Marfan Syndrome is a rare congenital disease characterized by the abnormalities of Sheletal system, eye and cardio-vascular system which was initially desiribed by Marfan in 1896. One case of marfan syndrome was treated at the department of Orthopedic Surgery, College of Medicine, Seoul National University for multiple skeletal deformities. Moderate degree of scoliosis was treated with Milwaukee brace and finger deformities with Z-plasty and full thickness skin graft.
Braces ; Congenital Abnormalities ; Fingers ; Marfan Syndrome ; Orthopedics ; Scoliosis ; Seoul ; Skin ; Transplants

Two hundreds cases of spine tuberculosis were treated surgically for the period of 7 years between 1970 and 1976 at Seoul National University Hospital and following results were obtained. 1. Many patients failed to be treated with conservative method. Spontaneous fusion was found in 10% of 200 cases with long duration of disease. Anterior fusion was performed in 166 cases and posterior fusion was carried out in 34 cases. In those 34 cases, anterior currettage was combined in 7 cases, anterior fusion combined in 19 cases in which 7 cases were performed anterior and posterior fusion at one stage. Posterior fusion alone was carried out only in 8 cases. 2. Anterior fusion is indicated for: a. decompression in cord involvement b. confirmation of diagnosis c. involvement of one joint space in children d. mild to moderate kyphosis in adult 3. Anterior and posterior fusion is indicated in: a. severe kyphosis for solid funsion b. involvement of more than two joint spaces in children to prevent increasing kyphosis c. one stage operation was technically possible and has many advantages 4. Posterior fusion is indicated in: a. poor general condition b. technically difficult cases.
Adult ; Child ; Decompression ; Diagnosis ; Humans ; Joints ; Kyphosis ; Methods ; Seoul ; Spine ; Tuberculosis

One case of osteoid osteoma in the lamina of the fifth lumbar spine with hamstring muscle tightness was experienced and successfully treated by surgery at the department of orthopedic surgery in Seoul National University Hospital. Literature on the subject was reviewed in detail.
Muscle Tonus ; Orthopedics ; Osteoma, Osteoid ; Seoul ; Spine

The term hydranencephaly designates the condition in which large protions of the cerebral hemispheres have been reduced to membranous sacs within a cranium of relatively normal size. Only basal ganglia and remnants of the mesencephalon are found at the base of the skull. We have experienced the 2 cases of infantile hydranencephaly. In surveying the pathogenesis of the hydranencephaly, it becomes apparent that the distribution of most of the lesions falls into certain distinct patterns, which is characterized by almost completely absent of both cerebral hemispheres and by relative gross preservation of the basal ganglia, a portion of lower temporal and occipital and contents of the posterior fossa in our experienced 2 cases. In our cases, the condition may present a defect in vascular ontogenesis or the outcome of vascular occlusion of both internal carotid arteries and their main branches on carotid angiogram and CT scan. This paper presents the essential features for the clinical diagnosis, suggesting pathogenesis in 2 cases of infantile hydranencephaly.
Basal Ganglia ; Carotid Artery, Internal ; Cerebrum ; Diagnosis ; Hydranencephaly* ; Mesencephalon ; Skull ; Tomography, X-Ray Computed

Diencephalic syndrome of infancy which is characterized by progressive and profound emaciation often with normal or accelerated skeletal growth, hyperkinesias, hypotension, and hypoglycemia. The profound emaciation so characteristic this syndrome has yet to be explained. The diencephalic syndrome is caused by low grade astrocytoma, ependymoma in the third ventricle including the hypothalamus. Authers report a case of diencephalic syndrome which was confirmed in this patient with ependymoma in the region of anterior hypothalamus by transcallosal approach.
Astrocytoma ; Emaciation ; Ependymoma ; Humans ; Hyperkinesis ; Hypoglycemia ; Hypotension ; Hypothalamus ; Hypothalamus, Anterior ; Third Ventricle

