In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), clinical presentation with movement disorders such as dystonia and progressive supranuclear palsy-phenotype are rarely reported. None of the CADASIL cases, to our knowledge, has been reported with chorea. Herein, we describe a Korean woman with CADASIL who had presented with chorea. 18F-fl uorodeoxyglucose positron emission tomography (FDG-PET) showed hypometabolism in the right basal ganglia. We found decreased FDG uptake of the right basal ganglia by SPM analysis.

PURPOSE: The aim of this study is to investigate the biomechanical and histological changes in the muscles after tendon injury that have been little studied previously. MATERIALS AND METHODS: Triceps insertion was tenotomized on the left hidlimbs of 60 rabbits. Mechanical extension testing with Instron (rate: 10 mm/min) was performed 1, 2, 4, 8, 12 and 16 weeks after tenotomy in vivo. A load deformation curve was obtained, and this was analyzed in terms of strength and deformation at the elastic limit and at the point of maximal load. Histologic changes were observed after H < E staining and Matson s trichrome staining to measure the percentage area of collagen fibers. RESULTS: Between weeks 1 and 4, the excursion range fell gradually, and after that period, this value showed little changes. After its initial period of disequilibrium, the muscle regained its elastic nature within its newly formed excursion range. The average amount of collagen fibers increased gradually after tenotomy until 8th week. CONCLUSIONS: unless tendon ruptures are repaired early, the accompanying muscle would be irreversibly damaged, both bimechanically and histologically.
Collagen ; Muscles ; Rabbits ; Rupture ; Tendon Injuries ; Tendons ; Tenotomy*

The purpose of this study is to confirm the effectiveness of stay-suture which is commonly used in the procedure of end to end anastomosis, to find out general biomechanical properties of peripheral nerve with gradual stretching and to evaluate the histological difference after breakage of the nerve. The sciatic nerves of adult rabbits were excised and they were grouped by I and II. Group I was composed of 14 normal sciatic nerves and group II, 14 sciatic nerves sutured with 5-0 nylon after transection. They were stretched on the "Instron" universal testing machine at a constant rate of 200mm/min until breakage took place and the results were compared. The mean maximal tensile strength was 13.6N in group I and 2.1N in group II, representing about one seventh value. The mean ultimate tensile strength was 4.2MPa in group I and 0.9MPa in group II, representing about one fifth value. In load-elongation curve of group I, viscoelastic property was noted as all other biological tissues have. The mean modulus of elasticity was 19 in normal sciatic nerves, which is somewhat lower value than other tissues, indicating superior elastic property of peripheral nerves. Transverse histologic examination showed that epineurium lost its continuity earlier than perineurium, implying perineurium is a more resistant structure than epineurium. However, to confirm this, it is thought to be necessary to examine the nerves serially during distraction at each different status. In conclusion, stay-suture provided about one fifth of ultimate tensile stress, implying its usefullness of resisting excessive tension in end to end anastomosis of peripheral nerves. Normal sciatic nerves showed viscoelastic property with mean modulus of elasticity of 19.
Adult ; Elastic Modulus ; Humans ; Nylons ; Peripheral Nerves ; Rabbits ; Sciatic Nerve ; Tensile Strength

A clinical review was made on 2,191 cases of general pediatric surgery under the age of 15 years which were operated upon at the Divisionof Pediatric Surgery, Department of Surgery, Chonnam University Hospital from January 1988 to December 1997. The number of operations in pediatric age were 13,144 (13.2%) out of total 99,555 operations at Chonnam University Hospital and the most prevalent age group was under 1 year of age (14.3%). The number of operations in Divisionof General Pediatric Surgery were 2,191 (16.7%) out of total 13,144 operations in pediatric age and the incidence of patients under 1 year of age in general pediatric surgery was 42.9% (941/2,191). The prevalent diseases under 1 month of age were anorectal malformations (20.6%) and hypertrophic pyloric stenosis (20.3%) and between 1 month to 1 year of age were inguinal hernia (32.4%) and intussusception (19.6%). The total motality rate in neonatal intensive care unit was 31.3%. Gastroschisis presented highest mortality.
Gastroschisis ; Hernia, Inguinal ; Humans ; Incidence ; Infant, Newborn ; Intensive Care, Neonatal ; Intussusception ; Jeollanam-do ; Mortality ; Pyloric Stenosis, Hypertrophic

Tuberous sclerosis is a complex neurocutaneous disease inherited as an autosomal dominant pattern, which is characterized by facial angio-fibroma, mental retardation and seizures. Many cases have been reported in the literatures, but familial cases are relatively rare. We present 9 cases of tuberous sclerois occuring througb 3 generations, which are 3 cases of angiofibroma, mental retardation and seizures, 2 cases of facial angiofibroma and seizures, 1 case of mental retardation and seizures and 3 cases who showed facial angiofibroma only. Two brothers in this family reveald icthyosis vulgaris without tuberous sclerosis.
Angiofibroma ; Family Characteristics* ; Humans ; Intellectual Disability ; Seizures ; Siblings ; Tuberous Sclerosis*

