Congenital pseudarthrosis is frequently associated with various stigmata of neurofibromatosis, particularly Cafe-Au-Lait spots and,subcutaneous nodules and in these patients, it is noteworthy that the treatment is very difficult even with extensive operative approach. Also it is interesting to find many controversies on etiological relation between neurofibroma and pseudarthrosis, per se and even the existance of nerve element in and around the tissue of pseudarthrosis. A thirteen years old female patient with definite neurofibromatosis associated with congenital tibial pseudarthrosis treated by intramedullary nailing and massive bone graft is reported.
Cafe-au-Lait Spots ; Christianity ; Female ; Fracture Fixation, Intramedullary ; Humans ; Neurofibroma ; Neurofibromatoses ; Pseudarthrosis ; Transplants

Hemimasticatory spasm is a rare neurologic disorder characterized by unilateral, paroxysmal involuntary contraction of the masticatory muscles. It can be confused with other unilateral facial spasms. In this report, we present two patients with hemimasticatory spasm who were initially misdiagnosed with other disorders. The first patient was a 54-year-old man with a 9-year history of right facial spasm. He underwent microvascular decompression, without benefit, at another hospital with the diagnosis of hemifacial spasm two years prior to presenting at our hospital.On examination, right masseter and temporalis muscles had irregular contractions with twitches and prolonged spasms. His brain magnetic resonance imaging was normal.He showed a good response to carbamazepine. The second case was a 60-year-old man presented with a 10-year history of jaw-closing movement. Previous treatment for temporomandibular joint disorder was ineffective. He was previously diagnosed as oromandibular dystonia at another hospital, and biperiden and diazepam treatment resulted in slight improvement. There was tonic contraction and hypertrophy of the left masseter. He improved with carbamazepine. These cases illustrate the importance of hemimasticatory spasm as differential diagnosis, and the good response to carbamazepine.
Nervous System Diseases

Osteopoikilosis is a familial, rare condition only discovered on radiological examination for a coincident disease. It is characterized by numerous nodules of dense bone in the spongiosa of the metaphysis or in the epiphysial nucleus. They are particularly seen in the small bones of the hands and feet and at the ends of the large bones of the extremities. Most of the bones have been reported as being affected but the skull is rarely involved. This condition is usually symptomless and no specific treatment is necessary. A case of osteopoikilosis is presented with a brief review of literatures.
Extremities ; Foot ; Hand ; Osteopoikilosis ; Skull

Foreatiers disease is a peculiar type of ankylosing hyperostosis of the spine characterized by ossification of the anterior and right lateral aspects of the vertebral column, particularly in the thoracic region. The clinical, pathological and reontgenographic features of the disorder allowed its differentiation from other spinal diseases including ankylosing spordylitis and osteoarthritis. A case of Forestiers disease is presented with brief review of literatures.
Hyperostosis ; Hyperostosis, Diffuse Idiopathic Skeletal ; Osteoarthritis ; Spinal Diseases ; Spine

Renal blood flow was measured by single injection technique of HippuranI13 in 13 patients with chronic glomerulonephritis and 11 control subjects. There was a significant decrease in renal blood flow in chronic glomerulonephritis particulary in those with elevated blood urea nitrogen. Renal blood flow was inversely proportionate to renal vascular resistance but no correlation was noted between renal blood flow and creatinine clearance.
Blood Urea Nitrogen ; Creatinine ; Glomerulonephritis* ; Humans ; Renal Circulation* ; Vascular Resistance

BACKGROUND: Aprotinin, which is a nonspecific serine protease inhibitor, has an antiinflammatory and thrombogenic effect. However, it has an antithrombogenic effect during the cardiopulmonary bypass. This study was performed to evaluated the effects of aprotinin on the activated clotting time(ACT) and the total amount of the heparin used during the cardiopulmonary bypass. MATERIAL AND METHOD: From December 1998 to November 1999, 82 consecutive patients electively underwent open heart surgery at Gachon medical school. The patients were older than 18 years. Eighty two patients were classified into a control group(group C, n=36) and a aprotinin-treated group(group A, n=46). Body weight, height, body surface area(BSA), pump time(PT), aortic cross clamping time(ACCT), and body temperature(BT) were determined. Total amount of heparin and protamine during the CPB were also measured. ACT was determined before heparin administration, at 20, 40 and 60 minutes after heparin administration, and after protamine administration. RESULT: No significant differences were noted in either group in body weight, height, BSA, BT, and the total amoun of heparin and protamine. Group A demonstrated a significant(p<0.05) increase in age, PT, ACCT, and ACT at 20, 40, and 60 minutes after heparin administration. CONCLUSION: In summary, the use of aprotinin prime resulted in an increase in ACT. The total amount of heparin in aproinin-treated patient was similar to that of the control group in spite of having the prolonged pump time. Therefore aprotinin may reduce the requirement of heparin.
Aprotinin* ; Body Height ; Body Weight ; Cardiopulmonary Bypass ; Constriction ; Heart* ; Heparin* ; Humans ; Schools, Medical ; Serine Proteases ; Thoracic Surgery*

