Dae Hee Han

A co-operative study : Clinical characteristics of 334 Korean patients with Moyamoya Disease Treated at Neurosurgical Institutes (1976-1994)

Summary A co-operative study was conducted to determine the clinical characteristics of patients with moyamoya disease who were diagnosed and treated at neurosurgical institutes in Korea before 1995. Twenty-six hospitals contributed 505 cases and among them, the clinical characteristics of 334 patients with definite moyamoya disease were evaluated. The number of patients began to increase from the late 1980s, and after that approximately 20 patients were treated each year. There were two age peaks: from six to 15 and from 31 to 40 years of age. Haemorrhagic manifestations occurred in approximately 43% of the patients. The major clinical manifestations were haemorrhage in adults (62.4%) and ischaemia in children (61.2%). Overall 54.5% of the patients experienced decreased consciousness levels, mainly due to intracranial haemorrhage or cerebral infarction. In the patients with ischemic manifestations, the adult patients were more likely to have cerebral infarction than the pediatric patients (80% vs. 39%) and the pediatric patients were more likely to have TIA (61% vs. 25%). Thirty eight percent of the patients underwent bypass surgery and 53% of these procedures were performed bilaterally. Treatment policies, including indications for bypass surgery and commonly used drugs, were somewhat different according to the institution. Overall favorable outcome was 73%, and the most significant factor affecting poor outcome was haemorrhagic manifestation. This article describes the characteristics of 334 patients with moyamoya disease, who were diagnosed and treated at neurosurgical institutes in Korea before 1995.

Clinical characteristics of dural arteriovenous fistula

Intracranial dural arteriovenous fistula (DAVF) is an uncommon neurosurgical condition; in particular, it has been infrequently reported in Korea. To understand the general clinical characteristics of DAVFs, the authors reviewed 53 cases and analyzed factors affecting DAVF hemorrhage of and treatment outcome. Since 1980 we have encountered 480 pial and 53 DAVFs, a ratio of 9.1 to 1. The age of these patients ranged from 1 month to 71 years, the most common being in the 6th decade, and females exceeded males by 1.65 to 1. All lesions except three were single, and symptoms were related to location and the venous drainage pattern. The most common location was the cavernous sinus, accounting for about 64% of cases, with the result that the most common clinical symptoms of DAVFs were ocular, namely proptosis and chemosis. The next was tinnitus also found in transverse-sigmoid sinus DAVFs. Intracranial hemorrhage was seen in eight cases,(15%) the primary cause of hemorrhage was retrograde intracranial venous drainage (P=0.017), and one hemorrhage was observed in cases with no intracranial venous drainage. Intracranial hemorrhage was more frequently in transverse-sigmoid than cavernous sinus DAVFs (P=0.049), and this proved to be so even where there was intracranial venous drainage. However, two of 34 patients with cavernous DAVFs became blind in one eye, demonstrating that in such patients, the clinical course could be aggressive. Thirteen patients were treated conservatively. The conservative treatment group was comprised of 13 patients, two of three patients with transverse-sigmoid sinus DAVF expired, and 7 of 10 with cavernous sinus DAVF experienced a clinical improvement or cure. Surgical excision was performed in only two patients. A total of 39 patients underwent embolization; clinical cure was achieved in 13, improvement of symptoms in 12, an unchanged or aggravated result occurred in 9, one died, and four were lost to follow up. During intervention, there was one hemorrhagic complication, owing to obstruction of the venous outflow with embolic materials. In this study, the most common location of DAVFs was the cavernous sinus. The cortical venous drainage remains the primary determinant of intracranial hemorrhage. Common indications for treatment include hemorrhage and neurological deficit. Endovascular treatment is preferred in the majority of cases except tentorial DAVF. The goal of embolization in cavernous DAVF is the alleviation of symptoms, not angiographic cure. But transverse-sigmoid sinus DAVF with venous restriction and leptomeningeal drainage should be treated aggressively.

