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Young-Seop Park, Seung-Jae Hyun, Ho Yong Choi, Ki-Jeong Kim and Tae-Ahn Jahng

OBJECTIVE

The aim of this study was to investigate the risk of upper instrumented vertebra (UIV) fractures associated with UIV screw fixation (unicortical vs bicortical) and polymethylmethacrylate (PMMA) augmentation after adult spinal deformity surgery.

METHODS

A single-center, single-surgeon consecutive series of adult patients who underwent lumbar fusion for ≥ 4 levels (that is, the lower instrumented vertebra at the sacrum or pelvis and the UIV of the thoracolumbar spine [T9–L2]) were retrospectively reviewed. Age, sex, follow-up duration, sagittal UIV angle immediately postoperatively including several balance-related parameters (lumbar lordosis [LL], pelvic incidence, and sagittal vertical axis), bone mineral density, UIV screw fixation type, UIV PMMA augmentation, and UIV fracture were evaluated. Patients were divided into 3 groups: Group U, 15 patients with unicortical screw fixation at the UIV; Group P, 16 with bicortical screw fixation and PMMA augmentation at the UIV; and Group B, 21 with bicortical screw fixation without PMMA augmentation at the UIV.

RESULTS

The mean number of levels fused was 6.5 ± 2.5, 7.5 ± 2.5, and 6.5 ± 2.5; the median age was 50 ± 29, 72 ± 6, and 59 ± 24 years; and the mean follow-up was 31.5 ± 23.5, 13 ± 6, and 24 ± 17.5 months in Groups U, P, and B, respectively (p > 0.05). There were no significant differences in balance-related parameters (LL, sagittal vertical axis, pelvic incidence–LL, and so on) among the groups. UIV fracture rates in Groups U (0%), P (31.3%), and B (42.9%) increased in sequence by group (p = 0.006). UIV bicortical screw fixation increased the risk for UIV fracture (OR 5.39; p = 0.02).

CONCLUSIONS

Bicortical screw fixation at the UIV is a major risk factor for early UIV compression fracture, regardless of whether a thoracolumbosacral orthosis is used. To reduce the proximal junctional failure, unicortical screw fixation at the UIV is essential in adult spinal deformity correction surgery.

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Jin Woo Chang, Jae Young Choi, Young Sul Yoon, Yong Gou Park and Sang Sup Chung

✓ The purpose of this paper was to present two cases of secondary trigeminal neuralgia (TN) with an unusual origin and lesion location. In two cases TN was caused by lesions along the course of the trigeminal nerve within the pons and adjacent to the fourth ventricle. Both cases presented with typical TN. Brain magnetic resonance imaging revealed linear or wedge-shaped lesions adjacent to the fourth ventricle, extending anterolaterally and lying along the pathway of the intraaxial trigeminal fibers.

The involvement of the nucleus of the spinal trigeminal tract and of the principal sensory trigeminal nucleus with segmental demyelination are suggested as possible causes for trigeminal pain in these cases. It is postulated that these lesions are the result of an old viral neuritis. The patients underwent gamma knife radiosurgery and their clinical responses have been encouraging to date.

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Keun Young Park, Jung Yong Ahn, Jae Whan Lee, Jong Hee Chang and Seung Kon Huh

Vascular complications, including vessel occlusion and hemorrhage, can arise after radiosurgery; however, hemorrhage due to a ruptured de novo aneurysm after Gamma Knife radiosurgery (GKS) for tumor is extremely rare. To the authors' knowledge, only a single case of de novo aneurysm formation after GKS for vestibular schwannoma has been previously reported. In this study, they describe their experience with the treatment of a 74-year-old woman with subarachnoid hemorrhage limited to the cerebellopontine cistern, who had undergone GKS for vestibular schwannoma 5 years earlier. Cerebral angiography demonstrated a left distal anterior inferior cerebellar artery aneurysm; coil embolization was attempted and failed. However, self-resolution of the aneurysm was revealed on follow-up angiography.

