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Chihiro Ohye, Tohru Shibazaki, Jie Zhang and Yoshitaka Andou

Object. The treatment of Parkinson disease and other kinds of involuntary movement by gamma knife radiosurgery (GKS) is presented. This is an extension of previous work. The clinical course and thalamic lesions were the main factors examined.

Methods. Seventeen new cases were added to the previously reported 36 cases. The course and results for the whole series of 53 patients were examined. Treatment was undertaken using a single 4-mm collimator shot to deliver 130 Gy to the target. The target was determined in the previously treated patients by using classic methods involved in conventional stereotactic thalamotomy with microrecording. More recently, target localization has been performed by relating the target point to the total length of the thalamus. Points may then be defined as percentages of that length measured from the anterior pole. Targets can then be determined in relationship to the appropriate percentage.

Thirty-five patients have been followed for more than 2 years and the longest follow up was 8 years. Two kinds of thalamic lesion were seen after GKS. Volumetric analysis on MR imaging revealed that the larger lesion was 400 to 500 mm3 at the beginning and gradually decreased in size. The smaller lesion occupied approximately 200 mm3 and also shrank over several months. Eighty percent of the treated cases showed good results and no significant complications, with the tremor subsiding at 1 year (Type 1). Several cases deviated from this standard course in four different ways (Types 2–5). If tremor persisted, conventional stereotactic thalamotomy with microrecording was performed. During such operations, normal neuronal activity was recorded from the region adjacent to the GKS thalamotomy target. This was the region showing a high signal on MR imaging. The activity patterns included the rhythmical grouped discharge of tremor rhythm.

Conclusions. Gamma thalamotomy for functional disorders is still under development, but because the results with careful target planning are satisfactory, there are grounds for increasing optimism.

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Chihiro Ohye, Tohru Shibazaki, Junji Ishihara and Jie Zhang

Object. The effects of gamma thalamotomy for parkinsonian and other kinds of tremor were evaluated.

Methods. Thirty-six thalamotomies were performed in 31 patients by using a 4-mm collimator. The maximum dose was 150 Gy in the initial six cases, which was reduced to 130 Gy thereafter. The longest follow-up period was 6 years. The target was determined on T2-weighted and proton magnetic resonance (MR) images. The point chosen was in the lateral-most part of the thalamic ventralis intermedius nucleus. This is in keeping with open thalamotomy as practiced at the authors' institution. In 15 cases, gamma thalamotomy was the first surgical procedure. In other cases, previous therapeutic or vascular lesions were visible to facilitate targeting.

Two types of tissue reaction were onserved on MR imaging: a simple oval shape and a complex irregular shape. Neither of these changes affected the clinical course. In the majority of cases, the tremor subsided after a latent interval of approximately 1 year after irradiation. The earliest response was demonstrated at 3 months. In five cases the tremor remained. In four of these cases, a second radiation session was administered. One of these four patients as well as another patient with an unsatisfactory result underwent open thalamotomy with microrecording. In both cases, depth recording adjacent to the necrotic area revealed normal neuronal activity, including the rhythmic discharge of tremor. Minor coagulation was performed and resulted in immediate and complete arrest of the remaining tremor.

Conclusions. Gamma thalamotomy for Parkinson's disease seems to be an alternative useful method in selected cases.

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Ren-Jie Zhang, Hui-Min Li, Hai Gao, Chong-Yu Jia, Tao Xing, Fu-Long Dong and Cai-Liang Shen

OBJECTIVE

Traditional trajectory (TT) screws are widely used in lumbar fixation. However, they may require revision surgery in some instances, especially in patients with osteoporotic spines. Cortical bone trajectory (CBT) screws may potentially be used to rescue a failed TT screw and vice versa in nonosteoporotic spines. This study aimed to investigate whether a CBT screw can salvage a compromised TT screw in osteoporotic lumbar spines and vice versa.

METHODS

A total of 42 vertebrae from 17 cadaveric lumbar spines were obtained. Bone mineral density was measured, and a CBT screw was randomly inserted into one side of each vertebra. A TT screw was then inserted into the contralateral side. The biomechanical properties of the screws were tested to determine their insertional torque, pullout strength, and fatigue performance. After checking the screws for the failure of each specimen, the failed screw track was salvaged with a screw of the opposite trajectory. The specimen was then subjected to the same mechanical tests, and results were recorded. A repeat pullout test on TT and CBT screws was also performed.

