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Objective: We performed a retrospective observational study to demonstrate the surgical risks and long-term prognoses of intramedullary tumors in Japan using a multicenter registry authorized by the Neurospinal Society of Japan.Methods: Data from 1,033 consecutive patients with intramedullary tumors, treated between 2009 and 2020, were collected from 58 centers. Patients with spinal lipomas or myxopapillary ependymomas were excluded. Patient characteristics, clinical presentations, imaging characteristics, treatments, and outcomes were analyzed. The modified McCormick scale was used to classify functional status. Survival was described using Kaplan-Meier curves, and multivariable logistic regression analyses were performed.Results: The mean age of the patients was 48.4 years. Data of 361 ependymomas, 196 hemangioblastomas, 168 astrocytic tumors, 160 cavernous malformations, and the remaining 126 cases including subependymomas, metastases, schwannomas, capillary hemangiomas, and intravascular B-cell lymphomas were analyzed. Twenty-two patients were undiagnosed. The mean follow-up duration was 46.1 ± 38.5 months. Gross total tumor removal was achieved in 672 tumors (65.1%). On the modified McCormick scale, 234 patients (22.7%) had worse postoperative grades at the time of discharge. However, neurological status gradually improved. At 6 months postoperatively, 251 (27.5%), 500 (54.9%), and 160 patients (17.6%) had improved, unchanged, and worsened grades, respectively. Preoperative functional status, gross total tumor removal, and histopathological type were significantly associated with mortality and functional outcomes.Conclusion: Our findings demonstrate better postoperative functional outcomes in patients with fewer preoperative neurological deficits. Degree of resection, postoperative treatments, and prognoses are closely related to the histology of intramedullary tumors.

Background: The redundant nerve root (RNR) syndrome is a pathological condition in which the cauda equina develops into a severely flexed/tortuous spiral mass above a level of severe lumbar stenosis. Case Description: A 70-year-old male presented with bilateral neurogenic claudication attributed to a MRI-documented intradural extramedullary lesion at the L1 level with severe adjacent level/inferior L2/3 stenosis. At surgery, intradural exploration at L1 revealed an edematous cauda equina consistent with the diagnosis of the RNR syndrome. Conclusion: The RNR syndrome should be included among the differential diagnostic considerations when non-enhancing lesions are encountered above levels of marked lumbar stenosis.

Objective When treating patients with cervical spondylotic myelopathy (CSM), we often note amelioration in concomitant hypertension after surgery. To assess the effects of surgery and the mechanisms thereof, blood pressure (BP) and parasympathetic nervous activity were monitored prospectively in CSM patients undergoing surgery. Methods Sixty-eight consecutive CSM patients who underwent surgery with myoarchitectonic spinolaminoplasty were enrolled. BP and electrocardiography were recorded preoperatively and at 1, 3, and 6 months postoperatively. Forty-six patients completed the scheduled follow-ups and were analyzed. Preoperatively, 17 had a mean BP higher than 100 mmHg (the HT group) and 12 had hypertension despite taking medication (the HT-refractory group). To evaluate alterations in parasympathetic function, the coefficient of variation of the RR interval (CVRR) was evaluated. Results A significant BP reduction was observed in the HT group 6 months after surgery, but not in the normotensive group (n=29). The effect was more remarkable in the HT-refractory group. A transient BP increase at 1 and 3 months after surgery was observed in all groups. Comparisons were made between groups classified by age (over 65 years or younger than 60 years) and the presence or absence of an intramedullary hyperintense T2 signal on magnetic resonance imaging, but no significant differences were detected. Measurements of CVRR did not significantly differ between the groups over the course of follow-up. Conclusion Hypertension coexisting with CSM can be ameliorated after surgical treatment. The effect is likely to be mediated by moderation of sympathetic activity, rather than parasympathetic activation. We believe that a combination of adequate decompression of the spinal cord and relief from musculoskeletal stresses effectuate this moderation.