In clinical experience, patients complaining of knee joint pain which is not belong to the category of well documented causative diseases, such as rupture of menisci, collateral ligaments and so forth, have been encountered, Since the report of Mayedas chorda cavi articularis genu in 1918, internal derangement of knee due to rupture of plica synovialis has been presented from time to time, Numerous nomenclatures have been offered and various histologic and clinicopathologic features have been observed. Majority of uncomplicated plica synovialis are asymptomatic and respond well to conservative treatment, whereas in complicated cases operation is often inevitable. Here, a symptomatic case, due to rupture of plica synovialis mediopatellaris which was hard to differentiate from rupture of meniscus in clinical aspect and on operation field is reported with available references.
Collateral Ligaments ; Humans ; Knee Joint ; Knee ; Rupture

The prognosis of infectious endophthalmitis has improved considerably in recent years with refinements in early diagnosis, proper antibiotic therapy and vitreous surgery. But the disease is still potentially catastrophic in spite of proper intervention. The purpose of this study was to evaluate the effect of continuous intravitreal irrigation(lavage) after vitrectomy and lensectomy in intractable endophthalmitis. We injected cultured Pseudomonas(1.5 X 10(5)organism/0.1ml) into the anterior chamber or vitreous cavity of 11 rabbits. In both experimental group(8 eyes) and control group(3 eyes), vitrectomy and lensectomy was done 12 hours after inoculation. In the experimental group, silicone tube was installed thru pars plana sclerotomy site as an infusion and drainage port. Vitreous cavity was irrigated for 3 days with normal saline(1000 ml/day) mixed with amikacin (10 mg/day) and dexamethasone(60 mg/day) via this silicone tube. In the control group, we injected 0.1 ml amikacin(100 microgram/0.1 ml) into vitreous cavity at the end of operation and on daily basis for 3 days. Both groups underwent subconjunctival(25 mg/day) and intramuscular (25 mg/kg/day) injection of amikacin for postoperative 3 days. Electroretinograms of two experimental eyes were taken to evaluate retinal toxicity. On postoperative 7th day, all eyes were enucleated to evaluate inflammatory and toxic changes with light microscope. Electroretinograms of experimental eyes became extinguished on the 7th postoperative day. But we observed that the experimental group showed less inflammatory change than control group in the vitreous cavity and retina.
Amikacin ; Anterior Chamber ; Dexamethasone ; Drainage ; Early Diagnosis ; Endophthalmitis* ; Prognosis ; Rabbits ; Retina ; Retinaldehyde ; Silicones ; Vitrectomy

The subretinal neovascularization(SRN) is the leading cause of blindness in various retinal degenerative disease. Yet the mechanism of subretinal new vessel formation is not fully known. We induced SRN using laser photocoagulation in the rabbit and investigated the efficacy of various combinations of laser parameters. In the present study, eighteen eyes of nine pigmented rabbits underwent argon laser photocoagulation, with different settings of power, spot size, and exposure time, Fluorescein angiography was performed 1, 3 and 8 wks after photocoagulation to evaluate the development of SRN, and the light microscopic and electron microscopic examination was done to verify it at the 3rd and 8th week. The mean incidence of SRN was 48.6%, SRN developed with the highest incidence(67%) when using the setting of 1000mW, 0.2 sec, 250 micrometer (P<0.01). Intraoperative retinal hemorrhage increased the incidence of SRN up to 76.4%(P<0.05). Our result provides the most efficient laser parameters to induce experimental SRN in the pigmented rabbits.
Argon* ; Blindness ; Fluorescein Angiography ; Incidence ; Light Coagulation ; Rabbits* ; Retinal Hemorrhage ; Retinaldehyde

The pharmacological treatment of Alzheimer's disease is based on symptomatic therapy of cognitive decline and behavioral problems. Numerous therapies have been investigated for the treatment and prevention of Alzheimer's disease. We reviewed the current evidence-based medical research and guidelines of treatment for Alzheimer's disease. The use of cholinesterase inhibitors (ChEI) and N-methyl-D-aspartate (NMDA) inhibitors can bring about significant but modest therapeutic improvement. There is insufficient evidence to recommend vitamine E, estrogen, ginko biloba, or nonsteroidal anti-inflammatory drugs (NSAIDs) for the prevention or treatment of Alzheimer's disease. This article reviews the available data on current pharmacological treatments through evidence-based medicine.
Alzheimer Disease ; Cholinesterase Inhibitors ; Estrogens ; Evidence-Based Medicine ; Ginkgo biloba ; Memantine ; N-Methylaspartate ; Vitamins