Biomedical subjects

Ho Jun Seol

Publications and source records attributed to Ho Jun Seol.

11 recordsLinked to original sources

PubMed2006

Familial occurrence of moyamoya disease: a clinical study.

BACKGROUND: We reviewed a consecutive series of moyamoya disease (MMD) in children and studied their familial pedigrees to determine whether they showed specific clinical features or patterns of inheritance, and to investigate any correlation between familial MMD and common Asian diseases. METHODS: Cases of familial MMD (N=10) were reviewed in the aspect of clinical presentation, such as, symptoms and signs, age of onset, imaging studies including magnetic resonance imaging (MRI), cerebral angiography, and single photon emission computed tomography (SPECT), and operative results including complications, to identify differences between these patients and those with sporadic MMD (N=194). The male to female ratio in those with familial MMD was 4:6 and mean age was 8 years (3-17). All were ischemic cases and five showed cerebral infarction on MRI. As a preliminary genetic study, familial pedigrees were examined. In addition, their familial histories concerning common Asian diseases, such as, hepatic disease, cancers, stroke, coronary heart disease, amyloidosis, and systemic lupus erythematosus, were investigated by telephone survey. RESULTS AND CONCLUSIONS: The familial MMD cases did not reveal any differences from the other MMD children in terms of clinical findings, imaging data, or surgical results. In our series, five cases (50%) showed MMD between siblings. Familial MMD relations were also observed with cousins, a mother, and an aunt. No specific pattern of genetic inheritance was observed, and no relation was found between the familial occurrence of MMD and common Asian diseases.

Adolescent↗

PubMed2006

Surgically treated tuberculum sellae and diaphragm sellae meningiomas: the importance of short-term visual outcome.

OBJECTIVE: The visual outcome in patients with tuberculum and diaphragm sellae meningiomas treated with microsurgery was evaluated. Prognostic and diagnostic values of short- and long-term postoperative visual outcome and etiology for postoperative visual deterioration are discussed with special attention. METHODS: Clinical data for 30 surgically treated patients with tuberculum and diaphragm sellae meningiomas were reviewed retrospectively. The mean duration of the follow-up period was 75.9 months (range, 12-151 mo). Mean tumor diameter and volume was 25.9 mm (range, 16.3-63.3 mm) and 12.4 cm (range, 2.3-125.6 cm). A visual impairment score was used to assess the short-term (< or =2 wk after surgery) and the long-term (>6 mo after surgery) postoperative visual outcome. Various predictive factors for visual outcome were tested statistically. RESULTS: Complete resection was achieved in 23 out of 30 (76.7%) patients. Average preoperative, short- and long-term visual impairment scores were 48.2, 43.4, and 40.9, respectively. Favorable visual outcome was achieved in 80% of patients in the short term and 70% in the long term. Short-term postoperative aggravation of visual function was an ominous sign of further aggravation or at least of little hope for recovery, whereas there was a tendency to improve in the long term if short-term postoperative visual function showed favorable outcome. Recurrence or regrowth of tumor fully was responsible for late deterioration of visual function. No significant prognostic factor for visual outcome could be found. CONCLUSION: Short-term postoperative visual outcome was a strong indicator of permanent visual outcome after surgery for tuberculum sellae and diaphragm sellae meningiomas.

Adult↗

PubMed2006

Optimal extent of resection in vestibular schwannoma surgery: relationship to recurrence and facial nerve preservation.

Surgical treatment of vestibular schwannoma is targeted at complete removal with preserved neurological function. Complete removal may cause significant deficits, whereas subtotal tumor removal is associated with a high recurrence rate. The present study assessed the risk of tumor recurrence and postoperative facial nerve function in relation to the extent of surgical resection by reviewing the clinical records and radiological findings of 116 patients with vestibular schwannoma treated between 1990 and 1999. The extent of resection was classified as follows: gross total resection (GTR), near total resection (NTR), and subtotal resection (STR). Facial nerve function was graded using the modified House-Brackmann grade, and patients grouped into good (grades 1-2) and intermediate or poor (grades 3-6). Of the 116 patients, 26 (22%) underwent GTR, 32 (28%) NTR, and 58 (50%) STR. The recurrence rates were 3.8% (1/26 cases), 9.4% (3/32), and 27.6% (16/58) for GTR, NTR, and STR, respectively. GTR and NTR showed no statistically significant difference in terms of recurrence rate (p=0.620). However, recurrence was significantly less after NTR than STR (p=0.043). Immediately postoperative facial nerve function was good in 15.4% of patients after GTR, 40.6% after NTR, and 46.6% after STR. The STR and NTR carried a lower risk of facial nerve palsy than GTR in the immediately postoperative stage (p=0.006 and 0.036, respectively). Nevertheless, no statistical significance was observed in extent of resection and postoperative facial nerve outcome between the groups at last follow up (p=0.227). GTR is the ideal surgical treatment for vestibular schwannoma, but NTR is a good option, with better facial nerve function preservation than GTR without significantly increasing the risk of recurrence.

Adult↗

PubMed2005

Unilateral (probable) moyamoya disease: long-term follow-up of seven cases.