RESULTS

When CBT screws were used to rescue failed TT screws, the original torque increased by 50%, an average of 81% of the pullout strength of the initial TT screws was retained, and the fatigue performance was equal to that of the original screws, which were considerably stronger than the loose TT screws—that is, the TT repeat screws/TT screws were 33% of the pullout strength of the initial TT screws. When the TT screws were used to salvage the compromised CBT screws, the TT screws retained 51% of the original torque and 54% of the original pullout strength, and these screws were still stronger than the loose CBT screws—that is, the loose CBT screws retained 12% pullout strength of the initial CBT screws. Fatigue performance and the ratio of the pullout strength considerably decreased between the CBT rescue screws and the original CBT screws but slightly changed between the TT rescue screws and the original TT screws.

CONCLUSIONS

CBT and TT screws can be applied in a revision technique to salvage each other in osteoporotic lumbar spines. Additionally, CBT and TT screws each retain adequate insertional torque, pullout strength, and fatigue performance when used for revision in osteoporotic lumbar spines.

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Yaxiong Li, Fengshi Fan, Jianguo Xu, Jie An and Weining Zhang

Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.

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Xinru Xiao, Zhen Wu, Liwei Zhang, Guijun Jia, Jie Tang, Guolu Meng and Junting Zhang

Object

In this paper the authors describe a modified far-lateral transcondylar approach to remove hypoglossal neurilemmomas (HGNs).

Methods

Between September 2008 and June 2011, 11 consecutive patients with HGNs underwent tumor removal via a modified far-lateral transcondylar approach. The average age at presentation, tumor characteristics, cranial nerve (CN) deficits, and outcomes were assessed. The modified far-lateral transcondylar approach comprises several important steps. The first step is to remove the limited posterior aspect of the condylar facet to open the hypoglossal canal. The second step is to expose the posterior arch and the transverse process of C-1. A fat layer covers the venous plexus of the vertebral artery, and careful dissection along this surface of the fat layer is important to protect the vertebral artery from damage. The neck muscles are dissected caudally to expose the extracranial component of the tumor, which is located in front of the transverse process of C-1.

Results

Eleven cases of HGNs were treated during the study period. The mean patient age was 47.4 ± 8.9 years (range 31–59 years); there were 3 men and 8 women. The mean follow-up period was 14.1 ± 9 months. All patients presented with hypoglossal nerve deficits; other commonly observed deficits included glossopharyngeal and vestibular/cochlear nerve deficits. Gross-total resection of the tumor was achieved in 10 patients. A subtotal resection of the tumor was achieved in the remaining patient. Two patients had transient postoperative facial nerve palsies, 1 patient developed a new CN XI palsy postoperatively, and 5 patients experienced transient hoarseness and difficulty swallowing. Two patients required a tracheotomy because they demonstrated dysfunction of the caudal CNs and subsequently developed postoperative pneumonia. Postoperatively, 5 patients required the temporary placement of a nasogastric feeding tube. There were no surgery-related deaths in this series.

Conclusions

The modified far-lateral transcondylar approach is an effective treatment for HGNs, yielding a high total tumor removal rate with an acceptable rate of morbidity.

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Shuyu Hao, Christopher S. Hong, Jie Feng, Chunzhang Yang, Prashant Chittiboina, Junting Zhang and Zhengping Zhuang

Maffucci syndrome is a rare disease characterized by multiple enchondromas and soft-tissue hemangiomas. Additionally, neuroendocrine tumors including pituitary adenomas have been described in these patients. The underlying genetic etiology lies in somatic mosaicism of mutations in isocitrate dehydrogenase 1 (IDH1) or isocitrate dehydrogenase 2 (IDH2). This report describes a patient with Maffucci syndrome who presented with intracranial tumors of the skull base and suprasellar region. The patient underwent resection of both intracranial tumors, revealing histopathological diagnoses of chondrosarcoma and pituitary adenoma. DNA sequencing of the tumors was performed to identify common IDH1/2 mutations. Clinical, radiological, and biochemical assessments were performed. Genotypic studies used standard Sanger sequencing in conjunction with a target-specific peptide nucleic acid to detect IDH1 mutations in tumor tissues. DNA sequencing demonstrated identical IDH1 mutations (c.394C > T) in both tumors.

To the authors’ knowledge, this report provides the first genetic evidence for the inclusion of pituitary adenomas among tumors characterizing Maffucci syndrome. In patients who are newly diagnosed with Maffucci syndrome, it is appropriate to monitor for development of pituitary pathology and neuroendocrine dysfunction.