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Dong-Kyu Jang, Kwan-Sung Lee, Hyoung Kyun Rha, Pil-Woo Huh, Ji-Ho Yang, Ik Seong Park, Jae-Geun Ahn, Jae Hoon Sung and Young-Min Han

OBJECTIVE

In this study the authors evaluated whether extracranial-intracranial bypass surgery can prevent stroke occurrence and decrease mortality in adult patients with symptomatic moyamoya disease (MMD).

METHODS

The medical records of 249 consecutive adult patients with symptomatic MMD that was confirmed by digital subtraction angiography between 2002 and 2011 at 8 institutions were retrospectively reviewed. The study outcomes of stroke recurrence as a primary event and death during the 6-year follow-up and perioperative complications within 30 days as secondary events were compared between the bypass and medical treatment groups.

RESULTS

The bypass group comprised 158 (63.5%) patients, and the medical treatment group comprised 91 (36.5%) patients. For 249 adult patients with MMD, bypass surgery showed an HR of 0.48 (95% CI 0.27–0.86, p = 0.014) for stroke recurrence calculated by Cox regression analysis. However, for the 153 patients with ischemic MMD, the HR of bypass surgery for stroke recurrence was 1.07 (95% CI 0.43–2.66, p = 0.887). For the 96 patients with hemorrhagic MMD, the multivariable adjusted HR of bypass surgery for stroke recurrence was 0.18 (95% CI 0.06–0.49, p = 0.001). For the treatment modality, indirect bypass and direct bypass (or combined bypass) did not show any significant difference for stroke recurrence, perioperative stroke, or mortality (log rank; p = 0.524, p = 0.828, and p = 0.616, respectively).

CONCLUSIONS

During the treatment of symptomatic MMD in adults, bypass surgery reduces stroke recurrence for the hemorrhagic type, but it does not do so for the ischemic type. The best choice of bypass methods in adult patients with MMD is uncertain. In adult ischemic MMD, a prospective randomized study to evaluate the effectiveness and safety of bypass surgery to prevent recurrent stroke is necessary.

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Eun Jung Lee, Jeong Hoon Kim, Eun Suk Park, Young-Hoon Kim, Jae Koo Lee, Seok Ho Hong, Young Hyun Cho and Chang Jin Kim

OBJECTIVE

Advances in neuroimaging techniques have led to the increased detection of asymptomatic intracranial meningiomas (IMs). Despite several studies on the natural history of IMs, a comprehensive evaluation method for estimating the growth potential of these tumors, based on the relative weight of each risk factor, has not been developed. The aim of this study was to develop a weighted scoring system that estimates the risk of rapid tumor growth to aid treatment decision making.

METHODS

The authors performed a retrospective analysis of 232 patients with presumed IM who had been prospectively followed up in the absence of treatment from 1997 to 2013. Tumor volume was measured by imaging at each follow-up visit, and the growth rate was determined by regression analysis. Predictors of rapid tumor growth (defined as ≥ 2 cm3/year) were identified using a logistic regression model; each factor was awarded a score based on its own coefficient value. The probability (P) of rapid tumor growth was estimated using the following formula:

FD1

RESULTS

Fifty-nine tumors (25.4%) showed rapid growth. Tumor size (OR per cm3 1.07, p = 0.000), absence of calcification (OR 3.87, p = 0.004), peritumoral edema (OR 2.74, p = 0.025), and hyperintense or isointense signal on T2-weighted MRI (OR 3.76, p = 0.049) were predictors of tumor growth rate. In the Asan Intracranial Meningioma Scoring System (AIMSS), tumor size was categorized into 3 groups of < 2.5 cm, ≥ 2.5 to < 4.0 cm, and ≥ 4.0 cm in diameter and awarded a score of 0, 3, and 6, respectively; the parameters of calcification and peritumoral edema were categorized into 2 groups based on their presence or absence and given a score of 0 or 2 and 1 or 0, respectively; and the signal on T2-weighted MRI was categorized into 2 groups of hypointense and hyperintense/isointense and given a score of 0 or 2, respectively. The risk of rapid tumor growth was estimated to be < 10% when the total score was 0–2, 10%–50% when the total score was 3–6, and ≥ 50% when the total score was 7–11 (Hosmer-Lemeshow goodness-of-fit test, p = 0.9958). The area under the receiver operating characteristic curve was 0.86.

