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1.  Clinical Features and Treatment Outcome of Chordoid Meningiomas in a Single Institute 
Meningioma is the second most common primary central nervous system neoplasm. In contrast, chordoid meningioma is rare; due to the paucity of cases, little is known about its clinical features or treatment outcomes. The objectives of this study were to describe the clinical characteristics and outcomes for patients with chordoid meningioma.
In total, 16 patients, with newly diagnosed chordoid meningioma who underwent surgical excision between 1999 and 2012 were included. We retrospectively evaluated the medical records, radiological findings, and pathological findings. The median follow-up period was 56.5 (range, 3-170) months. The MIB-1 labeling index ranged from 1 to 26.60% (median, 5.04).
Simpson grade I, II, and III resections were performed in four, nine, and three patients, respectively. The overall recurrence rate was 37.5%. Overall progression-free survival (PFS) after resection was 94.7 months (95% CI=62.9-126.6). Of the 4 patients with Simpson grade I resection, recurrence occurred in one patient. Among the Simpson grade II and III resection groups, eight patients underwent adjuvant radiation therapy and they showed significantly longer PFS (121 months, 95% CI=82.1-159.9) than the patients who underwent surgery alone (40.5 months, 95% CI=9.6-71.3) by the log-rank test (p<0.05).
Chordoid meningiomas are difficult to manage and have a high rate of recurrence. Complete resection of the tumor is a key determinant of better outcomes. Adjuvant radiation therapy is recommended, eparticulary when Simpson grade I resection was not achieved.
PMCID: PMC4217054  PMID: 25368760
Chordoid meningioma; MIB-1 labeling index; Adjuvant radiation therapy
2.  Endoscopic Decompression for Optic Neuropathy in McCune-Albright Syndrome 
McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies including human growth hormone excess. Acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon. Surgical excision may be challenging due to the associated severe fibrous dysplasia of the skull base. Through the endoscopic procedures, we treated a case of MAS presenting with compressive optic neuropathy due to fibrous dysplasia and acromegaly caused by growth hormone secreting pituitary adenoma. We reviewed the literature on GH excess in MAS to highlight its surgical and medical challenges.
PMCID: PMC4217071  PMID: 25368777
Fibrous dysplasia; McCune-Albright syndrome; Acromegaly
3.  Delayed Cranial Nerve Palsy after Microvascular Decompression for Hemifacial Spasm 
Microvascular decompression (MVD) for hemifacial spasm (HFS) is a safe and effective treatment with favorable outcomes. The purpose of this study was to evaluate the incidence of delayed cranirve ( VI, VII, and VIII ) palsy following MVD and its clinical courses.
Between January 1998 and December 2009, 1354 patients underwent MVD for HFS at our institution. Of them, 100 patients (7.4%) experienced delayed facial palsy (DFP), one developed sixth nerve palsy, and one patient had delayed hearing loss.
DFP occurred between postoperative day number 2 and 23 (average 11 days). Ninety-two patients (92%) completely recovered; however, House-Brackmann grade II facial weakness remained in eight other patients (8%). The time to recovery averaged 64 days (range, 16 days to 9 months). Delayed isolated sixth nerve palsy recovered spontaneously without any medical or surgical treatment after 8 weeks, while delayed hearing loss did not improve.
Delayed cranial nerve (VI, VII, and VIII) palsies can occur following uncomplicated MVD for HFS. DFP is not an unusual complication after MVD, and prognosis is fairly good. Delayed sixth nerve palsy and delayed hearing loss are extremely rare complications after MVD for HFS. We should consider the possibility of development of these complications during the follow up for MVD.
PMCID: PMC3488634  PMID: 23133714
Delayed facial palsy; Microvascular decompression; Delayed cranial palsy; Delayed hearing loss; Delayed abducens palsy
4.  Clinical Efficacy of Radiation-Sterilized Allografts for Sellar Reconstruction after Transsphenoidal Surgery 
The aim of this study was to assess the safety and efficacy of radiation-sterilized allografts of iliac bone and fascia lata from cadaver specimens to repair skull base defects after transsphenoidal surgery.