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Chun-de Li, Shi-qi Luo, Jian Gong, Zhen-yu Ma, Ge Jia, Yu-qi Zhang and Jie-fei Li

Hypothalamic hamartoma (HH) is a rare condition that often manifests as central precocious puberty (CPP). There is a lack of information available concerning the long-term effectiveness of surgery for the treatment of CPP due to HH. Here the authors describe 3 cases of CPP due to HH, with a follow-up ranging from 9 to 11 years after surgery. Three girls experienced breast growth and menses at 5–18 months of age and 5–36 months of age, respectively. Serum concentrations of luteinizing hormone, follicle-stimulating hormone, and estradiol concentrations ranged from 2.5 to 6.5 mIU/ml, 4.8–5.9 mIU/ml, and 47.9–133.0 pg/ml, respectively. Magnetic resonance imaging confirmed that CPP was caused by HH. Lesions were resected using a right pterional approach. After surgery, endocrine hormone concentrations were normalized, breasts shrunk, and menses ceased in each patient. Moreover, all of them subsequently developed normally and experienced age-appropriate onset of puberty. Each patient's height and weight were normal at the most recent follow-up (9–11 years after surgery), and none had experienced learning difficulties. Central precocious puberty due to HH can be successfully treated with resection. In the 3 cases presented, this approach was associated with both short- and long-term efficacy.

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Jie Zhang, Dong-Xiao Zhuang, Cheng-Jun Yao, Ching-Po Lin, Tian-Liang Wang, Zhi-Yong Qin and Jin-Song Wu

OBJECT

The extent of resection is one of the most essential factors that influence the outcomes of glioma resection. However, conventional structural imaging has failed to accurately delineate glioma margins because of tumor cell infiltration. Three-dimensional proton MR spectroscopy (1H-MRS) can provide metabolic information and has been used in preoperative tumor differentiation, grading, and radiotherapy planning. Resection based on glioma metabolism information may provide for a more extensive resection and yield better outcomes for glioma patients. In this study, the authors attempt to integrate 3D 1H-MRS into neuronavigation and assess the feasibility and validity of metabolically based glioma resection.

METHODS

Choline (Cho)–N-acetylaspartate (NAA) index (CNI) maps were calculated and integrated into neuronavigation. The CNI thresholds were quantitatively analyzed and compared with structural MRI studies. Glioma resections were performed under 3D 1H-MRS guidance. Volumetric analyses were performed for metabolic and structural images from a low-grade glioma (LGG) group and high-grade glioma (HGG) group. Magnetic resonance imaging and neurological assessments were performed immediately after surgery and 1 year after tumor resection.

RESULTS

Fifteen eligible patients with primary cerebral gliomas were included in this study. Three-dimensional 1H-MRS maps were successfully coregistered with structural images and integrated into navigational system. Volumetric analyses showed that the differences between the metabolic volumes with different CNI thresholds were statistically significant (p < 0.05). For the LGG group, the differences between the structural and the metabolic volumes with CNI thresholds of 0.5 and 1.5 were statistically significant (p = 0.0005 and 0.0129, respectively). For the HGG group, the differences between the structural and metabolic volumes with CNI thresholds of 0.5 and 1.0 were statistically significant (p = 0.0027 and 0.0497, respectively). All patients showed no tumor progression at the 1-year follow-up.

CONCLUSIONS

This study integrated 3D MRS maps and intraoperative navigation for glioma margin delineation. Optimum CNI thresholds were applied for both LGGs and HGGs to achieve resection. The results indicated that 3D 1H-MRS can be integrated with structural imaging to provide better outcomes for glioma resection.

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Ying Guo, J. Lynn Palmer, Loren Shen, Guddi Kaur, Jie Willey, Tao Zhang, Eduardo Bruera, Jean-Paul Wolinsky and Ziya L. Gokaslan

Object

Total or partial sacrectomy is a rare procedure in patients with locally invasive tumors involving the sacrum; it may be associated with functional loss, such as bowel and bladder dysfunction and gait abnormality. In this study the authors examined functional outcome following sacrectomy.

Methods

The authors reviewed the charts of 50 consecutive patients who had undergone sacrectomy between July 1993 and August 2002. There were 23 male and 27 female patients whose mean age was 46 years (range 13–86 years). Twelve patients with rectal cancer underwent a separate analysis. The patients without rectal cancer were divided into two groups: those who had undergone colostomy for bowel diversion (Group 1, six cases), and those who had not (Group 2, 32 cases).

In Group 1 patients the median hospital length of stay (LOS) was 48.5 days (the 25th% and 75th percentiles are 26 and 58, respectively), and in Group 2 patients the median LOS was 18.5 days (the 25th and 75th percentiles are 8 and 41, respectively; p = 0.14). In Group 2 (non—rectal cancer without colostomy), LOS was greater in patients in whom a myocutaneous flap was used compared with those in whom no flap was used (36 days compared with 8.5 days, respectively; p = 0.0012); in patients with bowel incontinence the median LOS was significantly longer than that in patients with bowel continence (39 days compared with 8 days, respectively; p = 0.0026). The incidence of bowel incontinence in Group 2 was closely related to the integrity of the S-3 nerve root (p = 0.05).

Conclusions

Awareness of the association between S-3 nerve root resection and bowel and bladder incontinence may help surgeons' decision-making process.