Paralytic hand is the ultimate result of permsnent damage of the central nervous system, failure of the functional repair of peripheral nerve injuries and extensive muscular or tendinous defect resulting in the impairment of hand function. There are a lot of controversies in the side of treatment methods in the paralytic hand, and it is very difficult to formulate the most adequate surgical reconstruction for a given pstient. At Depsrtment of Orthopedic Surgery, Seoul National University Hospital, 66 cases oi paralytic hands in 62 patients have been treated surgically with tendon transfers for 7 years from Jan, 1980 to Dec, 1986. 49 case in 46 patients were followed up for more than one year, and surgical method and its results were anslysed. They consist of CNS lesion (17), peripheral nerve lesion(23) and musulotenidinous defect(9). The methods of surgical reconstruction were opponenesplasty(12), standard set extensor reconstruction(9), Green and Bsnks FCU transfer(11), intrinsic reconstruction(7), pronator rerouting(7), adductorplasty (5) and so on. The mean follow-up was 2.2 years, and in 38 cases good or excellent results were obtained.
Central Nervous System ; Follow-Up Studies ; Hand ; Humans ; Methods ; Orthopedics ; Peripheral Nerve Injuries ; Peripheral Nerves ; Seoul ; Tendon Transfer

Posteroanterior radiographs of normal wrist were taken in seventeen volunteers, and analysed to determine the dynamic relationships between the radiocarpal and midcarpal joint during radioulnar deviation. The total arc of radioulnar deviation was 59 degrees, and the motion was 44% radiocarpal and 56% midcarpal. During radioulnar deviation, the motion at the midcarpal joint was more than that at the radiocarpal joint. It is suggested thst dorsoradial and palmar-ulnar flexion as it occurs at the midcarpal joint can be correlated in radio-ulnar deviation of the wrist more than the coronal sliding motions as it occurs mainly at the radiocarpal joint.
Joints ; Volunteers ; Wrist

BACKGROUND: One of the main disadvantages of caudal block is the long latent period before a satisfactory blockade is obtained. Many investigators have used various preparations of local anesthetic solutions to improve the speed of onset. This study was performed to evaluate the effectiveness of prewarming of lidocaine HCl for caudal block. METHODS: Fifty healthy young patients (ASA I) were allocated into two groups, A and B. In group A, the local anesthetic solution were injected at room temperature (25 degrees C), while in group B, they were injected at 37oC. All the caudal block were performed using 2% lidocaine HCl 20 ml with fentanyl 100ug and epinephrine 1:200,000. The onset time was defined as the period from completion of injection until the patient first noticed loss of sensation to pin-prick on perianal region. Assessment of sensory loss was made at 15 seconds interval. We have compared the onset of sensory blockade between groups. The duration of analgesia and any significant side effects were also recorded. RESULTS: The onset of sensory blockade was significantly faster in group B (3.5 +/- 0.5 minutes) than group A (6.2 +/- 0.9 minutes). The duration of analgesia were not significantly changed between groups. Side effects of urinary retention, pruritus and nausea were noted between both groups but the difference was not significant. CONCLUSIONS: We have found that the onset time was 44% faster with warm lidocaine-fentanyl mixture (37 degrees C) than with the room temperature (25 degrees C). The improved clinical usefulness was achieved with no increase in side effects. The technique is a safe and effective method to reduce the latency of onset.
Analgesia ; Epinephrine ; Fentanyl ; Humans ; Lidocaine ; Nausea ; Pruritus ; Research Personnel ; Sensation ; Urinary Retention

A chemical analysis of subretinal fluid was made in 15 patients (16 eyes) who had undergone retinal detachment surgery from March 1, 1991 to July 31, 1991 at St. Mary's Hospital, Catholic University Medical College. The protein concentration of subretinal fluid ranged from 1.0gm/dl to 7.1gm/dl, and the average protein concentration of subretinal fluid of retinal detachment for 90 days or more showed 5.13 +/- 1.71gm/dl and that of subretinal fluid of retinal detachment for less than 90 days was 2.06 +/- 1.29gm/dl. This revealed increasing tendency of protein concentration in proportion to the duration of detachment (p<.05), and the protein concentration of subretinal fluid did not show any differences according to the size of retinal detachment. Sugar ranged from 32mg/dl to 96mg/dl, and chloride was from 92mEq/L to 126mEq/L. From this study, we found that the protein concentration of subretinal fluid increased in proportion to the duration of retinal detachment. On the contrary, sugar and chloride concentration was not related to the duration of retinal detachment.
Humans ; Retinal Detachment* ; Retinaldehyde* ; Subretinal Fluid*

Reconstruction of a injured digit needs good aesthetic result as well as functional recovery. Since Honda deported the first clinical application in 1984, the venous flap has been used increasingly for skin and soft tissue defects of the fingers and hands. From november 1992 to december 1995, 12 single-pedicled venous island flaps from hand dorsum were used for coverage of soft tissue defects of fingers over exposed bone, tendon, or joint. The pedicle of the flap included one superfitial dorsal vein of hand and its perivenous areolar tissue. Eight of twelve flaps survived completely and four necrosed partially possibly due to excessively long pedicle and poor perivenous areolar tissue. All donor sites were closed directly. Most patients were satisfied with postoperative result cosmetically and functionally. This method has several advantages : easy versatility and operability with superficial location of numerous dorsal vein, making good contour owing to proper thickness of flap, minimal donor site morbidity, and short operation time. We consider that the pedicled venous island flap is another reliable and useful method for reconstruction or finger defects as long as the pedicle length is not long and the perivenous areolar tissue is enough.
Fingers* ; Hand ; Humans ; Joints ; Skin ; Surgical Flaps ; Tendons ; Tissue Donors ; Veins