical decompression of the tumor was done and histopathology showed non-Hodgkin's lymphoma, diffuse large B-cell type. There were no findings suggestive of congenital or acquired immunodeficiency. After complete staging evaluation, we instituted chemotherapy with modified DeAngelis protocol. At 2 years post treatment, he is in complete remission with near normal neurological status. CONCLUSIONS: Intramedullary spinal cord diffuse B-cell lymphoma in a pediatric age group is very rare and hence requires a high index of suspicion in patients presented with myelopathy. The outcomes are encouraging with current multidisciplinary approach.

Supratentorial glioblastoma multiforme metastasizing to the cervical spinal cord.

Cases of glioblastoma multiforme (GBM) metastasizing to the leptomeninges or the intramedullary spine are rare and their prognosis are relatively poor. We present a case of supratentorial glioblastoma WHO grade IV which was later diagnosed to have cervical intramedullary metastasis 7 months after the primary surgery.

Intramedullary spinal cord astrocytomas: the influence of localization and tumor extension on resectability and functional outcome.

BACKGROUND: Intramedullary spinal cord tumors (IMSCT) are rare lesions, ependymomas and astrocytomas being the most common ones. Different studies have been published showing results of different treatment strategies as extensive/ limited surgery, biopsy and adjuvant radiation therapy with regard to functional outcome and survival. The present study was undertaken to analyse our series of surgically treated intramedullary astrocytomas in order to identify factors with impact on functional outcome and resectability. METHODS: Over a period of 20 years, among 215 patients with IMSCT 22 patients with astrocytomas were identified and enrolled into this analysis. Demographic data, clinical symptoms, localization and extension of the tumor, resection rate as well as pre- and postoperative neurological status were obtained. Patients were followed-up clinically and by MRI. RESULTS: Complete resection rate was higher in cervically located tumors (9 of 10) compared to non-cervical tumors (7 of 12). Tumor extension (1-3 segments vs. > 3 segments involved) did not influence on the resection rate. Cervical tumors showed a trend for better postoperative functional outcome than non-cervical lesions (3 of 10 cervical but 6 of 12 non-cervical tumors deteriorated postoperatively). In tumors extending more than 3 segments postoperative worsening was significantly increased. CONCLUSIONS: The present study shows a better resectability and functional outcome for cervically located intramedullary astrocytomas. Tumors extending more than three segments deteriorated significantly. These findings may help for decision-making process and treatment of these tumors.

Rehabilitation and treatment of spinal cord tumors.

CONTEXT: Due to advances in acute oncological treatment, patients with spinal cord tumors exhibit improved survival. However, these patients have not received the full benefits of rehabilitation services to address their neurological deficits and rehabilitation goals. OBJECTIVE: To evaluate the epidemiology and pathophysiology of spinal cord tumors, address methods of acute oncological management, review treatment for neurological sequelae, and understand the implications as they relate to rehabilitation. METHODS: An extensive literature review was performed regarding the epidemiology, pathophysiology, acute oncological management, neurological sequelae, and rehabilitation for patients with spinal cord tumors. Databases used included pubmed.gov and OVID, as well as individual journal and textbook articles. RESULTS: Access to treatment should be increased given improved survival and functional deficits for patients with spinal cord tumors. Individuals can benefit from inpatient rehabilitation programs, in spite of increased medical co-morbidity and neurological deficits. Specific areas of improvement include functionality, mood, quality of life, and survival. Adjustments to treatment plans must incorporate medical complications from cancer and its treatment, perceived quality of life, and prognosis. CONCLUSIONS: Patients with spinal cord tumors who participate in rehabilitation programs show general improvement in function, mood, quality of life, and survival. Adaptations to care plans should be made to accommodate medical co-morbidities from cancer and its treatment, patient perceptions, and prognosis.

Dumbbell ganglioneuroma mimicking lumbar neurofibroma: a case report and review of the literature.

Spinal ganglioneuromas may grow in a dumbbell fashion. We report the first case of an intradural, extramedullary ganglioneuroma in the lumbar spine and review the pertinent literature. Although rare, we propose that ganglioneuroma be considered a differential diagnosis in patients presenting with a large mass occupying both intraspinal and extraspinal compartments.