OBJECTS: Some cases of unilateral involvement have angiographic findings on the affected side which resemble those of definite cases of moyamoya disease (MMD). We analyzed these cases to determine whether the entity "unilateral MMD" actually exists and whether unilateral involvement represents only the early phase of the pathologic process found in definitive bilateral disease. METHODS: Between 1988 and 2000, seven cases of unilateral MMD were evaluated. The male-to-female ratio was 2:5, mean age was 5.1 years (7 months to 8 years), and mean follow-up duration was 64.7 months. The presenting symptoms of these cases were similar to those of definite MMD, and no familial occurrence was found. Two of the seven unilateral MMD cases showed progression to bilateral involvement. However, the remaining cases showed no evidence of contralateral progression during the follow-up period. CONCLUSIONS: Most cases of unilateral MMD seem to be an unusual form of stenoocclusive process of the proximal intracranial arteries, distinguishing the condition from definite MMD. Surgical treatment on the symptomatic side and close observation for signs of bilateral involvement are necessary.

Cerebral Angiography↗

PubMed2005

Aggressive vestibular schwannomas showing postoperative rapid growth - their association with decreased p27 expression.

Vestibular schwannomas (VSs) are relatively slow growing tumors. However, some rapidly regrow or recur after surgical resection. The objective of this study was to identify those molecular characteristics predicting rapid recurrence after surgical resection. Immunohistochemically determined expressions of several cell cycle regulators and apoptosis-associated proteins in 12 cases of aggressive VS (AVS) and in 15 control cases of usual VS (UVS) cases were compared. The expressions of p53 and Bax (pro-apoptotic protein), Bcl-2 (anti-apoptotic protein), Fas, and Fas-L (apoptotic death receptor and ligand), caspase 3 (apoptotic effector caspase proteins), and p27 and p21 (cyclin-dependent kinase inhibitors) were analyzed using tissue array blocks. Loss of p27 expression was observed in 8 of 12 AVS cases (67%) and in 3 UVS cases (20%); p21 was expressed in all cases. Loss of Bax was observed in 3 AVS and 3 UVS cases. The anti-apoptotic protein, Bcl-2, was expressed in 9 AVS (75%) and 11 UVS (73%), and p53, Fas-L, and caspase 3 were negative and Fas was positive in all AVS and UVS cases. Of these, only the loss of p27 was statistically significant (P = 0.02). The loss of p27 in AVS may explain the unusually high proliferative potential of AVS versus UVS, and p27 may be a predictor of VS aggressiveness. The expressions of other apoptosis associated proteins were not significantly different in the two groups. This may be the first report to identify a molecular entity associated with aggressive VS. However, further studies are required.

Cell Proliferation↗

PubMed2005

Headache in pediatric moyamoya disease: review of 204 consecutive cases.

OBJECT: Patients with moyamoya disease complain of headache before surgery, after surgery, or in both periods. To clarify the pathophysiological features of these headaches which are one of the major symptoms in moyamoya disease after indirect bypass surgery, the authors reviewed data obtained in patients with moyamoya disease who underwent such surgery at their institute. METHODS: The authors surveyed 204 children with moyamoya disease younger than 17 years of age who underwent indirect bypass surgery consecutively between 1988 and 2000. If headache and associated symptoms disturbed their daily activity, required rest and/or medication, and occurred at least once a month, the headache was regarded as significant and the patient was included in the study. A postoperative headache was defined as the presence of significant headache 12 months after the operation or later. Preoperative headache was documented in 44 patients. Postoperative headache was observed in 63% (28 of 44) of the patients with preoperative headache and in 6.3% (10 of 160) of those without preoperative headache. Aggravation on postoperative magnetic resonance images or single-photon emission computerized tomography scans did not correlate with this symptom (p = 0.729 and 0.203, respectively). The clinical course had the following features: 1) a coexisting stage of headache and transient ischemic attack; 2) a second stage of headache only; and 3) a final stage of improvement or disappearance of headache. CONCLUSIONS: The authors demonstrated that headaches can persist or develop after indirect bypass surgery despite successful prevention of cerebral ischemia. In addition to decreased cerebral blood flow, progressive recruitment and redistribution of blood flow should be considered as a cause of headaches in patients with moyamoya disease.

Activities of Daily Living↗

PubMed2004

Moyamoya disease among young patients: its aggressive clinical course and the role of active surgical treatment.

