Journal of Clinical Neuroscience

Chromosomal anomalies and prognostic markers for intracranial and spinal ependymomas

2012-06-01

Abstract: Ependymomas are neoplasms that can occur anywhere along the craniospinal axis. They are the third most common brain tumor in children, representing 10% of pediatric intracranial tumors, 4% of adult brain tumors, and 15% of all spinal cord tumors. As the heterogeneity of ependymomas has severely limited the prognostic value of the World Health Organization grading system, numerous studies have focused on genetic alterations as a potential basis for classification and prognosis. However, this endeavor has proven difficult due to variations of findings depending on tumor location, tumor grade, and patient age. While many have evaluated chromosomal abnormalities for ependymomas as a whole group, others have concentrated their efforts on specific subsets of populations. Here, we review modern findings of chromosomal analyses, their relationships with various genes, and their prognostic implications for intracranial and spinal cord ependymomas.

Vertebral augmentation in the treatment of vertebral compression fractures: Review and new insights from recent studies

2012-06-01

Abstract: Vertebral compression fractures (VCF) due to osteoporotic degeneration and metastatic disease represent an increasingly significant public health problem. Percutaneous vertebroplasty (VP) began as a simple, low-cost procedure that aimed to provide pain relief for patients with VCF. Balloon kyphoplasty (KP) was introduced later, and was presented not only as a “pain killer,” but also as a deformity correction procedure. The preponderance of evidence has shown that vertebral augmentation provides significant and sustained clinical benefit for patients with VCF. The debate raised by studies published in the New England Journal of Medicine comparing VP with a sham procedure highlights the importance of very careful patient selection for vertebral augmentation procedures, since osteoporotic VCF is usually a self-limiting condition. However, vertebral augmentation may be beneficial in patients with comorbidities that make prolonged bed rest dangerous, in patients with fractures that fail to heal, and in patients with painful VCF due to metastatic disease. Patient selection should be based on a combination of clinical and radiological indications. We review recent studies in the extensive literature on vertebral augmentation, with the goal of clarifying some of the controversy surrounding these procedures.

Anti-stroke prophylaxis – the ethics of collateral damage

Peter Teddy, Girish Nair — 2012-06-01

Abstract: Prophylactic anticoagulation therapy with agents such as dabigatran etexilate (as an alternative to warfarin) promises substantial benefits to both patients and monitoring physicians in the treatment of those at high risk of stroke from atrial fibrillation. Such treatment has, however, one potentially lethal consequence. There is, as yet, no easily administered rapid means of reversal in instances of acute surgical emergency. The problems that may then be encountered in neurosurgical practice are discussed.

Functional outcome prediction following intracerebral hemorrhage

2012-06-01

Abstract: The ICH score is a validated method of assessing the risk of mortality and morbidity after intracerebral hemorrhage (ICH). We sought to compare the ability of the ICH score to predict outcome assessed with three of the most widely used scales: the Barthel Index (BI), modified Rankin Scale (mRS), and Glasgow Outcome Score (GOS). All patients with ICH treated at our institution between February 2009 and March 2011 were followed-up at three months using the mRS, GOS, and BI. The ICH score was highly correlated with the three-month mRS (ρ=0.59, p<0.001), BI (ρ=–0.57, p<0.001) and GOS (ρ=0.61, p<0.001). The ICH score also predicted dependency for each measure well, with areas under the curve falling between 0.826 and 0.833. Our results suggest that future clinical studies that use the ICH score to stratify patients may employ any of the three outcome scales and expect good discrimination of disability.

Intracerebral hemorrhage following intravenous thrombolysis in Thai patients with acute ischemic stroke

2012-06-01

Abstract: In Asia, there is limited information regarding symptomatic intracerebral hemorrhage (ICH) in patients treated with intravenous (iv) recombinant tissue plasminogen activator (rtPA). The aim of this study was to identify independent factors associated with symptomatic ICH following iv rtPA. The study included 192 patients with acute ischemic stroke who were treated with iv rtPA. Baseline characteristics were compared between patients with or without ICH. Symptomatic ICH occurred in 5.7% of patients and asymptomatic ICH in 13.0% of patients. An international normalized ratio (INR) ⩾1.0 (odds ratio [OR]=4.89, p=0.036), atrial fibrillation (OR=7.21, p=0.009) and blood glucose concentration >8.325mmol/L (OR=9.00, p=0.004), were independent risk factors for symptomatic ICH. Atrial fibrillation (OR=3.56, p=0.012) and severe stroke (National Institutes of Health Stroke Scale ⩾15; OR=8.94, p<0.001) were independent risk factors for asymptomatic ICH. The prevalence of symptomatic ICH following iv rtPA in Thai patients was comparable to previous studies.

