Moyamoya disease is a rare occlusive cerebrovascular disease characterized by stenosis or occlusion of the main cerebral arteries. It has a tendency for multifactorial inheritance and familial occurrence, although its pathogenesis is not clear. We observed this disease in two girls from the same family:one was eight years old and the other was 45 months. They presented with transient ischemic attacks. We performed cerebral angiography on both patients and magnetic resonance angiography (MRA) on the younger. Both approaches showed the typical features of moyamoya disease, and MRA successfully revealed abnormal findings specific for the disease in the second child. Both children received encephaloduroarteriosynangiosis (EDAS) and this produced good results. MRA is thus a powerful and noninvasive way of detecting individuals at high risk of developing this disease. Considering the reported familial incidence of moyamoya disease in Japan, a careful search for family members using MRA would probably reveal many more such cases in Korea.
Cerebral Angiography ; Cerebral Arteries ; Child ; Constriction, Pathologic ; Female ; Humans ; Incidence ; Ischemic Attack, Transient ; Japan ; Korea ; Magnetic Resonance Angiography ; Moyamoya Disease* ; Multifactorial Inheritance

The widespread use of diagnostic radiography, especially using magnetic resonance imaging, has helped to increase the diagnosis of paralabral cysts in patients with chronic shoulder pain. These paralabral cysts are frequent in the anterior, the superior, and the posterior compartment of the shoulder joint but are rare in the inferior compartment. Paralabral cysts in the shoulder appear particularly in men in their third and fourth decades but rarely in elderly patients. We report a case of an inferior paralabral cyst in an elderly patient whom we treated through arthroscopic decompression.
Aged* ; Arthroscopy ; Decompression* ; Diagnosis ; Humans ; Magnetic Resonance Imaging ; Male ; Radiography ; Shoulder Joint ; Shoulder Pain ; Shoulder*

No abstract available.
Fibroblasts*

A case or right occipital lobe infarction initially detected by the visual field examination and confirmed with the computerized tomography is presented. Reduction of significance of visual field test for identification of intracranial lesion as, the initial diagnostic procedure after the orientation of computerized tomography is unlikely. An occipital lobe lesion developed following posterior cerebral artery thrombosis has introd uced silgnificant ophthalmic symptom, namey, visual field defect, diplopia, ipsilateral ocular pain, visual hallucination, photophobia, and Anton's syndrom, etc. A characteristic feature of left homonymous hemianopsia with macular sparing due to right posterior cerebral artery thromb osis is demonstrated.
Diplopia ; Hallucinations ; Hemianopsia ; Infarction* ; Occipital Lobe* ; Photophobia ; Posterior Cerebral Artery ; Thrombosis ; Visual Field Tests ; Visual Fields

BACKGROUND: In the emergency department, the diagnosis of ischemic stroke is difficult because the diagnostic modalities are limited to non-contrast brain CT and neurologic examination. Serum S100B protein, a bio-marker for ischemic stroke, is needed as an additional diagnostic aid in acute ischemic stroke. METHODS: We retrospectively reviewed 50 patients diagnosed with ischemic stroke between August 2007 and December 2008 by brain MRI after brain CT and serum S100B measurement in the emergency department. The serum levels of S100B protein were analyzed and the diagnostic sensitivity of non-contrast brain CT combined with abnormal elevation of S100B protein was compared with that of non-contrast brain CT alone. RESULTS: The overall sensitivity of non-contrast brain CT in the diagnosis of ischemia was 54%. S100B protein in early ischemia had a sensitivity of 58%. However, combining non-contrast brain CT and S100B increased the sensitivity to 74%. CONCLUSIONS: A biomarker-based diagnostic test would not replace the necessity for CT or other early imaging studies, and before contemplating any reperfusion strategy, neuro-imaging must be performed to rule out intracranial hemorrhage. However, S100B protein, a serum bio-marker, is able to help emergency physicians evaluate patients with suspected ischemic stroke and decide on treatment.
Brain ; Diagnostic Tests, Routine ; Early Diagnosis ; Emergencies ; Humans ; Intracranial Hemorrhages ; Ischemia ; Nerve Growth Factors ; Neurologic Examination ; Reperfusion ; Retrospective Studies ; S100 Proteins ; Stroke

Maffucci syndrome is a rare, congenital, and nonhereditary syndrome characterized by the occurrence of multiple enchondromas and hemangiomas. According to previous studies, patients diagnosed with Maffucci syndrome have a higher risk of developing malignant tumors, such as ovarian cancer, pancreatic cancer, breast cancer, and central nervous system cancer, in addition to malignant transformations of enchondromas. The authors report a case of a 37-year-old male patient with Maffucci syndrome who presented with multiple enchondromas and hemangiomas. This patient’s presentation and management are discussed, along with a review of the literature. Maffucci syndrome should be expected when encountering a patient with multiple enchondromas and suspected hemangiomas in the limbs. Even if benign lesions such as enchondromas or hemangiomas are diagnosed, regular follow-up is required to monitor for malignant transformations and the development of malignant tumors in other organs.