OBJECTIVE: The prognosis for moyamoya disease (MMD) among young patients is known to be worse than that among older patients. The aim of this study was to investigate the clinical features and treatment outcomes of young patients with MMD. METHODS: A total of 204 patients with MMD who underwent encephaloduroarteriosynangiosis, with or without bifrontal encephalogaleosynangiosis, were categorized into three groups according to their ages at the time of surgery, i.e., Group A (n = 23, <3 yr of age), Group B (n = 50, 3-6 yr of age), and Group C (n = 131, >6 yr of age). For each group, patterns of presentation and the occurrence of subsequent preoperative or surgery-related infarctions were assessed. Clinical outcomes and postoperative hemodynamic status were analyzed. RESULTS: At initial presentation, infarctions were significantly more frequent in Group A (87%) and Group B (58%) than in Group C (46%). Subsequent preoperative infarctions occurred significantly more frequently in Group A (39%) than in Group B (6%) or Group C (0.8%). The median interval between the onset of symptoms and a subsequent preoperative infarction was 3 months (range, 1-14 mo). No significant difference in the rates of surgery-related infarctions among the three groups was observed. The rate of favorable clinical outcomes was significantly lower in Group A (58%) than in Group B (84%) or Group C (86%), although the rates of postoperative hemodynamic improvements were similar among the groups. The poor clinical outcomes for Group A were caused mainly by preoperative infarctions. CONCLUSION: Young-age MMD demonstrates rapid disease progression and results in poor clinical outcomes. These findings indicate the necessity of early surgery for young patients with MMD; however, the actual benefits should be verified with additional controlled studies, with long-term follow-up monitoring.

Adolescent↗

PubMed2003

A case of recurrent subependymoma with subependymal seeding: case report.

Subependymoma is a rare, slow growing, rarely recurrent tumor. We report a case of re current subependymoma with subependymal seeding. An intraventricular tumor in the left temporal horn was detected in a 48-year-old female who presented with a 4-year history of dizziness and memory disturbance. Following near total surgical resection, a tumor diagnosis of subependymoma was confirmed by scattered clusters of isomorphic nuclei embedded in a dense fibrillary matrix of glial cell processes. Twenty-six months after surgery, follow-up (F/U) magnetic resonance (MR) imaging revealed tumor recurrence in the previous site which necessitated linear accelerator radiosurgery (LINAC). A further 21 months later, F/U MR imaging showed recurrent, multiple, enhanced, nodular lesions in the enlarged left lateral ventricle for which the patient underwent reoperation. Radiological and operative findings revealed local relapse with subependymal seeding. The pathological finding was similar to that of the previous tumor and compatible with subependymoma. The patient underwent radiation therapy for the residual tumor. This case history suggests that symptomatic residual tumors require close observation even though the clinical course of subependymoma is usually benign.

Brain Neoplasms↗

PubMed2002

Surgical approach to anterior compression in the upper thoracic spine.

OBJECT: The anterior upper thoracic spine (T1-3) is difficult to access because most neurosurgeons are unfamiliar with the anatomy. This study was performed to evaluate the different surgical options by retrospectively analyzing data on operations performed for anterior upper thoracic compression at the authors' institution. METHODS: Eighteen patients underwent surgery between November 1993 and May 2001. There were eight men and 10 women; their mean age was 55 years (range 28-80 years). All patients presented with pain and/or neurological deficits. The causes of anterior compression were diverse, although metastatic spinal tumor was most common. The approach chosen was primarily dictated by the axial involvement of the lesion. Anterior approaches, mainly the transmanubrium approach, were performed in six and posterior approaches in 12. In all cases except one, in which only an iliac bone graft was placed, instrumentation was used. The mean follow-up period was 11.4 months (range 1-57 months). One postoperative death occurred. Postoperative follow-up imaging studies, especially plain radiography, demonstrated no instrumentation failure. Improvement was shown in eight patients, an aggravation of symptoms in one, and stable clinical status in eight. CONCLUSIONS: Decompression may be achieved on the anterior side of the upper thoracic spine if the operative approach is scrupulously chosen; this choice involves consideration of the locations of the lesion, the nature of the primary disease, and the surgery-related risk.

Adult↗

PubMed2002

Radiosurgical treatment of a cerebral arteriovenous malformation in a patient with moyamoya disease: case report.

OBJECTIVE AND IMPORTANCE: A case of a cerebral arteriovenous malformation (AVM), which was treated with gamma knife surgery, in a patient with moyamoya disease is described. This is the first report of radiosurgical treatment of a cerebral AVM in a patient with moyamoya disease; the outcome was satisfactory. CLINICAL PRESENTATION: The patient presented with frequent episodes of transient ischemic attacks and seizures. INTERVENTION: Staged encephaloduroarteriosynangiosis was performed on both sides, with an interval of 10 months. Postoperative cerebral angiography demonstrated augmented cerebral perfusion, which increased the size of the AVM nidus. The patient subsequently underwent gamma knife surgery, and the AVM completely disappeared 2 years later. CONCLUSION: Radiosurgery may be a good treatment option for AVMs accompanying moyamoya disease, allowing the preservation of collateral vessels and the prevention of possible misery perfusion.

Adult↗

PubMedDate not supplied

Inflammatory pseudotumor in the epidural space of the thoracic spine: a case report and literature review of MR imaging findings.

We present a rare case of pathologically proven inflammatory pseudotumor in the thoracic spine. The lesion showed an isointense signal on T1-weighted images, a heterogeneous iso- and hyperintense signal on T2-weighted images, and strong homogeneous enhancement. There was no evidence of abnormalities in the adjacent bone. Whereas the exact pathogenesis of this lesion is unknown, it has been regarded as an unusual response to insults such as trauma or acute infection, a post-inflammatory reparative process, or low-grade malignancy.

Adult↗