Plate-only open-door laminoplasty with fusion for treatment of multilevel degenerative cervical disease

Ji-Le Jiang, Xi-Lei Li, Xiao-Gang Zhou, Hong Lin, Jian Dong — 2012-06-01

Abstract: Adequate fixation of the opened laminar arch is the key indicator of a successful laminoplasty surgery; poor outcomes, including secondary narrowing of the spinal canal and a high risk of axial neck pain are possible when using a suturing method during conventional laminoplasty. Rigid fixation including spacers or bone struts yields satisfactory clinical outcomes. However, this approach is also associated with a longer surgical time and an increased risk of instrumentation dislodgement. Plate-only fixation with fusion was developed in our hospital to improve conventional laminoplasty; in addition, the supraspinous ligament is preserved in this procedure. We evaluated both the safety and efficacy of the procedure. Twenty-six patients with multilevel cervical degenerative disease were enrolled for selective open-door laminoplasty with miniplate fixation; autologous bone debris was placed on the hinge side to promote fusion, without bone struts on the open side. The Japanese Orthopedic Association (JOA) score, X-ray, three-dimensional CT scan and MRI were used for the pre- and postoperative evaluations. The mean follow-up period was 22months (range=12–34months), and all patients achieved osseous fusion within six months of the operation. Patients who underwent open-door laminoplasty showed an improvement in the JOA score of 60.7%; a 23.0% incidence of axial neck pain and a 3.2° loss of range of motion (ROM) were also observed. No instrumentation failure or clinical deterioration was observed in our study. Thus, open-door laminoplasty with miniplate fixation is a safe, simple surgery for multilevel cervical disease that has significant clinical efficacy. This approach can maintain the cervical ROM, reduce the incidence of postoperative axial neck pain, decrease surgical time and cost, and avoid complications related to fusion.

Serum D-dimer as a predictor of mortality in patients with acute spontaneous intracerebral hemorrhage

2012-06-01

Abstract: It has been found that the hemostatic system is activated following a brain injury. To explore the role of D-dimer in spontaneous intracerebral hemorrhage (ICH), this prospective study aimed to evaluate the association between serum D-dimer concentration, clinical outcome and radiographic findings of ICH patients in the emergency department (ED). Patients with acute (<24hours) spontaneous ICH were enrolled in this study. The D-dimer concentration was related to: baseline ICH volume (r=0.198, p=0.01); Glasgow Coma Scale (GCS) score 3–8 (p=0.01); GCS score 13–15 (p=0.002); midline shift >15mm (p=0.016); and to subarachnoid extension of the blood (p<0.0001). Diabetes mellitus (odds ratio [OR]: 2.93; 95% confidence interval [CI]: 1.1–7.76, p=0.031), ICH volume (OR: 1.16; 95% CI: 1.07–1.27, p<0.0001) and D-dimer concentration (OR: 2.72; 95% CI: 1.08–6.9, p=0.002) were associated with 30-day mortality. This study shows that in patients with spontaneous ICH, a higher initial D-dimer concentration is associated with higher 30-day mortality.

Prediction of flow augmentation and complications of extracranial–intracranial bypass in symptomatic cerebrovascular diseases

Tsung-Lang Chiu, Sheng-Tzung Tsai, Cheng-Hui Chiu — 2012-06-01

Abstract: Augmentation of the cerebral blood supply to correct cerebral hemodynamic insufficiency by extracranial–intracranial bypass may be an appropriate method to reduce the risk of ischemic stroke. Eighty-five patients with ischemic symptoms, decreased regional cerebral blood flow, and decreased regional cerebrovascular reactivity were recruited for surgery. The post-bypass mean regional blood flow increased by 35.8% compared to the pre-bypass value (p<0.001). Only minor re-establishment of vasculature after anastomosis was detected in three of four patients with middle cerebral artery stenosis, which suggests that there are fewer benefits of bypass surgery in this situation. Cerebral infarction occurred immediately post-operation in one patient who was predisposed to stroke due to a bilateral carotid occlusion. Hyperperfusion injury was infrequent in this series; only one patient developed intracerebral hemorrhage three weeks after the bypass. One ischemic and one hemorrhagic stroke occurred during the 90months following surgery.

Quantitative analysis of neovascular permeability in glioma by dynamic contrast-enhanced MR imaging

Zhongzheng Jia, Daoying Geng, Tianwen Xie, Jiaoyan Zhang, Ying Liu — 2012-06-01

Abstract: This study was designed to quantitatively analyse neovascular permeability in glioma by dynamic contrast-enhanced MRI (DCE–MRI). Forty-four patients with glioma were included in this study. The highest value of volume transfer constant (Ktrans) and volume of extravascular extracellular space per unit volume of tissue (Ve) were obtained and the differences in Ktrans and Ve between low-grade glioma (LGG) and high-grade glioma (HGG) were analyzed. The correlations between Ktrans, Ve and glioma grade were performed. Receiver operating characteristic (ROC) curve analyses were conducted. The values of Ktrans and Ve of LGG were significantly lower than those of HGG. The correlation analysis demonstrated statistically significant relationships between Ktrans and glioma grade, between Ve and glioma grade, and between Ktrans and Ve. The ROC curve analyses of Ktrans (0.035/min) and Ve (0.130) for differentiating LGG from HGG were statistically significant. Thus, DCE–MRI can be used to estimate neovascular permeability and for pre-operative grading of glioma.