Between May 2009 and January 2010, 31 consecutive patients underwent endonasal transsphenoidal surgery and all patients received sellar reconstruction using allografts following tumor removal. The allografts were obtained from the local tissue bank and harvested from cadaver donors. The specimens used in our approach were tensor fascia lata and the flat area of iliac bone. For preparation, allografts were treated with gamma irradiation after routine screening by culture, and then stored at -70℃.
The mean follow-up period after surgery was 12.6 months (range, 7.4-16 months). Overall, postoperative cerebrospinal fluid (CSF) leaks occurred in three patients (9.7%) and postoperative meningitis in one patient (3.2%). There was no definitive evidence of wound infection at the routine postoperative follow-up examination or during re-do surgery in three patients. Postoperative meningitis in one patient was improved with the use of antibiotics and prolonged CSF diversion.
We suggest that allograft materials can be a feasible alternative to autologous tissue grafts for sellar reconstruction following transsphenoidal surgery under selected circumstances such as no or little intraoperative CSF leaks.
PMCID: PMC3272510  PMID: 22323936
Endoscopic endonasal approach; Transsphenoidal approach; Allograft; Sellar reconstruction; CSF leak
5.  Moyamoya Syndrome Precipitated by Cranial Irradiation for Craniopharyngioma in Children 
Recently, combination of surgery and radiation therapy (RT) has been recommended in the treatment of craniopharyngioma. RT could be associated with late complications, including vasculopathy. We report two cases of the moyamoya syndrome seen in children with craniopharyngioma who received RT after surgical resection. Thirty-five patients in pediatric age with craniopharyngioma were surgically treated. Fifteen out of 35 patients underwent surgical resection followed by RT or gamma knife surgery. Two of the 15 were found to have symptoms of transient ischemic attack and were diagnosed as moyamoya syndrome through the cerebral angiography. Age at RT was 4 and 13 years, respectively. The latent period for development of the moyamoya syndrome was 27 months and 3 years, respectively, after RT. The RT dose of both patients was 54 Gy. These two patients received bilateral encephaloduroarteriosynangiosis procedures. We report here these two cases of radiation-induced moyamoya syndrome in pediatric craniopharyngioma. Pediatric patients with craniopharyngioma who received RT should be reminded, during follow-up, about the risk of development of the moyamoya syndrome.
PMCID: PMC3272518  PMID: 22323944
Moyamoya syndrome; Craniopharyngioma; Radiation; Vasculopathy
6.  Leukoencephalopathy and Disseminated Necrotizing Leukoencephalopathy Following Intrathecal Methotrexate Chemotherapy and Radiation Therapy for Central Nerve System Lymphoma or Leukemia 
Intrathecal methotrexate (MTX) therapy combined with whole brain radiotherapy (WBRT) is one of the major treatment modalities for leukemia and lymphoma involving the central nervous system (CNS). The purpose of this study was to retrospectively determine the incidences of leukoencephalopathy and disseminated necrotizing leukoencephalopathy (DNL) following intrathecal MTX therapy for CNS lymphoma or leukemia and to assess the potential risk factors.
Between January 2000 and August 2009, 143 patients with CNS lymphoma or leukemia received intrathecal MTX therapy alone or in combination with WBRT at a single institution. Patients were followed up clinically and radiologically at regular two- or three-month intervals. Medical records were reviewed to obtain information regarding the patients' demographics, medical histories, radiologic characteristics, treatments, and clinical courses.
On follow-up MR images, leukoencephalopathy was found in 95 of 143 patients (66.4%). The median time to develop leukoencephalopathy was 6.6 months. Among those with leukoencephalopathy, four patients showed seven extensive white-matter changes with strongly enhancing lesions demonstrating DNL. Histological confirmation was done in six lesions of three patients and radiological diagnosis alone in one patient. Four lesions spontaneously disappeared on MR images without any treatment, with a mean duration of 14 months before disappearance of DNL.
