A Dumbbell-shaped Thoraco-lumbar Extradural Ganglioneuroma: Case Report

Ganglioneuromas commonly arise from sympathetic ganglia. These neoplasm may be located wherever ganglion cells are normally found from skull base to pelvis including adrenal gland. Ganglioneuromas in spinal cord are very rare. We report a case of dumbbell-shaped extradural spinal ganglioneuroma. A 7-year-old boy was admitted to our hospital with a 20 day-history of both leg pain and a 10 day-history of progressive paraparesis. On magnetic resonance image, there was a dumbbell-shaped extradural spinal cord tumor in T11-L4 level. Two stage operation(First : Laminoplastic laminotomy of T11-L4 to remove the extradural portion of the tumor in the spinal canal, Second : Right retroperitoneal approach for the removal of paraspinal tumor located in L2-3 level) was performed. Pathologic diagnosis was a ganglioneuroma and paraparesis improved postoperatively.

Familial Occurrence of Cerebellar Gliomas

Several cases of familial occurrence of gliomas have been reported, but little has been known on the importance of genetic factors, which indeed remains controversial. In establishing the genetic basis for cancer susceptibility, the evaluation of a single family is perhaps the most satisfactory and meaningful approach. We report a family in which the 47-year-old father had glioblastoma in cerebellar vermis and his 14-year-old daughter developed pilocytic astrocytoma in cerebellar hemisphere. Karyotypic analysis of this family showed no abnormal findings in chromosomes.

Surgical Experiences of 2,178 Cases of Intracranial Aneurysms

OBJECTIVE: The present study is conducted to evaluate the overally surgical results in 2,178 patients with intracranial aneurysms operated in our institution from January 1980 to December 30th, 2000. METHODS: The anterior communicating artery aneurysms was 720 case, internal carotid artery aneurysms 576, middle cerebral artery aneurysms 588, anterior cerebral artery aneurysms 57 and vertebro-basilar artery aneuryms was 78 case. The male to female ratio was 0.7 to 1. Surgical methods were 1,968 clippings, 170 coatings and wrappings, 22 aneurysmorraphy, 18 proximal ligations. RESULTS: Incidence of the rebleeding was 5.6% of the early operation group, 17% of the late operation group. Incidence of the clinical vasospasm was 18.6%, angiographic vasospasm was 26.2%. The multiple aneurysms was 8.6%, dissecting aneurysm 4 cases(0.2%), "De Novo" aneurysm 4 cases(0.19%), lobectomy cases 7 cases(0.32%), and incidental aneurysms 108 cases(5.01%) respectively. Overall surgical result was favorble outcome in 86% and mortality in 7%. In early surgery group, favorable outcome was 88%, mortality was 6%. The calcium-channel blocker and "Triple-H" therapy improved the post-operative morbidity significantly. In old age group, favorable outcome was 85.5% and 8.5% mortality rate in early operation group, favorable outcome 69.8%, mortality 11.3% in late operation group. Intraoperative angiography reduced residual aneurysm or remained aneurysm in large, giant aneurysm, and complicated aneurysm especially in anterior communication artery aneurysm. CONCLUSION: It is important to know our own statisticts about the cerebrovascular disease in Korea. We present the large series of aneurysm surgery in one institute.

Surgical Experiences of 2,178 Cases of Intracranial Aneurysms

OBJECTIVE: The present study is conducted to evaluate the overally surgical results in 2,178 patients with intracranial aneurysms operated in our institution from January 1980 to December 30th, 2000. METHODS: The anterior communicating artery aneurysms was 720 case, internal carotid artery aneurysms 576, middle cerebral artery aneurysms 588, anterior cerebral artery aneurysms 57 and vertebro-basilar artery aneuryms was 78 case. The male to female ratio was 0.7 to 1. Surgical methods were 1,968 clippings, 170 coatings and wrappings, 22 aneurysmorraphy, 18 proximal ligations. RESULTS: Incidence of the rebleeding was 5.6% of the early operation group, 17% of the late operation group. Incidence of the clinical vasospasm was 18.6%, angiographic vasospasm was 26.2%. The multiple aneurysms was 8.6%, dissecting aneurysm 4 cases(0.2%), "De Novo" aneurysm 4 cases(0.19%), lobectomy cases 7 cases(0.32%), and incidental aneurysms 108 cases(5.01%) respectively. Overall surgical result was favorble outcome in 86% and mortality in 7%. In early surgery group, favorable outcome was 88%, mortality was 6%. The calcium-channel blocker and "Triple-H" therapy improved the post-operative morbidity significantly. In old age group, favorable outcome was 85.5% and 8.5% mortality rate in early operation group, favorable outcome 69.8%, mortality 11.3% in late operation group. Intraoperative angiography reduced residual aneurysm or remained aneurysm in large, giant aneurysm, and complicated aneurysm especially in anterior communication artery aneurysm. CONCLUSION: It is important to know our own statisticts about the cerebrovascular disease in Korea. We present the large series of aneurysm surgery in one institute.