A prospective study of Gasserian ganglion pulsed radiofrequency combined with continuous radiofrequency for the treatment of trigeminal neuralgia

2012-06-01

Abstract: We conducted a prospective randomized controlled study to evaluate whether continuous radiofrequency (CRF) combined with pulsed radiofrequency (PRF) to the Gasserian ganglion (GG) decreases the side effects of CRF while preserving efficacy. Sixty patients diagnosed with classic trigeminal neuralgia (TN) were treated with either 75°C CRF for 120s to 180s (SCRF group), 75°C CRF for 240s to 300s (LCRF group), or 42°C PRF for 10minutes (min) followed by 75°C CRF for 120s to 180s (PCRF group). Patients were assessed for pain intensity, quality of life (QOL), and intensity of facial dysesthesia before (baseline), and at seven days, three months, six months, and 12months after the procedure. The efficacy in pain relief was most significant on the seventh day after treatment and there were no significant differences between groups. After 12months, >70% of patients in each group had complete pain relief, and the QOL in all three groups had increased significantly compared to baseline. The intensity of facial dysesthesia was mildest in the SCRF group and most severe in the PCRF group on the seventh day after the procedure, but most persistent in the LCRF group. Patients who receive PRF combined with CRF to the GG can achieve comparable pain relief to those who receive CRF alone, and shorter exposure of CRF could result in less destruction of the target tissue.

Minimally invasive surgery compared to open spinal fusion for the treatment of degenerative lumbar spine pathologies

Ralph J. Mobbs, Praveenan Sivabalan, Jane Li — 2012-06-01

Abstract: This clinical study prospectively compares the results of open surgery to minimally invasive fusion for degenerative lumbar spine pathologies. Eighty-two patients were studied (41 minimally invasive surgery [MIS] spinal fusion, 41 open surgical equivalent) under a single surgeon (R. J. Mobbs). The two groups were compared using the Oswestry Disability Index, the Short Form-12 version 1, the Visual Analogue Scale score, the Patient Satisfaction Index, length of hospital stay, time to mobilise, postoperative medication and complications. The MIS cohort was found to have significantly less postoperative pain, and to have met the expectations of a significantly greater proportion of patients than conventional open surgery. The patients who underwent the MIS approach also had significantly shorter length of stay, time to mobilisation, lower opioid use and total complication rates. In our study MIS provided similar efficacy to the conventional open technique, and proved to be superior with regard to patient satisfaction, length of hospital stay, time to mobilise and complication rates.

A clinicopathological study of the significance of the proportion of choroid morphology in chordoid meningioma

2012-06-01

Abstract: Chordoid meningiomas (CM) account for approximately 0.5% to 1.0% of intracranial meningiomas. This tumor has a strong risk of recurrence and aggressive growth (World Health Organization grade II). Histological analysis of CM tumors shows that the tissue is often dominated by chordoid morphology; however, the exact relationship between the percentage of the chordoid component and other clinicopathological features is unknown. We collected 26 surgical specimens from 17 patients who had a histological diagnosis of CM between January 1986 and June 2010. The chordoid elements constituted 30% to 98% of the area of the tumor. In 12 of 17 (70.6%) primary tumors, over 50% of the area displayed the chordoid pattern. Recurrence was noted in nine of these patients and five underwent a second operation. These five patients showed a histopathological progression of aggressive features. The proportion of chordoid elements in each recurrent tumor also increased. Thus, the chordoid proportion in CM is associated with a greater likelihood of recurrence.

Risk factors for recurrent shunt infections in children

2012-06-01

Abstract: Risk factors for recurrent shunt-related cerebrospinal fluid (CSF) infections were analyzed. A total of 58 children were treated for initial shunt infections (ISI): all children were treated with antibiotics and CSF drainage, either by removal of the shunt system and insertion of an external ventricular drainage (EVD) catheter (44 children, 75.9%) or by externalization of the existing ventricular catheter (14 children, 24.1%). Recurrent shunt infections (RSI) were detected in 15 children: nine had been treated with shunt removal and insertion of a new EVD catheter and six had been treated with externalization of the existing ventricular catheter. There was a statistically significant increase in the number of RSI in children treated with externalization of the existing ventricular catheter. Thus, to reduce the risk of RSI, total shunt removal and insertion of a new EVD catheter is preferred.

Central neurocytoma

Haihui Chen, Rongrong Zhou, Jiayi Liu, Jintian Tang — 2012-06-01

Abstract: Central neurocytomas (CN) are rare intraventricular tumors with prominent neuronal differentiation. CN commonly arise in the lateral ventricles of young adults who predominantly present with raised intracranial pressure. Few studies have described the clinical, pathological, and radiological features of these tumors, and those that have are typically single case reports. Herein, we report ten patients with CN with variable clinical and pathological features and discuss the management of these tumors. Nine tumors occupied the lateral ventricle and only one was located in the sellar region. On MRI, all 10 tumors showed heterogeneous hypo-or iso-intensity on T1-weighted and hyperintensity on T2-weighted MRI. Contrast enhancement varied greatly from very slight to intense. All patients were surgically treated by macroscopic total or subtotal removal. Postoperative radiotherapy was given to six patients (four of whom had undergone subtotal resection and two of whom had undergone total resection). The surgical and histopathological data of these patients were reviewed and analyzed. No recurrences were noted although we were unable to contact two patients for follow-up. A brief review of the literature concerning differential diagnosis and therapeutic aspects of these tumors is also presented.

