BACKGROUND: The loss of ganglion cells observed in Alzheimer's disease (AD) patients might be attributable to a neurodegenerative process involving the neuroretinal structure. The objective of this study was to determine the retinal thickness in patients with AD using optical coherence tomography (OCT). METHODS: Neuro-ophthalmologic examinations were performed in eight AD patients and eight age-matched control subjects. The average thicknesses of the retinal nerve fiber layer (RNFL) and the ganglion cell-inner plexiform layer (GCIPL) were measured using spectral-domain OCT in both subject groups, and the results were compared. RESULTS: The RNFL was thinner in AD patients than in the age-matched controls (p<0.05), especially at the nasal and inferior retina, and the GCIPL was also significantly thinner (p<0.05). CONCLUSIONS: The decreased retinal thickness observed in AD patients suggests that OCT can be used as a biological marker of AD. The findings of this preliminary study suggest that the extent of the reduction in GCIPL and RNFL thicknesses should be investigated further.
Alzheimer Disease ; Biomarkers ; Ganglion Cysts ; Humans ; Nerve Fibers ; Retina ; Retinaldehyde ; Tomography, Optical Coherence

Ilizarov technique has been successfully applied to limb lengthening for several decade, one of its main drawback is, however, long application of external fixator over 6 months. To lessen this time, simultaneous intramedullary fixation which convert later to static fixation by insertion of interlocking screws has been proposed. The indication for surgical limb lengthening includes familial short stature below the 3rd percentile. We compared the results of thirteen tibial lengthening with Ilizarov method to that of eleven hybrid methods. The hybrid system is composed of an unreamed AO intramedullary nail (IM) and ring fixator. Once the lengthening is completed, distal interlocking screws were inserted. In the group who were treated by hybrid system, the mean lengthening of tibia was 7.1cm (range, 6.3-9.5), the mean of external fixation period 7 months (range, 5-9), the mean healing index 1.2 month/cm (range, 1.1-1.4). On the other hand, the mean lengthening of tibia was 7.5cm (range, 5-10.3), the mean of external fixation period 9.8 months (range, 6-15), the mean healing index 1.4 month/cm (range, 1.1-1.8) in the group treated by Ilizarov method. Mean follow-up period was 23 months (range 14-47). There was no rotational or angular deformities and no loss of lengthening in the hybrid group. However we experienced three cases of angular deformity at the distraction site during lengthening in Ilizarov group. There was no deep infection in both groups. In the hybrid group we found several advantages such as no deformity, no loss of lengthening, and less scar by early removal of fixator, but some disadvantages such as need of subsequent procedures and limitation in intramedullary nailing.
Cicatrix ; Congenital Abnormalities ; External Fixators ; Extremities ; Follow-Up Studies ; Fracture Fixation, Intramedullary* ; Hand ; Ilizarov Technique* ; Tibia

Deformity of the forearm is common in patients with hereditary multiple osteochondromas. It produces cosmetic and functional impairment. The deformity of the forearm in hereditary multiple osteochondromas that we described is often associated with osteochondroma of the distal part of ulna causing ulnar longitudinal hypoplasia. This osteochondroma mechanically disturbs the axial alignment of the adjacent joints, either directly or though tension on the interosseous membrane. We reviewed 9 cases of forearm deformity caused by hereditary multiple osteochondromas in 9 patients, in whom ulnar lengthening with excision of osteochondroma was a main procduree. The patients were followed along for a mean of 3 year 6 months. The procedures included ulnar lengthening with excision of osteochondroma in the distal part of the ulna in 6 patients, ulnar lengthening with excision of the osteochondroma and corrective osteotomy of the radius in 2 patients, and stapling of the distal physis of the radius with excision of osteochondroma in 1 patient. Ulnar lengthening was performed by an immediate bone graft and internal fixation in 5 patients, or gradual distraction with and external fixator in 3 patients. The cosmetic results of the surgery were very gratifying and the range of motion of radial deviation at the wrist was increased. Partial recurrence of the deformity was seen during follow up in the skeletally immature patients. However, in general, Ulnar lengthening with excision of osteochondroma was an effective procedure to restore cosmesis as well as function of the wrist.
Congenital Abnormalities ; Exostoses, Multiple Hereditary ; External Fixators ; Follow-Up Studies ; Forearm ; Humans ; Joints ; Membranes ; Osteochondroma ; Osteotomy ; Radius ; Range of Motion, Articular ; Recurrence ; Transplants ; Ulna ; Wrist