Gamma-Knife Radiosurgery for Vestibular Schwannoma

OBJECT: The goals of radiosurgery include preservation of neurological function and prevention of tumor growth. We document the results of gamma-knife radio-surgery for vestibular schwannoma. METHOD AND OBJECT: Eighty-two patients underwent stereotactic radiosurgery for an vestibular schwannoma from October, 1994 to December, 2000. Sixty-five of these patients were followed up for radiological and clinical evaluation. As pregamma-knife modality, surgical resection were done in 23 patients,and V-P shunt in 2 patients. Initial symptoms were headache(n=45), dizziness(n=16), tinnitus(n=17). While normal facial function(House-Brackmann grade 1) was present in 48 patients(73.8%), other patients showed grade 2 function in 8, grade 3 function in 7,and grade 4 function in 2. The Gardner/Robertson scale was used to code hearing function. Male to female ratio was 1:3. Mean tumor volume was 7.98cm3. Mean dose delivered to the tumor margin was 14.2Gy,and mean maximal dose was 28.3Gy. RESULTS: Mean follow-up duration of 19.9 months. Thirty-five showed decrease(53.8%) in size, 19 patients(29.2%) stationary, 3(4.6%) initial decrease follow up increase, 5(7.6%) initial increase follow up decrease,and 59 patients (90.8%) were well controlled. Two patients experienced transient facial neuropathy, one transient trigeminal neuropathy, and one transient hearing deterioration. After gamma-knife radiosurgery, ventriculoperitoneal shunt was done in 4 patients. CONCLUSIONS: Gamma-knife radiosurgery can be used to treat postoperative residual tumors as well as in patients with concomitant medical problems in patients with preserved hearing function. Gamma-knife radiosurgery is safe and effective method to treat small, medium sized(less than 3cm in extracanalicular diameter), intracanalicular vestibular schwannoma, associated with low rate of cranial neuropathy.

Gamma Knife Radiosurgery on Uveal Melanoma

Uveal melanoma is uncommon but life-threatening intraocular malignancy and has been treated by irradiation, local excision and enucleation. Gamma-Knife radiosurgery allows a high dose of radiation to be delivered to an intracranial target with a very high spatial accuracy and has been used for the treatment of ocular melanomas. We have treated two cases of uveal melanoma between October 1994 and December 1999. They include one man and one woman(34, 62 years, respectively). They were followed up for 12 momths. Mean maximal dose was 65Gy. In one case, the tumor disappeared 7 months after gamma-knife radiosurgery. In another case, multiple tumors (uveal, suprasellar and cerebellar tumor) had decreased in size. These results show that single and high dose gamma-knife radiosurgery is may be an option in the local control of uveal melanoma which can spare the eyeball and vision.

Preliminary Report of Gamma Knife Radiosurgery for the Movement Disorders

No abstract available.

Multiple External Carotid Artery Aneurysms with Neurofibromatosis: Case Report

No abstract available.