Incidence, risk factors and management of delayed wound dehiscence after craniotomy for tumor resection

Kaveh Barami, Rui Fernandes — 2012-06-01

Abstract: Dehiscence after a wound has healed is a known complication of craniotomy for tumor resection. We conducted a retrospective analysis of 64 patients who underwent craniotomy for tumor resection followed by radiation or radiosurgery between 2006 and 2010. Five patients (7.8%) were identified who showed wound dehiscence from two to eight months after the craniotomy wound had healed. Four patients had previously undergone additional craniotomies, additional radiosurgery or had been treated with the anti-angiogenic factor, bevacizumab. These treatments may be risk factors for developing delayed dehiscence and, in combination, may potentiate local wound healing problems. Potential mechanisms and management strategies are discussed.

Minimally invasive transforaminal lumbar interbody fusion and spondylolisthesis

Antonio Tsahtsarlis, Martin Wood — 2012-06-01

Abstract: The purpose of this study was to assess the clinical and radiological outcomes of minimally invasive transforaminal lumbar interbody fusion (MI-TLIF) surgery for lumbar spondylolisthesis. A prospective analysis was conducted of 23 consecutive patients with grade I or grade II lumbar spondylolisthesis who underwent a MI-TLIF using image guidance between August 2008 and September 2010. The patient group comprised 13 males and 10 females (mean age 57years), 22 of whom underwent single level fusion and one patient with a two level fusion. All patients underwent postoperative CT scans to assess pedicle screw and cage placement and fusion at six months. The Oswestry Disability Index (ODI) scores were recorded preoperatively and at the six-month follow-up. We found that 22 of 23 (95.7%) patients showed evidence of fusion at six months with a mean improvement of 26.7 on ODI scores. The mean length of hospital stay was four days. The mean operative time was 172minutes. Anatomical reduction of the spondylolisthesis was complete in 16 patients and incomplete in seven. Regarding complications, we observed: one of 94 (1.1%) pedicle screws misplaced, which did not require revision postoperatively; one of 23 patients (4.3%) with a pulmonary embolism and one of 23 (4.3%) patients with transient nerve root pain. There were no occurrences of infection and no postoperative cerebrospinal fluid leaks. We conclude that MI-TLIF offers patients a safe and effective surgical option for lumbar spondylolisthesis treatment. Furthermore, it may offer patients additional advantages in terms of postoperative pain and recovery.

Endoscopic endonasal management of trigeminal schwannomas extending into the infratemporal fossa

Qiuhang Zhang, Kong Feng, Chen Ge, Guo Hongchuan, Li Mingchu — 2012-06-01

Abstract: Extracranial trigeminal schwannomas extending into the infratemporal fossa are rare. The traditional surgical approaches to the infratemporal fossa are associated with complications, such as facial nerve dysfunction, hearing loss, dental malocclusion and cosmetic problems. We report eight patients (four males, four females, age range=31–62years) who were treated between 2004 and 2009 for extracranial trigeminal schwannomas extending into the infratemporal fossa. Schwannomas were surgically removed using a purely endoscopic endonasal approach. The maximum diameters of the tumours ranged from 30mm to 70mm and all tumours were completely removed. There were no intraoperative or postoperative complications in this series. There were no recurrences during the follow-up period which ranged from 10 to 74months (mean=30months). The purely endoscopic endonasal approach may provide a minimally invasive and safe approach to remove extracranial trigeminal schwannomas extending into the infratemporal fossa. Radical resection was associated with an excellent long-term outcome in this series.

Neuroprotective effects of anti-tumor necrosis factor-alpha antibody on apoptosis following subarachnoid hemorrhage in a rat model

2012-06-01

Abstract: Recent studies have emphasized the importance of apoptosis in subarachnoid hemorrhage (SAH) and the subsequent early brain injury. However, the apoptotic pathways induced by SAH in different brain regions are not fully understood. We investigated gene expression levels of classical apoptosis-related molecules (caspase-3, bax, and bcl-2) following SAH in the hippocampus of male Wistar rats. Temporally specific changes were found in caspase-3 and bax messenger RNA only. Interestingly, we found increased expression of bax, but not caspase-3, in the prefrontal cortex, which indicates different molecular mechanisms of apoptosis in distinct brain regions. Most important, changes in expression were reversed by functional blockade of tumor necrosis factor-alpha, which has a critical role in brain injury. In addition, we found that apoptosis induced by SAH may be associated with a relative elevation of pro-brain derived neurotrophic factor.