BACKGROUND: Bone loss after liver transplant (LT) is a long-term problem associated with an increased morbidity due to pathologic fractures. We reviewed our management of post-LT bone loss. METHODS: We collected retrospective data from 82 adult LT recipients between January 2006 and December 2009 who had preoperative and postoperative (12 to 24 months) bone mineral density (BMD) data measured by dual energy X-ray absorptiometry (DXA). BMD was decreased in 52 out of 82 patients before LT. These patients were managed with calcium plus alendronate, calcium only, or no treatment. We compared the efficacy of these three modalities and the factors influencing BMD changes and investigated the incidence of pathologic fractures. RESULTS: In decreased BMD patients (n=52), the postoperative spinal BMD was increased with all three treatment modalities. A more significant increase was found with ALN treatment (+0.103) compared to NO treatment (+0.029) (P-value: 0.016). However, femoral BMD decreased despite ALN treatment. Alendronate use was a significant factor for post-LT spinal BMD improvement in the univariate and multivariate analysis. There were significant newly-developed pathologic fractures after LT especially in osteoporotic patients (28%). CONCLUSIONS: Weekly alendronate with daily calcium may be helpful for the spinal bone mineral protection in preoperative patients with decreased BMD.
Absorptiometry, Photon ; Adult ; Alendronate ; Bone Density ; Calcium ; Fractures, Spontaneous ; Humans ; Incidence ; Liver ; Liver Transplantation ; Multivariate Analysis ; Osteoporosis ; Retrospective Studies ; Transplants

BACKGROUND: Bone loss after liver transplant (LT) is a long-term problem associated with an increased morbidity due to pathologic fractures. We reviewed our management of post-LT bone loss. METHODS: We collected retrospective data from 82 adult LT recipients between January 2006 and December 2009 who had preoperative and postoperative (12 to 24 months) bone mineral density (BMD) data measured by dual energy X-ray absorptiometry (DXA). BMD was decreased in 52 out of 82 patients before LT. These patients were managed with calcium plus alendronate, calcium only, or no treatment. We compared the efficacy of these three modalities and the factors influencing BMD changes and investigated the incidence of pathologic fractures. RESULTS: In decreased BMD patients (n=52), the postoperative spinal BMD was increased with all three treatment modalities. A more significant increase was found with ALN treatment (+0.103) compared to NO treatment (+0.029) (P-value: 0.016). However, femoral BMD decreased despite ALN treatment. Alendronate use was a significant factor for post-LT spinal BMD improvement in the univariate and multivariate analysis. There were significant newly-developed pathologic fractures after LT especially in osteoporotic patients (28%). CONCLUSIONS: Weekly alendronate with daily calcium may be helpful for the spinal bone mineral protection in preoperative patients with decreased BMD.
Absorptiometry, Photon ; Adult ; Alendronate ; Bone Density ; Calcium ; Fractures, Spontaneous ; Humans ; Incidence ; Liver ; Liver Transplantation ; Multivariate Analysis ; Osteoporosis ; Retrospective Studies ; Transplants

Hamartomatous polyp is an uncommon lesion. Most of the gastric hamartomatous polyps occur in patients in their 40s because of polyposis coli. Gastric hamartomaotus polyps are usually asymptomatic, but they occasionally manifest with hematemesis or anemia. If they are congenital, then sessile polyps in children should be observed with some measurable frequency. However, many reports have revealed that if the polyp size is big and the risk of rebleeding is high, then ESD or surgical resection can be used to completely remove them. An inverted gastric hamartomatous polyp may have an early gastric cancer component. We report here on an unusual polyp in a 14-years male who presented with hematemesis. Those were about two 3 cm sized large polyps at the proximal antrum and mid body of the stomach, respectively. The polyps were successfully removed by an endoscopic mucosal resection and the lesions were diagnosed as gastric harmatomas.
Anemia ; Child ; Hematemesis ; Hemorrhage ; Humans ; Male ; Polyps ; Stomach ; Stomach Neoplasms

Weber-Christian disease (WCD) is an inflammatory disease of subcutaneous fat tissue which is characterized by relapsing, febrile tender nodules and histologically lobular panniculitis. Any area of the body containing fat can be affected by WCD. Several cases of WCD have been reported as involvement of the heart, lung, liver and kidney. Acute illness (ex. acute myocardiac infarction, infection, etc) can be complicated with stress-related hyperglycemia and increase insulin restistance. Finally this case show that Weber-Christian disease accompanied by marked hyperglycemia and insulin resistance, which resolved soon after corticosteroid.
Heart ; Humans ; Hyperglycemia* ; Infarction ; Insulin ; Insulin Resistance ; Kidney ; Liver ; Lung ; Panniculitis ; Panniculitis, Nodular Nonsuppurative* ; Subcutaneous Fat