No abstract available.
Cranial Irradiation ; Leukoencephalopathies* ; Parkinsonian Disorders*

Pituitary tumors occur in 15-50% of patients with multiple endocrine neoplasia of type 1 (MEN1). To the best of our knowledge, no MEN1 case in which the initial lesion was an invasive giant prolactinoma has been reported from Korea. We describe a patient in whom a skull-base tumor involved the sellar and parasellar spaces. A 49 year-old female presented with headache and diplopia. The tumor was ultimately identified as a giant prolactinoma; the serum prolactin concentration increased from 155.6 ng/mL to 3,234.3 ng/mL after cranial irradiation. She was evaluated in terms of incidental hypercalcemia and was found to have parathyroid hyperplasia. Genetic analysis revealed a missense mutation in the MEN1 gene (c.643G>A, p.Val215Met). Two years of treatment with a dopamine agonist reduced, but did not normalize, the serum prolactin concentration. We highlight the aggressive behavior of the giant skull-base tumor, and the diagnostic delay caused by a high-dose hook effect of the MEN1-related prolactinoma.
Cranial Irradiation ; Diplopia ; Dopamine Agonists ; Female ; Headache ; Humans ; Hypercalcemia ; Hyperplasia ; Korea ; Multiple Endocrine Neoplasia Type 1* ; Multiple Endocrine Neoplasia* ; Mutation, Missense ; Pituitary Neoplasms ; Prolactin ; Prolactinoma*

No abstract available.
Child* ; Cranial Irradiation* ; Humans ; Precursor Cell Lymphoblastic Leukemia-Lymphoma*

We have treated adult acute leukemia 64 patients between January 1990 and October 1991 at the Chungnam National University Hospital. They were examined for the impact of presenting WBC count on the initial course and from them we have chosen twenty patients whose leukocyte count is over one hundred thousands per cubic milimeter. We divided the twenty patients into 4 groups on the base of treatment modalities: conservative therapy only, chemotherapy only, cranial irradiation only, and chemotherapy with cranial irradiation. Early sudden death rate is lower in cranial irradiation with/without chemotherapy groups than the conservative only or chemotherapy only patients. Also the remission rate is high in cranial irradiation with chemotherapy patients. Therefore we suggest that the rapid intervention of cranial irradiation in adult acute leukemia could be helpful in reducing the early sudden death rate and perhaps in increasing the remission rate.
Adult* ; Chungcheongnam-do ; Cranial Irradiation* ; Death, Sudden ; Drug Therapy ; Emergencies* ; Humans ; Leukemia* ; Leukocyte Count ; Leukocytosis*

Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.
Cranial Irradiation ; Down Syndrome ; Humans ; Moyamoya Disease* ; Natural History ; Neurofibromatoses ; Neurofibromatosis 1 ; Thyroid Diseases

PURPOSE: To evaluate the MR imaging findings and the usefulness of MR imaging in the diagnosis and followup of leukoencephalopathy following CNS prophylaxis therapy in pediatric leukemia. MATERIALS AND METHODS: We retrospectively evaluated the MR imaging findings of eight children with white matter abnormalities on MR out of seventeen acute leukemic patients with various neuropsychiatric symptoms who received intrathecal methotrexate administration, with or without cranial irradiation. In all cases, initial MR was performed within a week of the onset of neuropsychiatric symptoms. Follow-up MR was performed one to sixteen months after initial study, and the MR imaging findings were compared with the initial findings. RESULTS: The initial MR imaging findings were classified into three categories: focal or multifocal white matter abnormalities (3/8), and diffuse white matter abnormalities without enhancement (3/8), and diffuse white matter abnormalities with enhancement (2/8). At follow-up MR, diffuse or focal atrophic changes were noted in all children. White matter abnormalities improved in two out of three patients with focal or multifocal white matter abnormalities. In five with diffuse white matter abnormalities, the extent of these showed no significant change, but contrast enhancement was markedly reduced in two children in whom diffuse white matter abnormalities with enhancement had been demonstrated. CONCLUSION: In pediatric leukemia, the MR imaging findings of leukoencephalopathy following CNS prophylaxis therapy are variable, but are specific with the clinical history of neuropsychiatric symptoms after intrathecal methotrexate administration, with or without cranial irradiation. The MR imaging is valuable in the diagnosis and follow-up of leukoencephalopathy following CNS prophylaxis therapy in pediatric leukemia.
Child ; Cranial Irradiation ; Diagnosis ; Follow-Up Studies ; Humans ; Leukemia* ; Leukoencephalopathies* ; Magnetic Resonance Imaging* ; Methotrexate ; Retrospective Studies

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.
Cerebral Angiography ; Child ; Cranial Irradiation ; Craniopharyngioma ; Follow-Up Studies ; Humans ; Ischemic Attack, Transient ; Moyamoya Disease