Progressive mental status changes and seizures with fluid attenuated inversion recovery (FLAIR) hyperintensity on brain MRI

Erick Tarula, Shakti Ramkissoon, Sean Pittock, Volney L. Sheen — 2012-06-01

A 31-year-old male presented to the hospital with disorientation and leg weakness after running. Several weeks prior to admission, he developed visual changes, described as yellow plastic masks of peoples’ faces. Over the previous week, he had complained of headaches, right foot numbness, and intermittent loss of leg control when running. At times, he had developed spontaneous extension of the right leg and inversion of the foot, tremulousness, and weakness lasting several seconds. His neurological exam was notable for impaired mental status with disorientation to date and place. His mood was labile with behavioral outbursts. He had marked abulia and mild anomia. His memory was poor with inability to recall daily and past details. His motor exam was notable for increased tone and clonus in his lower extremities. While in the hospital, he had periods of hypertension and tachycardia. Cerebrospinal fluid analysis revealed a white blood cell count of 14 106 cells/L with lymphocytic predominance, normal protein and glucose concentrations, and negative cytology. A routine electroencephalogram showed intermittent bursts of mixed theta/delta slowing in the left centroparietal region. His brain biopsy showed gliotic brain tissue with marked mixed inflammatory infiltrate consisting of lymphocytes with scattered plasma cells. The MR and functional imaging studies showed decreased metabolism and enhancement within the limbic system, as well as extension into the midline cortical structures. The anatomical abnormalities were consistent with the patient’s motor and cognitive behavioral issues ().

An unusual brainstem lesion

Yaxiong Li, Yuekang Zhang, Jianguo Xu, Ni Chen, Zhongxin Zhao — 2012-06-01

A 9-year-old girl presented with a 2-week history of a sudden onset of bilateral lower extremity weakness. She had difficulty walking for 2weeks. She denied cough, fever, headache, diplopia, or weight loss. There was no past medical history of tuberculosis (TB) for the patient or her family.

A novel adenoviral vector labeled with superparamagnetic iron oxide nanoparticles for real-time tracking of viral delivery

2012-06-01

Abstract: In vivo tracking of gene therapy vectors challenges the investigation and improvement of biodistribution of these agents in the brain, a key feature for their targeting of infiltrative malignant gliomas. The glioma-targeting Ad5/3-cRGD gene therapy vector was covalently bound to super-paramagnetic iron oxide (Fe3O4) nanoparticles (SPION) to monitor its distribution by MRI. Transduction of labeled and unlabeled vectors was assessed on the U87 glioma cell line and normal human astrocytes (NHA), and was higher in U87 compared to NHA, but was similar between labeled and unlabeled virus. An in vivo study was performed by intracranial subcortical injection of labeled-Ad5/3-cRGD particles into a pig brain. The labeled vector appeared in vivo as a T2-weighted hyperintensity and a T2-gradient echo signal at the injection site, persisting up to 72 hours post-injection. We describe a glioma-targeting vector that is labeled with SPION, thereby allowing for MRI detection with no change in transduction capability.

A novel murine model of human renal cell carcinoma spinal metastasis

2012-06-01

Abstract: There is currently no reproducible animal model of renal cell carcinoma (RCC) spinal metastasis that allows for laboratory study of the human disease. In this report, we describe an animal model that reliably reproduces RCC spinal metastasis using a human tumor cell line. A posterior surgical approach was used to implant tumor cells into the lamina of immunosuppressed mice. Histology sections were analyzed 12weeks after tumor cell implantation to quantify the location and extent of tumor growth. RCC xenografts grew in treated animals (8 mice) with a reproducible pattern of growth. After implantation, tumor growth occurred primarily in the antero-posterior dimension. At 8weeks after tumor cell implantation, there was visible tumor growth in all treated mice. Histologic correlation at 12weeks after tumor cell implantation confirmed tumor growth involving primarily the paraspinal region and lamina. Our investigation resulted in an orthotopic model of human RCC spinal metastasis. Ultimately this will allow testing of targeted therapies for RCC with spinal involvement. Furthermore, this model can be expanded to develop similar spinal metastasis models for other tumor cell lines.

Glioblastoma multiforme and cerebral cavernous malformations: intersection of pathophysiologic pathways

2012-06-01

Abstract: Cerebral cavernous malformations (CCM) are known to occur in both sporadic and familial forms. To date, there has been no identified association of CCM with glioblastoma multiforme. We present a 69-year-old woman with a 14year history of multiple CCM who developed progressive aphasia. She had no radiation exposure and had only undergone a single computed tomography scan in her entire life. MRI demonstrated irregular gadolinium enhancement in the area of a prior stable CCM, suspicious for a high grade tumor. Stereotactic biopsy revealed a glioblastoma multiforme. This is a unique case of glioblastoma multiforme arising from the “site” of a known CCM. We review the literature on the genetics of cavernous malformations and propose a mechanism for the tumorigenic potential of these vascular malformations.

Thyroid stimulating hormone microadenoma as a rare cause of thyrotoxicosis amenable to surgical cure

2012-06-01

Abstract: Hyperthyroidism due to a thyroid stimulating hormone (TSH) pituitary adenoma is rare. We report a 29-year-old woman with thyrotoxicosis and elevated serum 3,5,3′,5′-tetraiodothyronine and TSH levels that resolved after a transsphenoidal excision of the detected TSH pituitary adenoma. The diagnosis and management options in such patients are reviewed.