CONCLUSIONS

The authors suggest a weighted scoring system (AIMSS) that predicts the specific probability of rapid tumor growth for patients with untreated IM. This scoring system will aid treatment decision making in clinical settings by screening out patients at high risk for rapid tumor growth.

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Jae Meen Lee, Hye Ran Park, Young Doo Choi, Sung Min Kim, Beomseok Jeon, Han-Joon Kim, Dong Gyu Kim and Sun Ha Paek

OBJECTIVE

The authors investigated the incidence, clinical course, and predisposing factors associated with delayed facial palsy (DFP) following microvascular decompression (MVD).

METHODS

The authors reviewed the records of 310 patients (311 cases) who were followed after MVD for hemifacial spasm (HFS). Of these patients, 45 (14.5%) developed DFP after MVD. The clinical characteristics and predisposing factors of the patients with HFS were investigated to identify prognostic factors that predicted the development of DFP after MVD. Log-rank tests were used to compare times to symptom disappearance, and a logistic regression analysis was performed to compare clinical characteristics between patients who developed DFP and those who did not.

RESULTS

HFS was completely resolved immediately after MVD in 158 cases (50.8%), and HFS eventually disappeared in 289 (92.9%) of the cases. Of the 45 patients with DFP, 17 were men and 28 were women. DFP occurred between postoperative Days 1 and 44 (mean 9.67 days). Finally, 44 patients (97.8%) completely recovered. The average time to recovery was 3.9 months (range 1–24 months). Patients who had experienced an immediate disappearance of HFS experienced a significantly higher occurrence of DFP than those who did not (odds ratio 0.383, 95% confidence interval 0.183–0.802; p = 0.011). In addition, preoperative botulinum neurotoxin injections negatively influenced the occurrence of DFP (p = 0.016).

CONCLUSIONS

In this study, the incidence rate of DFP was slightly higher than previously reported values. Moreover, DFP can occur even when spasms disappear immediately after MVD, but the patients with DFP can fully recover within weeks.

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Eun-Hee Kim, Mi-Sun Yum, Young-Shin Ra, Jun Bum Park, Jae Sung Ahn, Gu-Hwan Kim, Hyun Woo Goo, Tae-Sung Ko and Han-Wook Yoo

OBJECT

Moyamoya disease (MMD) is an idiopathic cerebrovascular occlusive disorder prevalent in East Asia. In the pathogenesis of MMD, the important role of genetic factors is being elucidated, and RNF213 has recently been identified as a susceptibility gene for MMD. The aim of this retrospective study was to investigate the RNF213 genotype in patients with MMD and to determine their genotype-phenotype associations.

METHODS

The study involved 165 Korean MMD patients from 155 unrelated families who were diagnosed with MMD at a single center from 1995 to 2013. Their demographic, radiological, and clinical findings were evaluated. Direct sequencing of the major RNF213 single nucleotide polymorphisms was performed. The association of the common RNF213 variant with MMD risk was evaluated using historical controls for comparison. Correlations between RNF213 genotype and phenotype were statistically analyzed.

RESULTS

The c.14429G>A (p.R4810K) variant was identified in 125 (75.8%) of 165 MMD patients. Most patients (112) were heterozygous, and 13 patients had 2 copies of the c.14429G>A variant. A novel heterozygous variant, c.12086A>G (p.Q4029R), was found in 1 additional patient. The minor allele frequency of the c.14429G>A variant was significantly higher in the MMD group (138 [41.8%] of 330 patients) than in the control group (8 [1.36%] of 588 subjects; p < 0.001). The c.14429G>A (p.R4810K) variant significantly increased the risk of MMD in Korean patients, with an OR of 52.11 (p < 0.001) compared with controls. Moreover, c.14429G>A (p.R4810K) genotypes occurred more frequently in patients with a family history of MMD. The homozygous variant was highly associated with early-onset MMD (age at onset < 5 years), cerebral infarction at diagnosis, and cognitive impairment in long-term outcome.