PURPOSE: Acute leukemia with hyperleukocytosis (more than 105/mm3) is at high risk of early sudden death, usually from intracerebral hemorrhage. Emergency cranial irradiation is a relatively simple approach to solve this the problem. We summarized our experience of cranial irradiation in 24 leukemic children who presented with hyperleukocytosis. METHODS AND MATERIALS: Between 1990 and 1998, 40 children with acute leukemia presenting with hyperleukocytosis were referred for emergency cranial irradiation. Among these patients, 24 children were evaluable. There were 16 boys and eight girls, their ages ranged from 2 to 13 years (median 9.5 years). The initial leukocyte counts ranged 109,910/mm3 to 501,000/mm3. Peripheral blood smear was performed in all patients and noted the morphology of the blast. Introduction of emergency cranial irradiation was determined by the leukocyte counts (more than 100,000/mm) and the existence of the blast in peripheral blood smear. All patients were treated with intravenous hydration with alkaline fluid and oral allopurinol. Cranial irradiation started on the day of diagnosis. With 2 Gy in one fraction in 4 patients, 4 Gy in two fractions in 20 patients. RESULTS: The WBC count had fallen in 19 patients (83%) and no intracerebral hemorrhage occurred after irradiation. There were five cases of early deaths. Four patients died of metabolic complications, and one patient with intracerebral hemorrhage. He died 5 hours after cranial irradiation. No patient had any immediate side effect from cranial irradiation. CONCLUSION: Our data suggest, that emergency cranial irradiation can be safely chosen and effective in childhood leukemic patients presenting with high leukocyte counts.
Allopurinol ; Cerebral Hemorrhage ; Child ; Cranial Irradiation* ; Death, Sudden ; Diagnosis ; Emergencies ; Female ; Humans ; Leukemia ; Leukocyte Count

OBJECTIVE: We investigated the morphologic changes within 24 hours after a single gamma-irradiation in the rat brain. METHODS: Forty Sprague-Dawley rats were used. After a burr hole trephination on right parietal area, cerebral hemisphere was irradiated with 2Gy and 5Gy using iridium-192(192Ir), respectively. The effect was assessed at 4, 8, 12 and 24 hours after irradiation. The histological changes were scored following the detection of edema or disarray severity. TUNEL-positive cells exhibiting apoptotic morphology were counted in irradiated region. RESULTS: Cortical edema and disarray were initially showed at 4 or 8hour and almost all defined at 24hour after irradiation. And the injury was wedge shape. TUNEL-positive cells were minimal at 8hour after irradiation as the number of positive cells were 2.6+/-5.27(n=5) after 2Gy, and 0.8+/-0.84(n=5) after 5Gy. But, the number of apoptotic cells were increased markedly to 60+/-6.24 at 12hour after 2Gy and to 104+/-19.7 at 24hour after 5Gy. CONCLUSION: There were prominent morphologic changes immediately after gamma-irradiation. And, apoptosis was increased according to the time period. These findings implicate that brain irradiation induces rapid apoptotic change, which may play an important role in the pathogenesis of radiation-induced pathologic conditions.
Animals ; Apoptosis ; Brain* ; Cerebral Cortex* ; Cerebrum ; Cranial Irradiation ; Edema ; Rabeprazole ; Radiation Effects* ; Radiation Injuries ; Rats* ; Rats, Sprague-Dawley ; Trephining

We report three cases of patient with pleomorphic xanthoastrocytoma(PXA). PXA is a clinicopathologically distinct variant of cerebral astrocytoma which is a low-grade leptomeningeal glioma affecting under the age of 30 years. This tumor has a favorable prognosis, but histological and neuroradiological findings suggest malignant brain tumor. Occasionally these may be confused with malignant gliomas. Clinical features from our three patients were manifestation of increased intracranial pressure and all cases were under the age of 30 years and females. Two patients had superficial location in the right frontal and left temporal respectively, but remaining one(case 2) which was associated with arteriovenous malformation in the left frontal region was in the right lateral ventricle. The intraoperative histological evidence by frozen biopsy suggested glioblastoma or anaplastic astrocytoma in all cases which confirmed to be PXA by postoperative histological examination. Two cases which had been resected subtotally were followed by cranial irradiation without therapeutic efficacy. Optimal management of PXA is primary surgical resection to reduce recurrence of tumor and to secure long-term survival. Therefore, differentiation of PXA from malignant brain tumors and intraoperative diagnosis of PXA is imperative to treat PXA effectively.
Arteriovenous Malformations ; Astrocytoma ; Biopsy ; Brain Neoplasms ; Cranial Irradiation ; Diagnosis ; Female ; Glioblastoma ; Glioma ; Humans ; Intracranial Pressure ; Lateral Ventricles ; Prognosis ; Recurrence