Electrophysiological characteristics in four patients from Brazil with stiff person syndrome

2012-06-01

Abstract: Stiff person syndrome (SPS) is a rare immune-mediated disorder of the central nervous system characterized by muscle rigidity and episodic muscle spasms. The diagnosis of SPS is based on electrophysiological studies. We analyzed the electrophysiological features in four patients from Brazil who fulfilled the clinical criteria for SPS. The most common electrophysiological abnormalities were continuous motor unit activity, co-contracting, and the presence of the cutaneomuscular reflex. Despite all patients having clinical characteristics of SPS during the disease, no patient met all the electrophysiological criteria for SPS even after repeat electrophysiological studies. This shows that a diagnosis of SPS should not be restricted to patients with all the classic electrophysiological changes but should be considered in the presence of one or some of those changes.

Application of indocyanine green videoangiography in surgery for spinal vascular malformations

Basant K. Misra, Harshad R. Purandare — 2012-06-01

Abstract: We present our recent experience with indocyanine green videoangiography (ICGVA) in intra-operative evaluation of two patients with dorsal spinal dural arteriovenous fistula (SDAVF) and one patient with conus medullaris arteriovenous malformation (AVM). To our knowledge, the latter is the first report of this in the literature. Intra-operative ICGVA was used to identify an early filling vessel and to obliterate the site of fistulous connection. This was confirmed by a repeat ICGVA study and correlated with post-operative digital subtraction angiography (DSA). The abnormal fistulous site was identified in all three patients and disconnected. Complete obliteration was confirmed in all patients using ICGVA and with post-operative imaging. There was no untoward reaction to the dye injection. We conclude that ICGVA is a useful adjunct in surgical treatment of spinal vascular malformations since it is a real-time, non-invasive, radiation-free technique with good image resolution, and is repeatable and easily reproducible. Technical disadvantages can be minimized by proper exposure of the operative field.

Anti-NMDA receptor encephalitis with the initial presentation of psychotic mania

Yen Lin Kuo, Hsing Fang Tsai, Ming Chi Lai, Chien Ho Lin, Yen Kuang Yang — 2012-06-01

Abstract: We report a 16-year-old girl with suspected psychotic mania, who subsequently developed amnesia, catatonia, oro-lingual dyskinesia, consciousness disturbance, seizure and respiratory failure. Repeated studies of the cerebrospinal fluid (CSF), viral culture and serology, brain MRI, single photon emission CT scan, and autoimmune profiles were all normal. She was finally diagnosed with anti-N-methyl D-aspartate receptor (NMDAR) encephalitis based on the positive finding of NMDAR antibodies in CSF. Her abdominal CT scan showed no detectable malignancy and pulse steroid therapy failed to have any effect. After administration of intravenous immunoglobulin her consciousness improved gradually. Anti-NMDAR encephalitis, with a characteristic neuropsychiatric syndrome, predominantly affects females with an ovarian tumor and is frequently misdiagnosed as a psychiatric disorder. Immunotherapy and eradication of associated malignancy are the main treatment strategies. Early recognition and early intervention of the disease should improve the outcome.

A primary orbital hydatid cyst

Hakan Somay, Selin Tural Emon, Metin Orakdogen, Mehmet Zafer Berkman — 2012-06-01

Abstract: Echinococcosis, caused by the tapeworm Echinococcus granulosus, is an endemic zoonosis in which humans act as accidental intermediate hosts. Orbital hydatid cysts comprise 1% to 2% of all hydatid lesions. We describe a 10-year-old boy with an orbital hydatid cyst. The orbital cyst was removed totally by frontal craniotomy and orbitotomy. It should be noted that unilateral painless proptosis in patients from countries endemic for echinococcosis could be caused by an orbital hydatid cyst.

Delayed cerebrospinal fluid rhinorrhea after gamma knife surgery in a patient with a growth hormone-secreting adenoma

Yan Hongmei, Wang Zhe, Wang Jing, Wang Daokui, Cao Peicheng, Li Yongjie — 2012-06-01

Abstract: We report a patient who developed delayed cerebrospinal fluid (CSF) rhinorrhea 11years after gamma knife radiosurgery for a growth hormone (GH)-secreting adenoma. The treatment dose was 18Gy for the tumor margin (50% isodose). One year later, an MRI of the head revealed that the tumor size had decreased. Eleven years later, the patient developed CSF rhinorrhea from the left nostril. Subsequent MRI examination revealed complete remission of the tumor in the sella turcica and the empty sella. The patient was admitted for direct endoscopic surgical repair of the skull base. We suggest that the cause of the CSF rhinorrhea is secondary empty sella. The other potential causes may be the original invasiveness of the tumor or delayed radiation damage to the mucous membranes of the skull. Long-term follow-up is required to monitor recurrence of CSF rhinorrhea.