Aneurysms of the Posterior Inferior Cerebellar Artery

Aneurysms of the posterior inferior cerebellar artery are uncommon, comprising between 0.5 and 0.7% of all intracranial aneurysms and are usually tightly confined to the medulla oblongata, the anterolateral base of the skull, and the lower cranial nerves. During the period from January 1983 to December 1997, about 1,600 aneurysms were operated on the department of neurosurgery, Pusan Paik hospital, Inje University. Of these, there were 8 cases of aneurysms in the posterior inferior cerebellar artery(PICA). The average age was 43.2 years, with a range from 11 months to 56 years, and male to female ratio was equal. Presenting signs and symptoms on admission were mental deterioration and/or headache after subarachnoid hemorrhage in 7 cases and suboccipital headache due to mass effect in 1 case. The locations of these aneurysms were in the anterior-medullary segment in 4 cases, in the lateral medullary segment in one case, and telovelotonsillar segment in 3 cases. Surgical methods were as followed; unilateral suboccipital craniectomy or craniotomy(right side in 3 cases and left side in 2 cases) in lateral or park bench position was performed in 5 cases and suboccipital craniectomy in prone position was in 3 cases. Direct neck clipping were done in 5 cases, proximal ligation in 1 case, coating and trapping in 1 case each. Surgical results were good in 5 cases, fair in 2 cases, dead in a case. Although clipping of the aneurysm neck was preferable in the aneurysm of PICA, trapping was useful when neck clipping was impossible in segments distal to the choroidal arch.

Malignant Rhabdoid Tumor

Primary CNS rhabdoid tumor is an extremely rare malignant tumor affecting children. Clinical, radiological, and histopathological features of malignant rhabdoid tumor are presented. Following the complete extirpation of a large tumor at the frontoparietal region, a 16-year-old male patient suffered from several local recurrences. The neoplasm is, defined by its histopathological and ultrastructural characteristics and by the pattern of its antigenicity as a malignant rhabdoid tumor(MRT).

Huge Cyst Formation after Gamma Knife Radiosurgery for Arteriovenous Malformation: A Case Report

Radiosurgery is effective in obliterating cerebral arteriovenous malformation(AVM), and delayed cyst formation after radiosurgery is very rare. An 18-year-old man underwent Gamma Knife radiosurgery for arteriovenous malformation located in the left frontotemporal area, which had manifested with hemorrhaging. The 15,01cm3 nidus was covered with a 50% isodose volume. A central dose of 50Gy was used to obtain a marginal dose of 25Gy. One year later, he developed headache and right hemiparesis. MRI showed a huge, multilobulated cyst around the AVM. Follow-up cerebral angiography showed partial obliteration of the AVM nidus. Removal of cystic fluid(about 70cc) was done by stereotactic method, which was revealed as translucent, xanthochromic but had no tumor cell. Two months later, reaccumulation of the cyst was seen on the follow-up MRI insertion setting of Ommaya reservoir was performed. For the diagnosis of complications after radiosurgery for AVM, follow-up study, will be needed.

Three Cases of Intracranial Hematoma Associated with Meningioma

Spontaneous intracranial hematoma in patients with brain tumors is well recognized, However, intracranial hematoma associated with meningioma has been rarely reported. Not infrequently, clinical manifestations of such tumors have been masked by the associated hematoma, which preclude correct diagnosis of these tumors. Meningioma-associated hemorrhages have been reported regardless of sex, age, or location and histology of the tumor. The mechanisms of such hemorrhage are yet to be clarified. During the last two years, we have experienced three cases of intracranial hemorrhage associated with meningioma. Two of them occurred acutely without antecedent symptoms of the meningioma and other one after gamma knife surgery of meningioma. We reviewed partinent literature and discussed possible mechanisms of these rare intratumoral or peritumoral hemorrhages associated with meningiomas.

Systemic Lupus Erythematosus with Central Nervous System Involvement: Case Report

Systemic lupus erythematosus(SLE) is a disease of unknown cause in which tissues and cells are damaged by pathogenic autoantibodies and immune complexes. Clinical manifestations which fulfill American Rheumatism Association criteria for a diagnosis of SLE include malar or discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal, neurologic, hematologic or immunologic disorder, and antinuclear antibodies. Central nervous system (CNS) events may be single or multiple. Clinical manifestations of CNS involvement include organic brain syndromes, focal infarcts, headache, transverse myelitis, optic neuritis, cranial nerve palsies, depression, and anxiety. A 34-year-old female presented with generalized seizure. Antinuclear antibody and antibody to Sm were positive, and low levels of C3 and C4 were detected. On MRI, mild, non-enhancing, low signal intensity was seen on T1 weighted images, and illdefined higher intensities on T2 weighted images. On stereotactic biopsy, three pieces of yellowish material were obtained. Microscopically, coagulative necrosis and neutrophilic infiltration were seen, and the patient underwent steroid therapy. Two months later, MRI no longer showed abnormal signal intensity.