Pineal tumors: experience with 48 cases over 10 years

Abstract The authors retrospectively reviewed 48 patients treated at Seoul National University Hospital (SNUH) between 1986 and 1995. There were 35 children and 13 adults, accounting for 10.1% of 345 pediatric and 0.68% of 1914 adult brain tumors in SNUH during the same period. The 48 cases consisted of 33 cases of germ cell tumor (69%, GCT); 6 of pineoblastoma (PB, 12.5%); 3 of pineocytoma (PC, 6.3%); 3 of anaplastic astrocytoma (6.3%); 1 of astrocytoma; 1 of glioblastoma; and 1 of ependymoma. The median age was 13 years (range 1–59) and the male-to-female ratio was 3.36:1. The most frequent presenting symptom was due to increased intracranial pressure (90%), followed by Parinaud syndrome or diplopia (50%). Patients with a benign tumor, such as teratoma (TE), astrocytoma, or ependymoma, underwent surgery by the occipital transtentorial approach (OTT) for attempted radical resection without adjuvant therapy, while patients with immature teratoma (imTE), PC, and anaplastic astrocytoma underwent regional radiotherapy (RT) after debulking via OTT. Seven patients with nongerminomatous malignant GCT (NG-MGCT) and 3 with germinoma (GE) underwent craniospinal radiation only, 6 with GE, a NG-MGCT, and 2 with GE+TE received craniospinal radiotherapy (CSRT) after debulking via OTT. Three patients with GE, 4 with NG-MGCT, and 3 with PB underwent radiochemotherapy after debulking via OTT. Forty-four patients were followed up after treatment. The median follow-up period was 36 months. All patients with GE were alive after RT at 36 months (median) of follow-up (range 7–70 months). All with GE+TE and TE were alive. Three patients with PC or astrocytoma were also alive with stable or no evidence of disease. In 1 of the 3 cases of imTE there was a recurrence. However, 4 patients with NG-MGCT died, all of whom had undergone CSRT only; 2 PB patients were alive (12, 19 months), 1 in a moribund status (36 months), and 2 were dead (6, 60 months). The overall mean survival time with pineal tumors was 66 months and the 3-year survival rate was 84% with minimal posttreatment complications. It is concluded that pineal region tumors have male and childhood predominances, and the most common tumor is GCT. The majority of pineal region tumors are malignant. Pineal region tumors can be approached safely and effectively and the surgical complications are mostly transient. Their prognosis is dependent on the pathologies and treatment modalities.

Cerebral aspergillosis in immunologically competent patients

Aspergillosis of the central nervous system is a rare disease, especially if the patients immune system is not compromised. The authors report three cases of cerebral aspergillosis in the immunocompetent state: a rhinocerebral form in a diabetic patient, a direct extension from chronic Aspergillus otitis media, and a postoperative Aspergillus brain abscess after brain tumor surgery. In spite of the poor prognosis of cerebral aspergillosis, two of the patients survived. The pathogenesis, predisposing factors, radiologic findings including magnetic resonance image, and the outcome are presented. The pertinent literature of cerebral aspergillosis is also reviewed.

Cerebral sparganosis : analysis of 34 cases with emphasis on CT features

SummaryCerebral sparganosis is a rare parasitic CNS disease, producing chronic active granulomatous inflammation. We retrospectively reviewed the clinical data, CT scans and histopathologic specimens in 34 patients with cerebral sparganosis. The majority of the patients (89%) were rural inhabitants; 75% had a history of ingestion of frogs and/or snakes. The major presenting symptoms were seizure (84%), hemiparesis (59%) and headache (56%) of chronic course. On CT scans, the disease most frequently involved the cerebral hemispheres, particularly frontoparietal lobes, with occasional extension to the external and internal capsules and basal ganglia. The cerebellum was rarely involved. Bilateral involvement was seen in 26%. The main CT findings consisted of white matter hypodensity with adjacent ventricular dilatation (88%), irregular or nodular enhancing lesion (88%), and small punctate calcifications (76%). In combination, the CT triad above appears to be specific for this disease, and was noted in 62% of cases. Of 16 follow-up CT scans, 5 (38%) showed a change in the location of the enhancing nodule. With a single CT scan, it does not appear to be possible to determine whether the worm is alive or dead, information important for deciding whether to intervene surgically. Change in the location of the enhancing nodule and/or worsening of the other CT findings on sequential CT scans would suggest that the worm is alive and that the patient is a candidate for surgery.

Moyamoya disease in adults: characteristics of clinical presentation and outcome after encephalo-duro-arterio-synangiosis

To determine the clinical characteristics and the effectiveness of encephalo-duro-arterio-synangiosis (EDAS) in adulthood-onset Moyamoya disease (MMD), the authors retrospectively reviewed 26 patients suffering from MMD who were admitted to Seoul National University Hospital between 1987 and 1995. When they showed major symptoms, all were more than 16 years-old. The most common presenting symptom was intracranial hemorrhage (ICrH), found in 12 patients or 46% of the total; the second was infarction and transient ischemic attack, each found in seven or 27% of them. Only one patient was found to have seizures, which were associated with a cerebral infarction. The Suzuki angiographic stage 3 and less than stage 3 accounted for 73% of all 52 hemispheres. A total of 15 patients underwent single photon emission computed tomography (SPECT) preoperatively. When the derangement of cerebral perfusion was estimated with four SPECT grades (SG), 70% of their hemispheres revealed normal (SG1) or localized decreased-perfusion (SG2). The other 30% had extensive decreased-perfusion or localized perfusion defects (SG3). There was no case who had extensive perfusion defects (SG4). A total of 17 patients underwent EDAS operations (EDAS group) and nine did not undergo any operation (no-op group). The EDAS group had significantly better clinical outcomes than the no-op group after a 12-month median follow-up period (P < 0.05). The angiographic and SPECT follow-up studies comprised six and seven cases, respectively. There was also satisfactory angiographic revascularization in all follow-up cases and improvement in cerebral perfusion at SPECT follow-up in six of seven cases. It is concluded that the involvement of posterior circulation of MMD is not frequent and cerebral perfusion is preserved in adulthood-onset MMD patients. These findings may explain the reason why hemorrhages are frequent and the late onset of symptoms in adulthood-onset MMD. Surgical treatment with EDAS seems to be effective in adulthood-onset MMD in terms of clinical improvement.