Reversible antecollis associated with pramipexole in a patient with Parkinson’s disease

Hee Jin Kim, Beom S. Jeon, Sook Hui Kim, Seol-Heui Han — 2012-06-01

Abstract: Antecollis is a frequent complication in multiple system atrophy but is rare in Parkinson’s disease (PD). We report an 80-year-old patient with a four-year history of PD who developed antecollis six weeks after taking pramipexole (1mg/day). When assessed in the outpatient clinic, she had antecollis, cogwheel rigidity on the right side, and mild bradykinesia. We found no evidence of myopathic or neurogenic changes in the neck muscles on needle electromyography. We withdrew the pramipexole immediately, and, one week later, her antecollis improved dramatically. This report emphasizes the importance of considering dopamine agonists as a possible cause of antecollis and shows that immediate withdrawal of these drugs may reverse the symptoms.

Acute, monocular vision loss from Rathke’s cleft cyst

Bradley N. Bohnstedt, Neal B. Patel, Matthew C. Hagen, Daniel H. Fulkerson — 2012-06-01

Abstract: Rathke’s cleft cysts are benign, non-neoplastic sellar lesions that are often asymptomatic. The rare patient with symptoms may present with pituitary dysfunction, headache, or visual problems. Visual deficits are generally consistent with a chiasmal syndrome as associated with other sellar lesions. Therefore, the most commonly described deficit is bitemporal hemianopsia. We describe a 16-year-old female patient who presented with rare, rapidly progressing monocular blindness without signs of apoplexy or hemorrhage. She had complete return of vision after surgical decompression. The rapid loss and recovery of vision suggest that ischemic factors, rather than deformation of the optic apparatus, contributed to her symptoms. The relevant literature is reviewed.

Progression and management of superficial siderosis

E. Lekgabe, B. Kavar — 2012-06-01

Abstract: This report illustrates the progression and surgical management of a patient with superficial siderosis associated with a cervical pseudomeningocoele.

Subacute combined degeneration of the spinal cord despite prophylactic vitamin B12 treatment

Benjamin K.-T. Tsang, Nicholas Crump, Richard A. Macdonell — 2012-06-01

Abstract: We describe a 35year-old man presenting with a four-week history of non-painful limb paraesthesias and unsteady gait causing falls. On examination he had an ataxic gait associated with dorsal column sensory loss. He had a medical history of a partial gastrectomy six years prior and anaemia. He had received monthly intramuscular hydroxycobalamin injections since the gastrectomy. Laboratory tests revealed normal vitamin B12 and holotranscobalamin levels, a reduced serum caeruloplasmin of 0.05g/L (normal: 0.22–0.58g/L), a copper-to-caeruloplasmin ratio of 1.9μmol/L (11.0–22.0μmol/L) and a reduced 24-hour urinary copper concentration of <0.30μmol/L (0–0.3μmol/L). Cerebrospinal fluid analysis, nerve conduction studies, electromyography and visual-evoked responses were unremarkable. MRI revealed abnormal hyperintense signal in the cervical dorsal columns. Hypocupric myelopathy was diagnosed and he was treated with daily oral elemental copper. Three months later, his walking and balance had improved although there was no change noted on MRI.

Open ring enhancement in atypical brain demyelination

Vijayakumar Javalkar, Marc Manix, Jon Wilson, Anil Nanda — 2012-06-01

Abstract: Open ring enhancement is considered highly specific for atypical demyelination. In this report we present a patient with a history of headache, ataxia and sensory disturbances in the lower extremities. A cranial MRI scan showed a large frontal lesion with mass effect, midline shift and with open ring enhancement. These findings are characteristics of tumefactive multiple sclerosis. Such lesions can be confused with neoplasms and abscesses. Open ring enhancement may help in differentiating atypical demyelination from a neoplasm or an abscess.

Wingspan stenting of symptomatic middle cerebral artery stenosis and perioperative evaluation using high-resolution 3 Tesla MRI

MingChao Shi, ShouChun Wang, HongWei Zhou, YanHua Cheng, JiaChun Feng, Jiang Wu — 2012-06-01

Abstract: High-resolution MRI (HR MRI) was employed to study intracranial arterial walls in a patient who underwent angioplasty and stenting for middle cerebral artery (MCA) stenosis using the Wingspan stent (Boston Scientific, Natick, MA, USA). HR MRI clearly depicted the wall structure of the MCA. As a complementary method, HR MRI may improve intracranial atherosclerotic stenosis diagnosis and therapy.

Intraparenchymal haemorrhage after a cerebrospinal fluid tap-test for secondary normal pressure hydrocephalus

2012-06-01

Abstract: The lumbar tap test as a diagnostic and therapeutic procedure in idiopathic normal pressure hydrocephalus is used widely. Complications from lumbar punctures are rare. We report a man who underwent a tap-test for secondary normal pressure hydrocephalus, and after clinical improvement, suffered a fatal intraparenchymal brain haemorrhage three days later.

Delayed-onset focal dystonia of the leg secondary to traumatic brain injury

S. Kemp, S.D.H. Kim, D.J. Cordato, V.S.C. Fung — 2012-06-01

Abstract: A 42-year-old man presented with action-induced, stereotyped posturing of the left leg with task specificity following major traumatic brain injury less than a year earlier. Although adult onset primary leg dystonia is a recognised entity, our patient is unusual in that dystonia was an isolated abnormality without associated spasticity, was not preceded by ipsilateral hemiparesis and remained focal without progression to involve other body regions. MRI brain showed a small area of gliosis in the left frontal subcortical white matter but with no lesions in the basal ganglia or thalamus. The dystonia in our patient almost completely resolved with botulinum toxin therapy.