CONCLUSIONS

The findings indicate that the c.14429G>A (p.R4810K) allele of RNF213 is strongly associated with Korean patients with MMD. The homozygous c.14429G>A (p.R4810K) variant is particularly related to early-onset MMD, severe symptomatic manifestations at diagnosis, and poor prognosis. This genotypic variant may be a useful biomarker for early-onset MMD or unstable MMD with cerebral infarction, which requires early diagnosis and revascularization treatment.

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Bilateral vidian nerve schwannomas associated with facial palsy

Case report and review of the literature

Jin Hwan Cheong, Jae Min Kim, Koang Hum Bak, Choong Hyun Kim, Young Ha Oh and Dong Woo Park

✓ Intracranial schwannomas are relatively common benign tumors arising from Schwann cells. Among the cranial nerves, the vestibular division of the vestibulocochlear nerve is the site most commonly affected by these lesions, followed by the trigeminal nerve. The authors report a case of bilateral schwannomas arising from both of the pterygoid canals. A 13-year-old girl presented with intermittent headaches and left-sided facial palsy. Preoperative computerized tomography scans and magnetic resonance images revealed nonenhancing round masses within the bilateral vidian canals, bone erosion, and sclerosis. The transnasal transseptal transsphenoidal approach was used to remove the masses. Postoperatively, the patient recovered uneventfully. On histopathological examination, the masses were confirmed as schwannomas. The clinical presentation and probable histogenesis of schwannomas arising in this location are discussed together with a review of the literature.

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Yun-Sik Dho, Young Jae Kim, Kwang Gi Kim, Sung Hwan Hwang, Kyung Hyun Kim, Jin Wook Kim, Yong Hwy Kim, Seung Hong Choi and Chul-Kee Park

OBJECTIVE

The aim of this study was to analyze the positional effect of MRI on the accuracy of neuronavigational localization for posterior fossa (PF) lesions when the operation is performed with the patient in the prone position.

METHODS

Ten patients with PF tumors requiring surgery in the prone position were prospectively enrolled in the study. All patients underwent preoperative navigational MRI in both the supine and prone positions in a single session. Using simultaneous intraoperative registration of the supine and prone navigational MR images, the authors investigated the images’ accuracy, spatial deformity, and source of errors for PF lesions. Accuracy was determined in terms of differences in the ability of the supine and prone MR images to localize 64 test points in the PF by using a neuronavigation system. Spatial deformities were analyzed and visualized by in-house–developed software with a 3D reconstruction function and spatial calculation of the MRI data. To identify the source of differences, the authors investigated the accuracy of fiducial point localization in the supine and prone MR images after taking the surface anatomy and age factors into consideration.

RESULTS

Neuronavigational localization performed using prone MRI was more accurate for PF lesions than routine supine MRI prior to prone position surgery. Prone MRI more accurately localized 93.8% of the tested PF areas than supine MRI. The spatial deformities in the neuronavigation system calculated using the supine MRI tended to move in the posterior-superior direction from the actual anatomical landmarks. The average distance of the spatial differences between the prone and supine MR images was 6.3 mm. The spatial difference had a tendency to increase close to the midline. An older age (> 60 years) and fiducial markers adjacent to the cervical muscles were considered to contribute significantly to the source of differences in the positional effect of neuronavigation (p < 0.001 and p = 0.01, respectively).

CONCLUSIONS

This study demonstrated the superior accuracy of neuronavigational localization with prone-position MRI during prone-position surgery for PF lesions. The authors recommend that the scan position of the neuronavigational MRI be matched with the surgical position for more precise localization.