Familial Occurrence of Moyamoya Disease: Case Report

There is extensive evidence that moyamoya disease has a tendency for multifactorial inheritance, although the pathogenesis of moyamoya disease is not clear. The authors report a case of familial moyamoya disease involving a 51-year-old female and her 24-year-old daughter, both of whom initially presented, within a 1-year period, withintracerebral hemorrhage and intraventricular hemorrhage. Cerebral angiography showed typical features of moyamoya disease in both patients, but the cause of the pathology remains obscure. The pertinent literature is reviewed.

Surgical Experiences of 1,500 Cases of Intracranial Aneurysm

The principle role of treatment for ruptured aneurysm has been to obliterate the aneurysmal sac and secure the patient's life from the hazards of rebleeding and vasospasm. The present study was conducted to evaluate the overally surgical results in 1,500 patients with intracranial aneurysms operated in our institution from January 1980 to February 1997. Among these, 547 cases were anterior communicating artery aneurysms, 453 internal carotid artery aneurysms, 400 middle cerebral artery aneurysms, 43 anterior cerebral artery aneurysms and 57 cases of vertebro basilar artery aneurysms. Overall surgical result was favorable outcome in 84% and mortality in 8%. The rebleeding rate of early operation group was 7 percent, as compared to 23 percent in late operation group. In this retrospective study, the incidence of angiographic vasospasm was 34.2 percent and clinical vasospasm in 28.7 percent of good grade patients. Incidental aneurysms constistuted 5.6 percent of the this series, and multiple aneurysm 8.7 percent. The causes of the mortality and morbidity were initial insult of bleeding, rebleeding, vasospasm, surgical complications, and systemic medical problems. Regarding the surgical timing, it is difficult to decide whether early or late surgery would provide a better prognosis.

Terson's Syndrome in Aneurysmal Subarachnoid Hemorrhage

Terson's syndrome is the oculocerebral syndrome of retinal and vitreous hemorrhage associated with spontaneous subarachnoid hemorrhage(SAH) or all forms of intracranial bleeding. Recent observations indicate that the patients with spontaneous subarachnoid hemorrhage have an 18% to 20% concurrent incidence of retinal and vitreous hemorrhages and about 4% incidence of viterous hemorrhage alone. Therefore, clinical ophthalmologic findings may have a significant diagnostic and prognostic value for the clinician. Total of 627 consecutive patients with aneurysmal subarachnoid hemorrhage were retrospectively studied by means of indirect fundoscopy to address the question of occur and prognostic implications of vitreous hemorrhage after subarachnoid hemorrhage from January 1993 to December 1996. Among these, we experienced 21 patients with vitreous hemorrhage(Terson's syndrome). The patients with vitreous hemorrhage commonly occurred in anterior communicating and internal carotid artery aneurysm. Twelve patients had intraocular hemorrhage within 48 hours after SAH. There were bilateral involvement in 15 cases, and the 14 experienced complete recovery of vision following massive intravitreal hemorrhage. A favorable surgical outcome was obtained in 14 of 21 patients(66.7%) and a satisfactory result in vision was achieved in 15 cases among 17 survivors(88.2%). From these results, we emphasize the significance of ocular findings in patients with aneurysmal subarachnoid hemorrhage.

Gamma Knife Radiosurgery in Cerebral Arteriovenous Malformation

Between October 1994 and October 1996, 28 cerebral arteriovenous malformation(AVM) patients were treated with Gamma Knife radiosurgery at Pusan Paik Hospital, Inje University. Their ages ranged from 13 to 64(mean 32.9) years, and ten cases were followed up over one year. To reduce the size of AVM before surgery, intravascular embolization was performed in one case and surgical procedures(subtotal and partial resection)in three. All cases were symptomatic: seven had hemorrhage, Two involved seizures, and one suffered from headaches. The AVMs were located in the basal ganglia in four cases, were parietal in three, temporal in two and one was located in the frontal lobe. The volume of the AVM was more than 10cc in three cases, 4-10cc in one case and less then 4cc in six cases. After Gamma Knife radiotherapy, complete obliteration was confirmed by follow-up angiography or MRA in three cases, and partial obliteration in six; two of these six developed ICH at 6 and 10 months after GKS. In both, the nidus was located at the periventricular area, and the volume was more than 10cc. Maximum doses were 10,8-2.5Gy. The clinical results were that eight cases showed no change, one recovered but showed mild neurological deficit, and one died.