Leukoencephalopathy is a common phenomenon that occurs following intrathecal MTX therapy; however, DNL occurs at a very low incidence. For newly developed enhancing lesions, consideration for the occurrence of DNL should be taken to avoid unnecessary invasive procedures or therapies.
PMCID: PMC3243832  PMID: 22200011
Leukoencephalopathy; Methotrexate; Intrathecal; Lymphoma; Leukemia
7.  Concurrent Nocardia Related Brain Abscess and Semi-Invasive Pulmonary Aspergillosis in an Immunocompetent Patient 
We describe here the first case of a concurrent brain abscess caused by Norcardia spp. and semi-invasive pulmonary aspergillosis in an immunocompetent patient. After one year of appropriate antimicrobial therapy and surgical drainage of the brain abscess, the nocardia brain abscess and pulmonary aspergillosis have resolved.
PMCID: PMC3115156  PMID: 21716631
Brain nocardiosis; Semi-invasive pulmonary aspergillosis; Immunocompetent
8.  Transventricular Biopsy of Brain Tumor without Hydrocephalus Using Neuroendoscopy with Navigation 
It is usually difficult to perform the neuroendoscopic procedure in patients without hydrocephalus due to difficulties with ventricular cannulation. The purpose of this study was to find out the value of navigation guided neuroendoscopic biopsy in patients with peri- or intraventricular tumors without hydrocephalus.
Six patients with brain tumors without hydrocephalus underwent navigation-guided neuroendoscopic biopsy. The procedure was indicated for verification of the histological diagnosis of the neoplasm, which was planned to be treated by chemotherapy and/or radiotherapy as the first line treatment, or establishment of the pathological diagnosis for further choice of the most appropriate treatment strategy.
Under the guidance of navigation, targeted lesion was successfully approached in all patients. Navigational tracking was especially helpful in entering small ventricles and in approaching the third ventricle through narrow foramen Monro. The histopathologic diagnosis was established in all of 6 patients : 2 germinomas, 2 astrocytomas, 1 dysembryoplastic neuroepithelial tumor and 1 pineocytoma. The tumor biopsy sites were pineal gland (n = 2), suprasellar area (n = 2), subcallosal area (n = 1) and thalamus (n = 1). There were no operative complications related to the endoscopic procedure.
Endoscopic biopsy or resection of peri- or intraventricular tumors in patients without hydrocephalus is feasible. Image-guided neuroendoscopic procedure improved the accuracy of the endoscopic approach and minimized brain trauma. The absence of ventriculomegaly in patients with brain tumor may not be served as a contraindication to endoscopic tumor biopsy.
PMCID: PMC2899026  PMID: 20617084
Neuroendoscopy; Navigation; Without hydrocephalus
9.  The Efficacy and Safety of Microvascular Decompression for Hemifacial Spasm in Elderly Patients 
The purpose of this study was to examine the efficacy and safety of microvascular decompression (MVD) for hemifacial spasm (HFS) in elderly patients.
Between 1997 and June 2008, 1,174 patients had undergone MVD for HFS at our institute. Among these, 53 patients were older than 65 years. We retrospectively reviewed and compared the complication and the cure rates of these patients with those of younger patients.
There were 38 females and 15 males. The mean duration of symptoms of HFS of these patients was 94.6 months (range, 12-360 months), compared with 67.2 months (range, 3-360 months) in the younger group. The overall cure rate in elderly patients who underwent MVD for HFS during this period was 96.2%. Permanent cranial nerve dysfunctions, such as hearing loss and facial palsy, were seen in 2 patients (3.8%, 2/53) in the elderly group and 19 patients (1.7%, 19/1121) in the younger group. The difference in permanent cranial nerve dysfunction between the two groups was not statistically significant. There was no operative mortality in either group.
