BACKGROUND: "Rebuild-up" phenomenon, induced by hyperventilation, is a characteristic finding on EEG in children with Moyamoya disease. Its mechanism, however, remains obscure. In this study, we examined the relationship between cerebral lesions on MRI, stenosis or occlusion of cerebral vessel on cerebral angiography, and EEG findings in children with Moyamoya disease. METHODS: We have reviewed medical records of 33 patients, who were confirmed as Moyamoya disease by cerebral angiography at Asan Medical Center. EEG and brain MRI were carried out in all subjects. RESULTS: 1) Epidemiologic data were : the male to female ratio was 1:1.1; highest rate(90.7%) of onset in age group below 10 years; mean age at clinical onset was 7.4 years; average diagnostic interval from clinical onset to diagnosis was 1.9 years. 2) The most common initial and recurrent or residual symptoms were motor deficit. 3) The most common site of occlusion or stenosis of cerebral vessel on cerebral angiography was anterior cerebral area(>97%) and the most common cerebral infarction area on brain MRI was anterior cerebral area, too. 4) The hyperventilation(for 3 minutes) on EEG were carried out in 25 patients and the prolonged build-up or rebuild-up phenomenon was observed in 13 patients(52%). 5) The prolonged build-up or rebuild-up phenomenon on EEG was observed in 6 of 15 patients who were occlusion of cerebral vessel, and in 7 of 10 patients who were stenosis of cerebral vessel on angiography. CONCLUSION: 1) The background slowings on EEG maybe suggestive of the infarction stage of Moyamoya disease in children. 2) The prolonged build-up or rebuild-up phenomenon might indicate the preinfarction stage of Moyamoya disease in children.
Angiography ; Brain ; Cerebral Angiography ; Cerebral Infarction ; Child ; Chungcheongnam-do ; Constriction, Pathologic ; Diagnosis ; Electroencephalography ; Female ; Humans ; Hyperventilation ; Infarction ; Magnetic Resonance Imaging ; Male ; Medical Records ; Moyamoya Disease*

From Jan. 1987 to May 1994, we carried out cementless total hip arthroplasties using hydroxyapatite-coated Omnifit system, and among these, 63 cases in 49 patients were followed up more than 24 months. The average Harris Hip Score was 46.3 points preoperatively, 95.4 points at POD 1 year and 96.2 points at POD 2 years. Two patients (3%) complained thigh pain at POD 2 years. On the radiologic findings, endosteal bone formation was noted in 34 cases (53,9 %), radiolucent lines were seen at the non-coating region of distal stem in 41 cases (65.3%), which were less than 2mm and not progressive. Cortical hypertrophy was noted in 5 cases (8.4%), and calcar atrophy was observed in 31 cases (49 %).
Arthroplasty ; Arthroplasty, Replacement, Hip* ; Atrophy ; Durapatite* ; Hip ; Humans ; Hypertrophy ; Osteogenesis ; Thigh

No abstract available.
Biopsy, Fine-Needle* ; Diagnosis, Differential* ; Radionuclide Imaging* ; Thyroid Gland* ; Thyroid Nodule* ; Ultrasonography*

OBJECTIVE: Self-expanding stents are increasingly used for the treatment of complex intracranial aneurysms. The purpose of this study was to evaluate the usefulness and safety of a self-expanding nitinol stent (Enterprise) in the treatment of wide-necked intracranial aneurysms. METHODS: This was a retrospective study of 39 patients with 40 wide-necked intracranial aneurysms who were enrolled in a single-center registry of patients treated with the Enterprise between June 2009 and December 2011. Thirty patients were asymptomatic, four had cerebrovascular accident sequelae, and five had suffered subarachnoid hemorrhage. One aneurysm had reopened after prior coil embolization, while 39 had not been treated. Clinical charts, procedural data, and angiographic results, including both immediate post-procedural angiograms and follow-up imaging, were reviewed. RESULTS: The mean neck size of the aneurysms was 5.58 mm (range 3-15.1 mm). Embolization was successful in all patients. There were five procedure-related events. There were no fatalities, but one procedure-related morbidity was noted. The immediate angiographic results included eight complete occlusions (20%), six remnant necks (15%), and 26 remnant sacs (65%). At angiographic follow-up (mean: 11.3 months), out of 18 of the aneurysms treated with stent-assisted coiling, there were 13 (72.2%) complete occlusions, four (22.2%) remnant necks, and one recanalization (5.6%). CONCLUSION: Stent-assisted coiling using the Enterprise is effective for the treatment of wide-necked intracranial aneurysms. Further angiographic and clinical follow-up investigation will be needed for evaluation of the long-term outcomes.
Aneurysm* ; Embolization, Therapeutic ; Follow-Up Studies ; Humans ; Intracranial Aneurysm* ; Neck ; Retrospective Studies ; Stents* ; Stroke ; Subarachnoid Hemorrhage

No abstract available.
Age Factors* ; Hematoma, Subdural, Chronic*

No abstract available.
Aneurysm* ; Arteries*

No abstract available.
Child* ; Gastroenteritis* ; Humans* ; Infant*

Canavan disease(CD) is a rare autosomal recessive leukodystrophy caused by the deficiency of aspartoacylase and the accumulation in brain of N-acetylaspartate(NAA). CD has been reported mainly Ashkenazi Jews but also occurs in other ethnic groups. Usually it presents as early as the third month of life with megalencephaly, hypotonia later progressing to hypertonia, psychomotor and mental retardation, blindness, occasionally deafness and seizure. Diagnosis is based on the clinical feature, N-acetylaspartic aciduria, radiologic and pathologic findings. Histologically, the affected white matter shows extensive vacuolation and demyelination. There is no treatment for CD and the only prevention is through genetic counselling and prenatal diagnosis. We experienced a case of Canavan disease that was presented with hypotonia and developmental delay. Diagnosis was confirmed histologically. Radiologic findings are extensive high signal throughout the white matter on T2-weighted MRI and increased NAA peak and decreased choline peak of the white matter on MR spectroscopy.
Blindness ; Brain ; Canavan Disease* ; Choline ; Deafness ; Demyelinating Diseases ; Diagnosis ; Ethnic Groups ; Humans ; Intellectual Disability ; Jews ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Muscle Hypotonia ; Prenatal Diagnosis ; Seizures

No abstract available.
Acidosis*

Researchers have an ethical responsibility to report the results of research involving human subjects. Dissemination of results ensures that patient care is based on good science and that the field of medicine advances based on complete and accurate knowledge. However, current evidence suggests that publication is often neglected or substantially delayed, especially in the case of negative and inconclusive results. Researchers, editors and reviewers should value all high-quality research regardless of the conclusiveness of the results and ensure that all research involving human subjects is registered in a publicly accessible database.
Clinical Trials as Topic/*ethics ; *Ethics, Research ; Human Experimentation/*ethics ; Publishing/*ethics ; Republic of Korea ; *Research Report ; Research Subjects