Study design Basic animal research. Objective The effects of granulocyte colony-stimulating factor (G-CSF) were assessed in a rat chronic spinal cord compression model to explore the potential of G-CSF as a pharmacological treatment for cervical spondylotic myelopathy. Summary of background data G-CSF is a hematopoietic cytokine used clinically to treat neutropenia. Recently, neuroprotective effects of G-CSF have been reported in spinal cord disorders. Methods To introduce the chronic cervical cord compression, thin polyurethane sheets were implanted under C5-C6 laminae of rats and gradually expanded by absorbing water. This model reproduces delayed compressive myelopathy of the cervical spine. In sham operations, the sheets were immediately removed. G-CSF (15 μg/kg) or normal saline (NS) was administered subcutaneously 5 days a week. Experimental groups were sham operation given NS; cord compression given NS; and cord compression given G-CSF. To assess motor functions, rotarod performance, and grip strength were measured. Twenty-six weeks after surgery, cervical spinal cords were examined histopathologically. In the prevention experiment, G-CSF or NS administration was started immediately after surgery. In the treatment experiment, their administration was started 8 weeks after surgery. In another experiment, in three groups in the prevention experiment, terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate-biotin nick end labeling staining was performed to assess apoptotic cell death at 8 weeks after surgery. Results In the prevention experiment, administration of G-CSF preserved the motor functions and motor neurons throughout the 26 weeks, and significantly decreased the number of apoptotic cells at 8 weeks. In the treatment experiment, G-CSF administration from 8 weeks after surgery markedly restored the motor function temporarily to a level equal to the sham group. Conclusion G-CSF prevents the decline in motor functions and preserves motor neurons in the rat chronic cord compression model. G-CSF also improves motor function in the progressive phase of compression myelopathy. Level of evidence N/A.

Background Motor evoked potential (MEP) recording is used as a method to monitor integrity of the motor system during surgery for intramedullary tumors (IMTs). Reliable sensitivity of the monitoring in predicting functional deterioration has been reported. However, we observed false positives and false negatives in our experience of 250 surgeries of IMTs. Objective To delineate specificity and sensitivity of MEP monitoring and to elucidate its limitations and usefulness. Methods From 2008 to 2011, 58 patients underwent 62 surgeries for IMTs. MEP monitoring was performed in 59 operations using transcranial electrical stimulation. Correlation with changes in muscle strength and locomotion was analyzed. A group undergoing clipping for unruptured aneurysms was compared for elicitation of MEP. Results Of 212 muscles monitored in the 59 operations, MEP was recorded in 150 (71%). Positive MEP warnings, defined as amplitude decrease below 20% of the initial level, occurred in 37 muscles, but 22 of these (59%) did not have postoperative weakness (false positive). Positive predictive value was limited to 0.41. Of 113 muscles with no MEP warnings, 8 muscles developed postoperative weakness (false negative, 7%). Negative predictive value was 0.93. MEP responses were not elicited in 58 muscles (27%). By contrast, during clipping for unruptured aneurysms, MEP was recorded in 216 of 222 muscles (96%). Conclusion MEP monitoring has a limitation in predicting postoperative weakness in surgery for IMTs. False-positive and false-negative indices were abundant, with sensitivity and specificity of 0.65 and 0.83 in predicting postoperative weakness.

Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural crest-derived cells is considered to be related to pathogenesis in both conditions. However, association of NCM and PPV has not been reported to the best of our knowledge. Melanocytoma, which usually involves the leptomeninges or spinal cord, is extremely rare in the retroperitoneum. We present here a case of a patient with NCM, PPV, and melanocytic tumors in the spinal cord and retroperitoneum, which were treated surgically. A 40-year-old woman had a 2-year history of dysesthesia and weakness in the left leg. History included congenital giant blue nevus-like lesion in the trunk, a port-wine stain in the sacral area, and Caesarean section performed 8 years before, when diffuse pigmentation in the peritoneum was noted. Magnetic resonance (MR) imaging of the spine revealed an intramedullary tumor at T10 level with paramagnetic signal characteristics. The spinal cord tumor was totally removed, and the histological diagnosis was melanocytoma. Three months later, a left retroperitoneal mass with histological features of melanocytic tumor was removed. Neither tumors recurred and the patient stays ambulatory 4 years after the surgery. Multiple subtypes of melanocytic tumors with distinctive features of NCM and PPV can develop simultaneously, mimicking malignant melanoma. Gross total resection of each tumor, when indicated, is beneficial.