Gamma knife surgery of superficially located meningioma

OBJECT The authors analyzed tumor control rates and complications in patients with superficially located meningiomas after gamma knife surgery (GKS). METHODS Between 1998 and 2003, GKS was performed in 23 patients with 26 lesions in whom follow-up imaging for 1 year or more was available. The male/female ratio was 1:22. The mean age was 59 years. The median tumor volume was 4.7 cm3, and the mean margin dose was 16 Gy at the 50% isodose line. Peritumoral edema was revealed on magnetic resonance (MR) imaging in four patients before GKS. Magnetic resonance imaging and clinical examinations were performed every 6 months after GKS. The mean follow-up duration was 32 months. The tumor shrank in eight cases, was stable in 17, and enlarged in one; thus 25 (95%) of 26 tumors were controlled. A peritumoral high signal on T2-weighted MR images was found in eight lesions and preexisting edema was aggravated in three lesions after GKS. Ten of these 11 patients complained of severe headache, and three patients experienced neurological deficits at the same time after a mean latency of 3 months; however, high signal was not demonstrated on imaging before 6 months on average. Steroid agents, when required, gave relief to all patients. The complication rate was 43% (10 of 23 cases). High signal disappeared in nine patients and decreased in the remaining two. High signal was associated with a high integral dose and a large tumor volume. Tumor shrinkage at the last follow-up examination was more prominent in the patients with symptomatic high signal (p = 0.03). CONCLUSIONS There was a good tumor control rate with a high complication rate. Longer follow up of more patients is needed. Adjusting the dose-volume relationship should be considered to reduce complications.

Gamma knife radiosurgery for brain metastases: prognostic factors for survival and local control

The purpose of this paper was to note a potential source of error in magnetic resonance (MR) imaging. Magnetic resonance images were acquired for stereotactic planning for GKS of a vestibular schwannoma in a female patient. The images were acquired using three-dimensional sequence, which has been shown to produce minimal distortion effects. The images were transferred to the planning workstation, but the coronal images were rejected. By examination of the raw data and reconstruction of sagittal images through the localizer side plate, it was clearly seen that the image of the square localizer system was grossly distorted. The patient was returned to the MR imager for further studies and a metal clasp on her brassiere was identified as the cause of the distortion.A-60-year-old man with medically intractable left-sided maxillary division trigeminal neuralgia had severe cardiac disease, was dependent on an internal defibrillator and could not undergo magnetic resonance imaging. The patient was successfully treated using computerized tomography (CT) cisternography and gamma knife radiosurgery. The patient was pain free 2 months after GKS. Contrast cisternography with CT scanning is an excellent alternative imaging modality for the treatment of patients with intractable trigeminal neuralgia who are unable to undergo MR imaging.The authors describe acute deterioration in facial and acoustic neuropathies following radiosurgery for acoustic neuromas. In May 1995, a 26-year-old man, who had no evidence of neurofibromatosis Type 2, was treated with gamma knife radiosurgery (GKS; maximum dose 20 Gy and margin dose 14 Gy) for a right-sided intracanalicular acoustic tumor. Two days after the treatment, he developed headache, vomiting, right-sided facial weakness, tinnitus, and right hearing loss. There was a deterioration of facial nerve function and hearing function from pretreatment values. The facial function worsened from House-Brackmann Grade 1 to 3. Hearing deteriorated from Grade 1 to 5. Magnetic resonance (MR) images, obtained at the same time revealed an obvious decrease in contrast enhancement of the tumor without any change in tumor size or peritumoral edema. Facial nerve function improved gradually and increased to House-Brackmann Grade 2 by 8 months post-GKS. The tumor has been unchanged in size for 5 years, and facial nerve function has also been maintained at Grade 2 with unchanged deafness. This is the first detailed report of immediate facial neuropathy after GKS for acoustic neuroma and MR imaging revealing early possibly toxic changes. Potential explanations for this phenomenon are presented.In clinical follow-up studies after radiosurgery, imaging modalities such as computerized tomography (CT) and magnetic resonance (MR) imaging are used. Accurate determination of the residual lesion volume is necessary for realistic assessment of the effects of treatment. Usually, the diameters rather than the volume of the lesion are measured. To determine the lesion volume without using stereotactically defined images, the software program VOLUMESERIES has been developed. VOLUMESERIES is a personal computer-based image analysis tool. Acquired DICOM CT scans and MR image series can be visualized. The region of interest is contoured with the help of the mouse, and then the system calculates the volume of the contoured region and the total volume is given in cubic centimeters. The defined volume is also displayed in reconstructed sagittal and coronal slices. In addition, distance measurements can be performed to measure tumor extent. The accuracy of VOLUMESERIES was checked against stereotactically defined images in the Leksell GammaPlan treatment planning program. A discrepancy in target volumes of approximately 8% was observed between the two methods. This discrepancy is of lesser interest because the method is used to determine the course of the target volume over time, rather than the absolute volume. Moreover, it could be shown that the method was more sensitive than the tumor diameter measurements currently in use. VOLUMESERIES appears to be a valuable tool for assessing residual lesion volume on follow-up images after gamma knife radiosurgery while avoiding the need for stereotactic definition.This study was conducted to evaluate the geometric distortion of angiographic images created from a commonly used digital x-ray imaging system and the performance of a commercially available distortion-correction computer program. A 12 x 12 x 12-cm wood phantom was constructed. Lead shots, 2 mm in diameter, were attached to the surfaces of the phantom. The phantom was then placed inside the angiographic localizer. Cut films (frontal and lateral analog films) of the phantom were obtained. The films were analyzed using GammaPlan target series 4.12. The same procedure was repeated with a digital x-ray imaging system equipped with a computer program to correct the geometric distortion. The distortion of the two sets of digital images was evaluated using the coordinates of the lead shots from the cut films as references. The coordinates of all lead shots obtained from digital images and corrected by the computer program coincided within 0.5 mm of those obtained from cut films. The average difference is 0.28 mm with a standard deviation of 0.01 mm. On the other hand, the coordinates obtained from digital images with and without correction can differ by as much as 3.4 mm. The average difference is 1.53 mm, with a standard deviation of 0.67 mm. The investigated computer program can reduce the geometric distortion of digital images from a commonly used x-ray imaging system to less than 0.5 mm. Therefore, they are suitable for the localization of arteriovenous malformations and other vascular targets in gamma knife radiosurgery.