Microvascular decompression is the most effective surgical modality available for the treatment of HFS. Results of this study indicate that such technique can be performed in the elderly without higher rates of morbidity or mortality. Any patient with HFS, whose general health is acceptable for undergoing general anesthesia, should be considered as a candidate for MVD.
PMCID: PMC2899032  PMID: 20617090
Microvascular decompression; Hemifacial spasm; Elderly patients
10.  Combined Hyperactive Dysfunction Syndrome of the Cranial Nerves 
Combined hyperative dysfunction syndrome (HDS) defined as the combination of HDSs such as trigeminal neuralgia (TN), hemifacial spasm (HFS) and glossopharyngeal neuralgia (GPN), which may or may not occur simultaneously on one or both sides. We reviewed patients with combined HDS and demonstrated their demographic characteristics by comparing them with those of patients with a single HDS.
Between October 1994 and February 2006, we retrospectively studied a series of 1,720 patients who suffered from HDS and found 51 patients with combined HDSs. We analyzed several independent variables in order to evaluate the prevalence and etiologic factors of combined HDS.
The combined HDS group accounted for 51 of 1,720 (2.97%) patients with HDS; 27 cases of bilateral HFS, 10 cases of bilateral TN and 14 cases of HFS with TN. Their mean age was 52.1 years (range, 26-79 years). There were 5 men and 46 women. Seven patients had synchronous and 44 patients metachronous onset of HDSs. By comparison of combined and single HDS groups, we found that age and hypertension were closely associated with the prevalence of combined HDS (p < 0.05).
This study revealed that combined HDS was very rare. Hypertension and age might be the most important causative factors to evoke combined HDS.
PMCID: PMC2773393  PMID: 19893725
Combined hyperactive dysfunction syndrome; Prevalence; Hemifacial spasm; Trigeminal neuralgia; Etiology
11.  Prognostic Factors of Hemifacial Spasm after Microvascular Decompression 
The factors that influence the prognosis of patients with hemifacial spasm (HFS) treated by microvascular decompression (MVD) have not been definitely established. We report a prospective study evaluating the prognostic factors in patients undergoing MVD for HFS.
From January 2004 to September 2006, the authors prospectively studied a series of 293 patients who underwent MVD for HFS. We prospectively analyzed a number of variables in order to evaluate the predictive value of independent variables for the prognosis of patients undergoing MVD. The patients were followed-up at regular intervals and divided into as cured and unsatisfactory groups based on symptom relief. Uni- and multivariate analyses were performed using logistic regression models.
A total 273 of 293 (94.2%) patients achieved symptom relief within one year after the operation. Intraoperatively, the indentation of the root exit zone was observed in 259 (88.5%) patients. Uni- and multivariate analyses revealed that the symptoms at postoperative 3 months (p<0.001) and indentation of the root exit zone (p=0.036) were associated with good outcomes.
The intraoperative finding of root exit zone indentation will help physicians determine the prognosis in patients with HFS. To predict the prognosis of HFS, a regular follow-up period of at least 3 months following MVD should be required.
PMCID: PMC2711230  PMID: 19609416
Hemifacial spasm; Microvascular decompression; Prognosis; Chronology
12.  Hemifacial Spasm: A Neurosurgical Perspective 
Hemifacial spasm (HFS) is characterized by tonic clonic contractions of the muscles innervated by the ipsilateral facial nerve. Compression of the facial nerve by an ectatic vessel is widely recognized as the most common underlying etiology. HFS needs to be differentiated from other causes of facial spasms, such as facial tic, ocular myokymia, and blepharospasm. To understand the overall craniofacial abnormalities and to perform the optimal surgical procedures for HFS, we are to review the prevalence, pathophysiology, differential diagnosis, details of each treatment modality, usefulness of brainstem auditory evoked potentials monitoring, debates on the facial EMG, clinical course, and complications from the literature published from 1995 to the present time.
PMCID: PMC2588188  PMID: 19096569
Hemifacial spasm; Microvascular decompression; Craniofacial abnormalities

Results 1-12 (12)