Study design Sham-operation-controlled animal study to assess alterations in blood flow in the spinal cord in a chronic compression model. Laboratory investigation. Objective Cervical myelopathy is a common cause of disability in elderly patients. Hypothesis was made that ischemia subsequent to the spinal cord compression plays an important role in the pathogenesis of the spinal cord dysfunction. This study was undertaken to assess alterations in the blood flow of the spinal cord under chronic compression in a rat model. Summary of background data Histologic study of spinal cord from patients with spondylotic myelopathy showed ischemic tissue changes. Experimentally, spinal cord hypoperfusion in combination with chronic spinal cord compression induced myelopathy in dogs. We previously showed that chronic compression of the spinal cord in rats produces gradual deterioration of mobility of the animals accompanied by cord tissue degeneration compatible with ischemic changes. Methods Chronic compression of the cervical spinal cord was implemented by implantation of a thin urethane polymer sheet under the C5-C6 laminae, which expands by absorbing tissue water over 48-72 hours. The control group underwent sham operation. Twelve weeks later, blood flow to the C3-C4 and C5-C6 spinal cord segments were measured by fluorescent microsphere methods. Results In the control group, the blood flow in the C5-C6 segment was larger than C3-C4 segment. In the compression group, the blood flow in the C5-C6 was significantly reduced compared to the C3-C4 segment. Conclusion Under chronic focal spinal cord compression, there was a decrease of the blood flow in the compressed segment in comparison to the rostral segment. Our data are compatible with the hypothesis that alteration in the spinal cord blood flow contributes to pathogenesis of myelopathy.

Spine surgery has made radical advancements in the last two decades and provision has expanded a great deal. The history of the technical development is briefly reviewed. To analyze trends in utilization and to assess the macroeconomic demand for spine surgery, the incidence of all spine surgery per capita is estimated referring to diverse statistical data from the USA, Korea and Japan. When compared internationally, there is a great disparity in the utilization of spine surgery, especially for fusion/instrumentation. Medico-socioeconomic conditions underlying the variations are discussed. Adequate surgeon training has to be supplied in a matched volume, and the number of surgeons to balance the need is estimated.

Cervical laminoplasty was developed as an alternative to cervical laminectomy for treatment of cervical myelopathy, in which hinges are created to lift the lamina. Various techniques of laminoplasty have since been developed after two prototype techniques: Hirabayashi's open-door laminoplasty and Kurokawa's spinous process splitting (double-door) laminoplasty. Several in vitro studies report superior biomechanical stability of the cervical spine after laminoplasty compared with laminectomy. In clinical situation, randomized control studies are scarce and superiority of one procedure over another is not uniformly shown. Lack of hard evidence supporting the purported advantages of laminoplasty over laminectomy, that is, reduced rate of postoperative instability and kyphosis development, while preserving range of motion (ROM), has been a weak selling point. Currently, laminoplasty is performed by majority of spine surgeons in Japan, but is rarely performed in the United States and Europe. Recent development in laminoplasty is preservation of muscle attachment, which enabled dynamic stabilization of the cervical spine by neck extensor muscles. After treatment with new laminoplasty techniques with active postoperative neck ROM exercises, postoperative instability, kyphosis, axial neck pain, and loss of ROM seems minimal. Well-designed clinical trials to show the effectiveness and long-term outcome of this surgical procedure are warranted.

A 40-year-old woman presented with a right petroclival meningioma compressing the brainstem and manifesting as a 6-month history of headache and gait difficulty. The patient underwent subtotal removal of the tumor via an anterior transpetrosal approach. The postoperative course was complicated by cerebrospinal fluid rhinorrhea, bacterial meningitis, and acute hemorrhagic rectal ulcer. The patient was discharged home in good condition after prolonged medical treatment. Four months after the surgery, the patient noted recurrence of gait difficulty. Magnetic resonance (MR) imaging of the brain showed enlargement of the ventricles and no residual brainstem compression. A ventriculoperitoneal shunt was placed, but the symptoms were unchanged. The shunt was removed 2 months later because of infection. The patient's gait gradually deteriorated, although repeat brain MR imaging showed no significant increase in ventricular size. Ten months after the initial surgery she became paraplegic. MR imaging of the thoracic spine revealed a large arachnoid cyst extending from C-6 to T-6. The patient underwent T2-4 laminectomy, partial removal of the cyst wall, and duraplasty, but no clinical improvement was observed. Preexisting long-tract signs and coincidental hydrocephalus confused the neurological findings and delayed detection of the spinal lesion in this case. Neurosurgeons should be alert to the possibilities of insidious spinal lesion if the patient has progressive neurological disorder which does not match the known cranial lesion.

