OBJECTIVE: The results of the excision of both outer and inner membranes with fenestration to the basal and parasellar cisterns for symptomatic primary middle cranial fossa arachnoid cysts are presented. METHODS: Twenty-three symptomatic cases of middle cranial fossa arachnoid cyst treated by excision with fenestration from 1993 to 2001 at our hospital were analyzed retrospectively. RESULTS: There was no significant morbidity and mortality after surgery and no recurrence of cyst during the follow-up period(mean 40.8 months). We observed reduction of the cyst with expansion of the surrounding brain and clinical improvement in most of the patients. All cases of type III by Galassi classification, 83% of type II cases and half of type I cases were belonged to the excellent group(reduction of the cyst size over 50% during follow-up period). Seventeen cases(74%) were belonged to the excellent group and 6 cases(26%) were the good group(reduction of the cyst size under 50% during follow-up period). CONCLUSION: The results of this study suggest that the excision and fenestration procedure may be considered as the primary shunt-independent procedure in patients with symptomatic middle cranial fossa arachnoid cyst.
Arachnoid Cysts ; Arachnoid* ; Brain ; Classification ; Cranial Fossa, Middle* ; Follow-Up Studies ; Humans ; Membranes ; Mortality ; Recurrence ; Retrospective Studies

We correct the revised date of this article.

OBJECTIVE: This study was designed to validate the cell trafficking efficiency of the in vivo bioluminescence image (BLI) study in the setting of transplantation of the luciferase expressing bone marrow-derived mesenchymal stem cells (BMSC), which were delivered at each different time after transient middle cerebral artery occlusion (MCAO) in a mouse model. METHODS: Transplanting donor BMSC were prepared by primary cell culture from transgenic mouse expressing luciferase (LUC). Transient focal infarcts were induced in 4-6-week-old male nude mice. The experiment mice were divided into five groups by the time of MSC transplantation : 1) sham-operation group, 2) 2-h group, 3) 1-day group, 4) 3-day group, and 5) 1- week group. BLI for detection of spatial distribution of transplanted MSC was performed by detecting emitted photons. Migration of the transplanted cells to the infarcted area was confirmed by histological examinations. Differences between groups were evaluated by paired t-test. RESULTS: A focal spot of bioluminescence was observed at the injection site on the next day after transplantation by signal intensity of bioluminescence. After 4 weeks, the mean signal intensities of 2-h, 1-day, 3-day, and 1-week group were 2.6x10(7) +/- 7.4x10(6), 6.1x10(6) +/- 1.2x10(6), 1.7x10(6) +/- 4.4x10(5), and 8.9x10(6) +/- 9.5x10(5), respectively. The 2-h group showed significantly higher signal intensity (p < 0.01). The engrafted BMSC showed around the infarct border zones on immunohistochemical examination. The counts of LUC-positive cells revealed the highest number in the 2-h group, in agreement with the results of BLI experiments (p < 0.01). CONCLUSION: In this study, the results suggested that the transplanted BMSC migrated to the infarct border zone in BLI study and the higher signal intensity of LUC-positive cells seen in 2 hrs after MSC transplantation in MCAO mouse model. In addition, noninvasive imaging in real time is an ideal method for tracking stem cell transplantation. This method can be widely applied to various research fields of cell transplantation therapy.
Animals ; Cell Transplantation ; Enzyme Multiplied Immunoassay Technique ; Humans ; Infarction, Middle Cerebral Artery ; Luciferases ; Male ; Mesenchymal Stromal Cells ; Mice ; Mice, Nude ; Mice, Transgenic ; Photons ; Primary Cell Culture ; Stem Cell Transplantation ; Stroke ; Tissue Donors ; Track and Field ; Transplants

A rare case of spontaneous subarachnoid hemorrhage from newly developed cerebral aneurysm in glioblastoma patient is presented. A 57-year-old man was presented with headache and memory impairment. On the magnetic resonance image and the magnetic resonance angiography, a large enhancing mass was found at right frontal subcortex and intracranial aneurysm was not found. The mass was removed subtotally and revealed as glioblastoma. He took concurrent PCV chemotherapy and radiation therapy, but the mass recurred one month later after radiotherapy. He was then treated with temozolomide for 7 cycles. Three months after the completion of temozolomide therapy, he suffered from a subarachnoid hemorrhage due to a rupture of a small de novo aneurysm at distal anterior cerebral artery. He underwent an aneurysm clipping and discharged without neurologic complication.
Aneurysm ; Anterior Cerebral Artery ; Dacarbazine ; Glioblastoma ; Headache ; Humans ; Intracranial Aneurysm ; Magnetic Resonance Angiography ; Magnetic Resonance Spectroscopy ; Memory ; Middle Aged ; Rupture ; Subarachnoid Hemorrhage

Follow-Up Studies ; Fracture Fixation ; Humans ; Malocclusion ; Mandible ; Recurrence