Traumatic pseudoaneurysm of the intracavernous ICA presenting with massive epistaxis: Imaging diagnosis and endovascular treatment

Traumatic pseudoaneurysm of the intracavernous internal carotid artery (ICA) is a very rare cause of epistaxis but is a life‐threatening clinical situation when left untreated. The authors have experienced four cases of traumatic pseudoaneurysm involving the intracavernous ICA. Delayed massive epistaxes developed 1 to 8 months after trauma and initial transient epistaxis in all four patients. Three of the cases were successfully managed by the detachable balloon occlusion (DBO) of the ICA along with the aneurysm openings. In one case, a large pseudoaneurysm destroying a large area of the central skull base with peripheral blood clot was demonstrated on computed tomography, magnetic resonance imaging, and angiography; this patient died due to massive epistaxis before the trial of DBO. Imaging findings of pseudoaneurysms involving the intracavernous ICA in the four cases are described, and the role of endovascular treatment is discussed.

Intraarterial abciximab for treatment of thromboembolism during coil embolization of intracranial aneurysms: outcome and fatal hemorrhagic complications.

OBJECT Experience with intraarterial abciximab for the treatment of thromboembolism during endovascular coil embolization is limited. The authors report the outcome of intraarterial abciximab use, with an emphasis on fatal hemorrhagic complications. METHODS Between March 2003 and May 2006, the authors treated 606 aneurysms by using endovascular coil embolization, and in 32 (5.3%) of these aneurysms (31 patients) an intraarterial thrombus developed. Sixteen of these aneurysms were ruptured and the other 16 were unruptured. Arterial thrombi were totally occlusive in 3 and partially occlusive in the remaining 29 cases. Intraarterial abciximab was administered at a concentration of 0.2 mg/ml as a bolus of 4-15 mg over a period of 15-30 minutes. RESULTS Complete thrombolysis was achieved in 17 (53%) and partial thrombolysis in 15 (47%) of 32 lesions. Twenty-eight patients (90.3%) were asymptomatic after abciximab thrombolysis, but 3 had postprocedural rebleeding that occurred after abciximab treatment; all of these patients had recently experienced an aneurysm rupture. Of these patients, 1 displayed severe thrombocytopenia and the other 2 showed a > 25% reduction in platelet count after abciximab treatment. CONCLUSIONS Intraarterial abciximab is effective for the treatment of thromboembolic complications that occur during intracranial aneurysm coil insertion. Nevertheless, attention should be paid to prevent potentially fatal complications such as thrombocytopenia and hemorrhage, especially in patients with a ruptured aneurysm.