Teramethylammonium(TMA) in one of the synthetic compounds of nicotine that act at ganglionic site. The major action of TMA consists of initial stimulation followed by a more persistent depression of all autonomic ganglia by binding to a cholinergic receptor. It is well believed that the level of membrane potential in arterial smooth muscle is an important regulator of tension development. Depolarization and hyperpolarization by only few millivolts results in significant changes in tension. In general, the agents of vascular smooth muscle induce vascular relaxaion. The present study was undertaken to elucidate the effect of TMA on vascular contractility in the isolated rabbit thoracic aorta with or without endothelial cell, and mechanisms involved in the change of vascular contractility by TMA. The results obtained are summarized as follows ; 1) In the presence of endothelial cell, TMA induced a relaxtion of the aorta precontracted with norepinephrine but induced a contraction in the aorta without endothelial cells, indicating that in the rabbit aorta, relaxations produced by TMA were the endothelium-dependent. 2) The addition of inhibitor such as methylene blue, hemoglobin, hydroquinone and p-bromophenacyl bromide during the TMA-induced relaxation reversed the contractile tension to a level similar to or higher than that before the addition of TMA in rabbit thoracic aorta.This relaxation effect of TMA suggest that the TMA-inducdd relaxation in rabbit aorta is due to the release of endotheline derived relaxing factor(EDRF). 3) Relaxation induced by TMA was antagonized by atropine and thus the TMA does seem to act on the muscarinic receptors. 4) TMA reduced the norepinephrine-induced Ca++ influx into rabbit smooth muscle membrane. From the above results, it may be concluded that TMA-induced vacular relaxation in rabbit aorta is due to the release of EDRF. Mechanism involved in the relaxation induced by TMA may be the stimulation of soluble guanylate cyclase and increased tissue cGMP concentrations.
Aorta ; Aorta, Thoracic* ; Atropine ; Depression ; Endothelial Cells ; Endothelins ; Endothelium ; Endothelium-Dependent Relaxing Factors* ; Ganglia, Autonomic ; Ganglion Cysts ; Guanylate Cyclase ; Membrane Potentials ; Membranes ; Methylene Blue ; Muscle, Smooth ; Muscle, Smooth, Vascular ; Nicotine ; Norepinephrine ; Receptors, Muscarinic ; Relaxation

Plasma levels of growth hormone(GH), luteinizing hormone(LH) and cortisol were determined by radioimmunoassay following radiofrequency(RF) stimulation or coagulation of various nuclei in thalamus and hypothalamus. RF stimulation or coagulation of many nuclei in thalamus and hypothalamus consisted of pulvinar and dorsomedial nucleus in thalamus and anterior and posterior hypothalamic nuclei in hypothalamus. Anterior thalamic stimulation resulted in highly significant increase of plasma LH, GH, cortisol and TH levels. However thalamic stimulation resulted no change in the level of various plasma hormones. Hypothalamic lesion produced significantly decreased plasma LH, GH and cortisol levels. Plasma cortisol and LH levels were highest 2 hours after stimulation while GH levels did not increased until 6 hours and TH until 72 hours respectively after stimulation. The significant difference in latency for beginning of hormone secretion suggests that GH, cortisol and LH may be controlled by several separate neuronal networks. Plasma GH and cortisol levels were lowest 72 hrs after coagulation of the anterior hypothalamic area, while GH, cortisol and LH levels did not change following stimulation or coagulation of posterior hypothalamic nucleus and thalamic nucldi. It was also noted that the anterior hypothalamic stimulation or coagulation caused increased or decreased in GH, cortisol, and LH than that observed from stimulation or coagulation of other hypothalamic and thalamic nuclei respectively.
Anterior Hypothalamic Nucleus ; Hydrocortisone ; Hypothalamus* ; Lutein ; Mediodorsal Thalamic Nucleus ; Neurons ; Plasma ; Pulvinar ; Radioimmunoassay ; Thalamic Nuclei ; Thalamus*

No abstract available.
Rhizotomy* ; Scoliosis*

No abstract available.
Scoliosis* ; Syringomyelia*

In 1868 Charcot described the join in tabes dorsalis, which since then has been designated as Charcot neuroarthropathy. It is characterized by painless swelling and abnormal mobility of the affected joint. Arthropathy of the type described by Charcot has been associated with various other conditions, such as diabetes mellitus, syringomyelia, injury to nerve root, congenital indfference to pain, leprosy, and intra-articular use of steroid, etc. Authors present a case of Charcot joint which involved the left knee joint associated with pathologic fracture of the left tibia and fibula.
Arthropathy, Neurogenic ; Diabetes Mellitus ; Fibula ; Fractures, Spontaneous ; Joints ; Knee Joint ; Knee ; Leprosy ; Syringomyelia ; Tabes Dorsalis ; Tibia