Background Temporal bone chondroblastoma is a rare primary bone tumor that affects the floor of the middle cranial fossa. This tumor is known to have high recurrence rate after curettage, and wide resection is therefore recommended. However, the literature provides little information regarding long-term results after wide resection of temporal bone chondroblastoma. Methods Four cases of surgically treated temporal bone chondroblastoma underwent long-term follow-up. Results Four patients, 3 males and 1 female, with mean age of 34, were surgically treated at the neurosurgery department of Keio University Hospital. Two patients were treated for recurrent tumor and the other two for new disease. In all cases the tumor mainly involved the mandibular fossa with variable degree of infiltration into tympanic and petrous parts. The tumor was totally removed via zygomatic approach in all patients. In 3 patients, the mandibular condyle was removed to expose the tumor. These patients had temporary malocclusion and restricted motion postoperatively, which resolved within 3 to 12 months with conservative treatment. All patients have no recurrence to date with a mean follow-up period of 9 years. Conclusion Temporal bone chondroblastoma was removed totally with skull base surgical technique and no recurrence has occurred for 6 to 13 years postoperatively. We found that removal of the mandibular head does not cause permanent problems of mastication in patients with normal dentures.

OBJECTIVE AND IMPORTANCE Spinal accessory schwannomas unassociated with neurofibromatosis are very rare, and only 30 cases have been reported in the literature. To our knowledge, this is the first report of a spinal accessory schwannoma mimicking a tumor of the fourth ventricle. CLINICAL PRESENTATION A 50-year-old man presented with neck pain after being involved in a motor vehicle accident. There were no neurological deficits, but a computed tomographic scan revealed a large hypodense mass with punctuate calcifications in the fourth ventricle. The tumor exhibited low intensity on the T1-weighted magnetic resonance imaging scan and high intensity on the T2-weighted scan, and it showed inhomogeneous contrast enhancement. INTERVENTION The tumor was totally removed by a bilateral suboccipital craniectomy and C1 laminectomy. Dissection of the surgical specimen revealed that the tumor had originated from the left spinal accessory nerve. Histopathological examination confirmed the diagnosis of schwannoma. The patient experienced transient postoperative cerebellar ataxia but recovered completely. CONCLUSION Intracisternal-type spinal accessory schwannomas sometimes mimic a tumor of the fourth ventricle. Total surgical resection can be achieved with good outcome.

Scedosporium apiospermum, the asexual anamorph of Pseudallescheria boydii, is a ubiquitous saprophytic fungus that usually causes cutaneous/subcutaneous infection but may manifest as an invasive disease, often in immunocompromised hosts. Following an extensive literature review, we think that this case represents the first documented report of a primary infection of the spine in an immunocompetent patient. Despite extensive surgical debridement and itraconazole therapy, the patient died of multisystem organ failure of unknown etiology. Our case and three previously reported cases of P. boydii vertebral osteomyelitis highlight the importance of obtaining repeat cultures in patients with culture-negative vertebral osteomyelitis who fail to adequately respond to empiric standard antibacterial and/or antimycobacterial therapy. Combined surgical debridement and antifungal therapy have been required for eradication of P. boydii spinal infections in two previously reported immunocompromised patients, although the optimal antifungal regimen for this infection has not been established.