PURPOSE: The prevalence of rickets in the world is on the rise not only in developing but also in developed countries. In Korea, breastfeeding has increased. There have been few studies on the possible association of rickets with breastfeeding. The purpose of this study was to identify the development and the clinical presentation of subclinical rickets in breastfed infants. METHODS: We investigated patients who were breastfed and had hypovitaminosis D in the blood from May 2006 to April 2007, and who were diagnosed with vitamin D deficient rickets from May 2003 to April 2006. We evaluated the results of blood tests, x-rays and other relevant information in the medical record. A questionnaire that included questions on the diet of patients, the mothers activity during pregnancy and place of residence was administered. RESULTS: Twelve patients (66%: male, 34%: female) were enrolled in this study. There were eight in the asymptomatic and four in the symptomatic group. The median age for each group was 8 months (range 4-11 month) and 5.5 months (5-8 month). All patients in the symptomatic group were breastfed until diagnosed. In the asymptomatic group, they were breastfed for four to six months, and then weaned with only thin rice soup and vegetables. Nine patients had a vitamin D concentration below 20 ng/mL and three patients had levels between 20 and 29 ng/mL. Alkaline phosphatase (ALP) and parathyroid hormone (PTH) were elevated in both groups. There were statistically significant 25-OHD3 levels in the blood in both groups (P=0.008). Ten of the patients (83%) also had iron deficient anemia. CONCLUSION: Vitamin D deficiency and subclinical rickets has been identified in Korea. However, the prevalence of this disease has not been determined. The main limitation of this study was the small number of patients and the absence of a control group.
Alkaline Phosphatase ; Anemia ; Breast Feeding ; Developed Countries ; Diet ; Hematologic Tests ; Humans ; Infant* ; Iron ; Korea ; Male ; Medical Records ; Milk, Human ; Mothers ; Parathyroid Hormone ; Pregnancy ; Prevalence ; Surveys and Questionnaires ; Rickets* ; Vegetables ; Vitamin D ; Vitamin D Deficiency

Hemangioblastoma is uncommon, accounting for 1 to 2.5% of all primary neoplasms of the central nervous system. Ten to 20% of hemangioblastomas occur as part of Von Hippel-Lindau disease(VHL). Multiple hemangioblastomas are seen only with VHL and these are seen in up to half of VHL-associated hemangioblastomas. We have treated two cases of multiple hemangioblastomas associated with VHL disease in a family, sister(case I, 48/female) and her brother(case II, 41/male). Both patients had renal and pancreatic cysts in addition to CNS hemangioblastomas. Their hemangioblastomas were removed totally, and then their neurological symptoms had Improved. With a review of the literature, the authors present a family of VHL-associated multiple hemangioblastomas.
Central Nervous System ; Hemangioblastoma* ; Humans ; Pancreatic Cyst

Erythromelalgia is a rare clinical condition of unknown etiology characterized by severe burning pain in the distal limbs. It can be accompanied by pronounced erythema and increased skin temperature precipitated by heat or activity and can be improved by cooling the affected part. It can be divided into two categories, primary, which begins spontaneously at any age, and secondary, which is infrequently associated with diabetes mellitus. However, the significance of this association is little known. We report a rare case of intractable erythromelalgia in an adolescent with diabetes mellitus.
Adolescent* ; Burns ; Diabetes Mellitus* ; Erythema ; Erythromelalgia* ; Extremities ; Hot Temperature ; Humans ; Skin Temperature

OBJECTIVES: To determine the presentation, incidence, and risk factors of seizures in patients treated for brain tumors. METHODS: One hundred patients who consecutively underwent a craniotomy for the treatment of supratentorial brain tumors were assessed. The pathologies of the patients enrolled in the study included glioma (n=56), meningioma (n=31), metastatic brain tumor (n=7), primary central nervous system lymphoma (n=4), and central neurocytoma (n=2). Anti-epileptic drugs (AEDs) were administered to all patients for up to six months after the surgery. Pre-defined variables for outcome analysis included tumor grade and location, extent of tumor resection, number of seizures, age at tumor diagnosis, adjuvant therapy, medication and radiological abnormalities. RESULTS: Thirty patients (30%) presented at least a single episode of seizure at the time of admission. Five of these patients (16.7%) developed the seizure during the follow-up period. Newly developed seizure was noticed in six out of seventy patients (8.6%) without prior seizure. Histopathology was malignant gliomas in 10 and supratentorial meningioma in one. Early seizure developed only in two patients. CONCLUSION: Compared with patients without seizure, patients with seizure at the time of admission showed younger age (p=0.003), a higher portion of low-grade glioma (p=0.001), tumor location in the frontal and temporal lobes (p=0.003) and cortical involvement (p=0.017). Our study suggestes that tumor progression is considered a significant risk factor for seizure development in glioma patients.
Brain Neoplasms* ; Brain* ; Central Nervous System ; Craniotomy ; Diagnosis ; Follow-Up Studies ; Glioma ; Humans ; Incidence ; Lymphoma ; Meningioma ; Neurocytoma ; Pathology ; Risk Factors ; Seizures* ; Temporal Lobe

Menkes disease, so called kinky-hair syndrome, is a rare, genetic and progressive neurodegenerative disorder. It is caused by a mutation in the ATP7A gene, which codes for the copper transporting ATPase in the cell organelles. The dysfunction of many copper-dependent enzymes results in low concentration of copper in some tissues and accumulation of copper in others. We report a boy presented with kinky hairs, developmental delay, hypotonia and connective tissue abnormalities at the age of 4 months. Despite the treatment with various antiepileptic drugs, atonic seizures still persisted. At the age of 7 months, his atonic seizures was changed into extensor spasms with modified hypsarrhythmia for some years. The seizure were controlled by topiramate and vigabatrin. At the age of 22 months, serum copper and ceruloplasmin rechecked as 17 ug/dL(80-150 ug/dL) and 7.3 mg/dL(20-46 mg/ dL) respectively. The gene study showed ATP7A mutation and the patient was diagnosed as Menkes disease so that copper-histidine was daily injected. We experienced a case of a 4-month-old boy with Menkes disease and infantile spasm, confirmed by ATP7A mutation.
Adenosine Triphosphatases ; Anticonvulsants ; Ceruloplasmin ; Connective Tissue ; Copper ; Hair ; Humans ; Infant ; Infant, Newborn ; Male ; Menkes Kinky Hair Syndrome* ; Muscle Hypotonia ; Neurodegenerative Diseases ; Organelles ; Seizures ; Spasm ; Spasms, Infantile* ; Vigabatrin