Intracranial Meningeal Melanocytoma: Case Report

The majority of melanotic neoplasms of central nervous system(CNS) are metastatic in origin and rarely a primary CNS melanomas, and are usually histologically and clinically malignant in nature. The meningeal melanocytomas are benign, primary melanotic neoplasm and are less common than primary CNS melanoma. They usually present in early adult and have a characteristic appearance in light and electron microscope. A 42-year-old male is presented with pain on the neck and suboccipital region which was worse with flexion and Valsalva's maneuver. MRI revealed homogenous high signal intensity on the T 1 weighted image and low signal intensity on the T 2 weighted image. At operation, the tumor was well circumscribed, pigmented lesion and was attached to dura and cord. Microscopically, the tumor was composed of spindled and epithelioid cells with often prominent nucleoli, without evidence of mitosis and necrosis.

Postoperative Cerebral Blood Flow Evaluation by Stable Xenon Computed Tomography in Patients with a Ruptured Intracranial Aneurysm

In cerebrovascular disease, the measurement of cerebral blood flow(CBF) is an important indicator of a patient's clinical status, treatment and prognosis. The main advantage of Stable Xenon CT is that it noninvasively provides three-dimensional, high resolution, quantitative local cerebral blood flow information coupled to anatomic exposition. Using this modality, we measured postoperative CBF in 50 patients with subarachnoid hemorrhage from a ruptured intracranial aneurysm. The results were as follows : 1) There was a negative relationship between age and CBF. 2) Among postoperative disability patients, global and regional CBF were markedly diminished. 3) CBF on the ipsilateral side of a craniotomy site was markedly lower than on the contralateral side. Xenon CT is a very useful method for the evaluation of postoperative CBF in intracranial aneurysm patients.

Clinical Characteristics of Cerebrovascular Disease in Infants and Children and Its Outcomes

PURPOSE: The incidence and clinical features of cerebrovascular disease in childhood were not clearly clarified in domestic. This study was undertaken to evaluate the clinical characteristics of cerebrovascular disease in infants and children, and its outcome, a retrospective study was performed to adequate management. METHODS: We reviewed clinical records of the patients who had admitted to Inje University Pusan Paik Hospital during 6 years from 1989 to June 1994. A 6-year experience with childhood cerebrovascular disease unrelated to birth, intracranial infection,leukemia associated or trauma identified 41 patients. RESULTS: There were 14cases(34%)of ischemic cerebrovascular disease as well as 27cases(66%) of hemorrhagic cerebrovascular disease. 1) The male to female ratio was 1.73:1. 2) We observed two incidence peaks in hemorrhagic stroke,one in infant(1<) and the other in 10 year old,in ischemic stroke, one in between 2-5 year, the other in between 6-10 year. 3) The presenting symptoms and signs of hemorrhagic cerebrovascular disease were altered consciousness, vomiting, headache, diarrhea but that of ischemic cerebrovascular disease were hemiparesis, seizure, altered consciousness, headache. 4) The incidence of hemorrhagic stroke was higher than ischemic stroke. 5) In hemorrhagic stroke, Arteriovenous malformation was most common, and the next was late hemorrhagic disease of infancy. 6) In ischemic stroke,moyamoya disease was most common. 7) The mortality rate in stroke was 9.7 percent,it was only in hemorrhagic stroke. 8) Hemiparesis was the commonest of the residual deficits. CONCLUSIONS: Considering above results We concluded that there was higher initial mortality rate in hemorrhagic stroke, but relatively lower residual disabilities. And since the neurologic deficit was severe in ischemic stroke in spite of proper management,so intensive managements are required for preventing disability.