Two patients with giant intracavernous internal carotid artery (ICA) aneurysms were intolerant to balloon test occlusion of the ICA, and later developed spontaneous thrombosis of the aneurysm and the parent ICA without ischemic sequelae. Case 1: A 60-year-old female with a giant right intracavernous ICA aneurysm presented with right abducens nerve paresis. An unsuccessful extracranial-to-intracranial bypass graft operation was complicated by transient postoperative ophthalmoplegia. The patient did not tolerate balloon test occlusion of the right ICA after attempted bypass surgery, and was treated conservatively. The patient presented with acute onset of headache 3 years later. Case 2: A 50-year-old female with a giant right intracavernous ICA aneurysm presented with right abducens nerve paresis. The patient was managed conservatively after a positive balloon test occlusion of the right ICA. The patient suffered transient hypopituitarism and acute onset of headache 2 years later. Spontaneous thrombosis of the aneurysms and occlusion of the parent ICA were found in both patients. Neither had major hemispheric infarcts, but the first patient had asymptomatic infarcts, which were presumed to be thromboembolic in nature. Patients with intracavernous ICA aneurysms who have positive balloon test occlusions appear to develop tolerance to spontaneous and gradual occlusion of the ICA without significant sequelae. However, these patients have an increased risk of developing embolic infarctions. The role for anticoagulation and repeat hemodynamic tests remains unclear.

Drill-induced heat has been suspected as a cause of tissue injury, and there are ample experimental data to substantiate the implication. However, no clinical results have been presented with measurement of temperature in the vicinity of neural structures during the actual spinal procedures. Using a thermocouple, temperature in the gutters drilled in the midline and the lateral margins of the lamina was monitored closely in 61 patients, who underwent the French-door style of cervical spino-laminoplasty. The drilling was performed intermittently for a duration of 5 or 10 seconds, using 3- or 5-mm diamond burrs with sufficient continuous cooling irrigation. The correlations between bone temperature elevation and postoperative sensorimotor symptoms were then analyzed. In the lateral gutters at the most cephalad level (typically C3), where the drilling was performed underneath an overlying bundle of muscle attached to C2's spinous process, the temperature rose significantly. This occurred even with 5-second drilling sessions. The average peak temperature was 44 °C at this level. At all other sites, the temperature was maintained below 40 °C. In three patients, transient neurological deficit developed postoperatively, which did not correlate with the incidence of bone temperature elevation. Intermittent drilling with sufficient irrigation can prevent thermal neuronal damage generated by high-speed drills. Drilling with small diamond burrs in deep and narrow spaces covered by overlying muscles predisposes to inadequate irrigation and thermal elevation. Continuous, protracted drilling without frequent irrigation may result in excessive heat generation and nerve injury.

Fluorescence angiography with indocyanine green (ICG) provides real-time information regarding the patency of vessels. To enhance the capability to delineate flow direction, flow velocity and sequence of dye filling in different components of complex spinal vascular lesions such as perimedullary arteriovenous fistulas (AVFs), we tried selective intraarterial injection of ICG with catheterization in the proximity of the AVFs.Prior to taking the position for surgery, a metallic introducer sheath was placed into the femoral artery, and placed over the patient's lateral buttock. After the exposure of the AVFs, a standard angiographic catheter was advanced into the proximal portion of the feeding artery and a small volume of diluted ICG (0.06 mg in 5 ml saline for one examination) injected repeatedly. To avoid the thromboembolism, heparinized saline was perfused continuously thorough the catheter and sheath.The small injection volume and the close proximity of the injection site to the lesions resulted in quick rise and fall of the fluorescence without any background. Time and spatial resolution of analysis were enhanced; flow dynamics such as direction, velocity and alteration after temporary occlusion were well visualized. The feeders and drainers were clearly distinguished, and the shunts could be precisely identified.Selective intraarterial injection ICG fluorescence angiography was very useful for perimedullary AVFs. Albeit that it requires intraoperative selective catheterization, this repeatable technique has an advantage to improve temporary resolution and provides accurate information of the flow dynamics through the complex anatomy of vascular lesions.