AchR-positive myasthenia gravis with MRI evidence of early muscle atrophy

Vasiliki Zouvelou, Michael Rentzos, Panagiotis Toulas, Ioannis Evdokimidis — 2012-06-01

Abstract: Muscle atrophy, when it occurs in myasthenia gravis (MG), is usually associated with long-standing disease or with chronic corticosteroid treatment. Early muscle atrophy in a steroid-naive patient has been reported previously only in muscle-specific tyrosine kinase (MuSK)-MG. We report a 63-year-old male patient with acetylcholine receptor (AchR)-positive MG with a short duration of disease, no steroid treatment and MRI evidence of muscle atrophy.

Gamma-aminobutyric acid (GABA)-B receptor 1 in cerebellar cortex of essential tremor

C. Luo, A.H. Rajput, C.A. Robinson, A. Rajput — 2012-06-01

Abstract: Some reports suggest cerebellar dysfunction as the basis of essential tremor (ET). Several drugs with the action of gamma-aminobutyric acid (GABA) are known to improve ET. Autopsy studies were performed on brains from nine former patients followed at the Movement Disorders Clinic Saskatchewan, Canada, and compared with five normal control brains. We aimed to measure the concentration of GABA B receptor 1 (GBR1) in the brains of patients who had had ET and to compare them to the GABA concentration in brains of controls. Western blot was used to determine the expression of GBR1 in cerebellar cortex tissue. We found that compared to the controls, the ET brains had three different patterns of GBR1 protein concentration – two with high, four comparable, and three with marginally low levels. There was no association between the age of onset, severity or duration of tremor, the response to alcohol or other drugs and GBR1 level. Thus, we conclude that our study does not support that GBR1 is involved in ET. Further studies are needed to verify these results.

Pisa syndrome in a patient with progressive supranuclear palsy

2012-06-01

Pisa syndrome (PS) is an abnormal posture characterized by a tonic flexion of the trunk to one side accompanied by a slight rotation in the sagittal plane, originally described as the consequence of acute axial dystonia related to neuroleptic administration. Subsequently PS has been reported in patients treated with antiemetics, antidepressants, cholinesterase inhibitors and dopamine agonists. A similar picture may also occur as an idiopathic phenomenon and in neurodegenerative diseases, including parkinsonism. With regard to parkinsonism, although PS has been previously indicated as a red flag for multiple system atrophy (MSA), this abnormal posture is not infrequently observed in patients with typical Parkinson’s disease (PD) and other atypical parkinsonism disorders such as dementia with Lewy bodies (DLB). However, PS has not yet been described in patients with progressive supranuclear palsy (PSP). We describe a patient affected by PSP who developed a dystonic lateral flexion of the trunk compatible with the diagnosis of PS.

Progressive mental status changes and seizures with fluid attenuated inversion recovery (FLAIR) hyperintensity on brain MRI

Erick Tarula, Shakti Ramkissoon, Sean Pittock, Volney L. Sheen — 2012-06-01

B. N-methyl-D-aspartic acid (NMDA) receptor autoimmune encephalitis. Both serum and cerebrospinal fluid from this patient contained immunoglobulin (Ig)G that bound to central nervous system (CNS) tissue in a pattern consistent with NMDA receptor specific-IgG (; immunofluorescence titer [expressed as the reciprocal of doubling serum dilutions] was 3840). NMDA receptor specificity was confirmed using NMDA receptor transfected and non-transfected cells (, Euroimmun slides [Munich, Germany]). Radiographically, brain MRI in this disorder is usually unremarkable but focal enhancement or medial temporal lobe abnormalities have been reported recently. Both hypometabolism and hypermetabolism have been reported on 18F-fluorodeoxyglucose–positron emission tomography. The patient stabilized after treatment with high dose steroids followed by a 5-day course of intravenous immunoglobulin. He returned 5 weeks later for increased confusion and a seizure. Repeat testing for NMDA receptor antibodies was negative at that time by indirect immunofluorescence (titer <120), although positivity persisted on transfected cells (Euroimmun). A decision was made to treat with once weeklyinfusions of rituximab overa 5-week period. Over the ensuing 2 months, the patient made a dramatic recovery and returned back to his baseline.

An unusual brainstem lesion

Yaxiong Li, Yuekang Zhang, Jianguo Xu, Ni Chen, Zhongxin Zhao — 2012-06-01

C. Brainstem tuberculoma. The diagnosis was confirmed by a stereotactic biopsy of the brainstem lesion. On histological examination, a tuberculoma was identified associated with giant cells and caseous necrosis (A), and acid-fast staining was positive (B).

Erratum to: The pathological characteristics of glioma stem cell niches [19 (1) 121–127]

Hu He, Ming Wu Li, Chao Shi Niu — 2012-06-01

In the above article, In Table 2, “24” should be “22”.