Study Design. Basic animal research. Objective. Cervical spondylotic myelopathy is a common condition among elderly and often treated by surgery. To explore possibility of pharmacologic treatment, limaprost alfadex, a prostaglandin E1 derivative with vasodilatory and antiplatelet action, was tried in a rat chronic spinal cord compression model. Summary of Background Data. Limaprost increased the blood flow of cauda equina and improved motor functions in animal models of lumbar stenosis. The drug is clinically used to treat neurogenic intermittent claudication. Methods: Forty-two rats were allocated to four groups: (A) sham operation without permanent cord compression, given 5 mL/kg of distilled water twice a day (n = 6); (B) sham operation, receiving 300 μg/kg limaprost twice a day (n = 6); (C) cord compression, receiving the vehicle (n = 15); and (D) cord compression receiving the drug (n = 15). A thin polyurethane sheet that expands by absorbing water was implanted under the C5―C6 laminae to produce cord compression. For sham operation, the sheet was immediately removed. Exercise tests were repeated on a rotating treadmill until 26 weeks after surgery, and then the animals were killed and the spinal cord harvested for motor neurons counts. Results. Treadmill endurance (seconds, mean ± standard error of mean) 2 weeks after surgery was 497.7 ± 2.3, 434.5 ± 65.5, 423.1 ± 33.0, and 480.5 ± 19.5 in groups A, B, C, and D, respectively. At 26th week, the duration was 497.7 ± 2.3, 421.2 ± 78.8, 21.3 ± 11.7, and 441.3 ± 40.4 (P < 0.0001 for the decrease in C group, multivariate analysis of variance with correction for multiple measures.) The motor neuron counts were 38.3 ± 3.6, 38.2 ± 2.6, 32.6 ± 1.9, and 36.2 ± 2.3 in groups A, B, C, and D (P = 0.34), respectively. Conclusion. Limaprost alfadex prevented decline of forced locomotion capability in rats with chronic compression of the cervical cord.

Background In this prospective 18-month study, 29 patients underwent posterior thoracic instrumentation with placement of 209 transpedicular screws guided by intraoperative fluoroscopic imaging and anatomic landmarks. We assessed the safety, accuracy, complications, and early stability of this technique. Methods Pedicle and pedicle-rib units were measured, and screw cortical penetrations were graded on anatomy and depth of penetration. All 29 patients underwent preoperative computed tomographic (CT) imaging, and 28 underwent postoperative CT imaging (199/209 screws). Results From T2 to T12, screw diameters were >or=5 mm with mean medial screw angulation measuring 20-25 degree. Of the 209 screws placed from T1 to T12, 111 had diameters greater than or equal to the pedicle width. From T3 to T9, the mean diameter of the pedicle screws exceeded the mean pedicle width. Lateral pedicle wall penetration occurred significantly more often than superior, inferior, and medial pedicle wall penetrations and anterolateral vertebral body penetration. Five of six high-risk screw penetrations occurred in one patient when intraoperative technique was compromised. We observed no new postoperative neurologic deficits, visceral injuries, or pedicle screw instrumentation failures. The three high-risk anterolateral vertebral body penetrations at T1 and T2 were associated with a significantly decreased mean screw transverse angle; the three high-risk medial pedicle wall penetrations occurring from T3 to T9 were associated with a significantly increased mean screw transverse angle. Among all 26 patients available at postoperative follow-up (mean 11.9 months), the mean loss of kyphosis correction was 2.0 degree. Conclusions Guided by intraoperative fluoroscopic imaging and anatomic landmarks, thoracic pedicle screws can be placed safely. Early clinical follow-up reveals excellent results with minimal loss of kyphosis correction.

An 81-year-old female presented with severe headache. Computed tomography revealed subarachnoid hemorrhage. She developed right facial nerve paresis on the next day. Angiography revealed a right vertebral artery-posterior inferior cerebellar artery aneurysm. The aneurysm was successfully occluded with interlocking detachable coils (IDCs) on the 7th day. Magnetic resonance (MR) imaging 1 month after IDC placement showed partially thrombosed aneurysm near the internal acoustic meatus. Ten months after the ictus, MR imaging revealed marked resolution of the intra-aneurysmal thrombus and reduction of the aneurysm size. Her facial nerve function gradually recovered during this period. Her facial nerve paresis was probably caused by acute stretching of the facial nerve by the ruptured aneurysm that was in direct contact with the nerve. Intra-aneurysmal thrombosis using coils can reduce aneurysm size and alleviate cranial nerve symptoms.