Genu recurvatum deformity of the knee can be due to the deformity of the bone of the proximal end of the tibia or to the changes in the soft tissues, or both. The causes of acquired genu recurvatum include trauma, wire traction of the tibia, operative procedures involving the proximal tibial physis, Osgood-Schlatter's disease, osteomyelitis and prolonged immobilization. The most frequent symptoms of genu recurvatum are weakness, pain and instability of the knee, genu valgus, and shortening of the affected lower extremity. Operative treatment consists of tibial osteotomy and soft tissue procedures performed on the capsule and ligaments, and recently percutaneous corticotomy of the proximal tibia and gradual correction by the Ilizarov external fixator became one of the treatment modalities. From Nov. 1990 to June 1993, the authors have performed gradual correction by the Ilizarov external fixator in 6 patients of genu recurvatum and the results were as follows. 1. The mean age was 14 years ranging from 11 to 21. And there were 2 males and 4 females. 2. In the past history, there were ipsilateral femur fractures in 5 cases in which 4 cases were treated with skeletal pin traction on the proximal tibia, and one case had ipsilateral open tibiofibular fracture. 3. After Ilizarov external fixation, proximal tibial corticotomy just inferior to the tibial tuberosity were performed in all 6 cases. 4. The follow up period was 19 months in average. 5. The results were evaluated according to a scoring system that was suggested by Lecuire et al.: 2 cases were excellent, 3 cases were good and 1 case was fair. 6. Ilizarov gradual correction was very effective not only in the treatment of genu recurvatum but also in the concurrent correction of limb length inequality and mechanical axis deviation of the lower extermity.
Congenital Abnormalities ; External Fixators ; Extremities ; Female ; Femur ; Follow-Up Studies ; Humans ; Immobilization ; Knee ; Ligaments ; Lower Extremity ; Male ; Osteomyelitis ; Osteotomy ; Socioeconomic Factors ; Surgical Procedures, Operative ; Tibia ; Traction

OBJECTIVES: Aim of this study is to investigate the clinical efficacy of the vestibular function tests (VFTs) and the predictability of lesion side of vestibular asymmetry parameters in acute unilateral peripheral vestibulopathy. METHODS: Medical records and results of VFTs (caloric, rotatory chair, and head impulse tests) of 57 patients with acute unilateral vestibulopathy were reviewed retrospectively. The VFTs were examined within 7 days after the clinical onset. RESULTS: For the caloric test, 74% showed significant canal paresis and the predictability of lesion side was 88%. For the sinusoidal harmonic acceleration test, 91% had low gain in at least 1 Hz, phase lead showed 70%, 89% showed phase asymmetry and the predictability of lesion side was 90%. For velocity step test, 67% had abnormal Tc asymmetry and the predictability of lesion side was 95%. In bedside head impulse test (HIT), abnormal catch up saccades were observed in 89% and the predictability of lesion side was 100%. For the video HIT, cover or overt catch-up saccades were observed in 95% and the predictability of lesion side was 100%. One hundred percent (100%) had low gain on the video HIT, but the lesion sides were uncertain because of bilateral involvements or artifacts. CONCLUSIONS: The most important things in the diagnosis of acute unilateral vestibulopathy are typical clinical symptoms and spontaneous nystagmus. A combination of rotatory, caloric, and HITs will result in a more complete examination of the vestibular system. Among them, HIT is recommended as the best tool in acute unilateral vestibulopathy.
Acceleration ; Artifacts ; Caloric Tests ; Diagnosis ; Exercise Test ; Head ; Head Impulse Test ; Humans ; Medical Records ; Paresis ; Retrospective Studies ; Saccades ; Treatment Outcome ; Vestibular Function Tests

BACKGROUND: Tamoxifen, a synthetic antiestrogen, increases disease-free and overall survival when used as adjuvant therapy for primary breast cancer. Because it is given for long periods, it is important to know whether tamoxifen affects bone mineral metabolism in women. However few reports on this topic have been published in Korea. METHODS: We classified patients into four subgroups by age, hormone receptor status, and menstrual status, and during a five-year randomized, uncontrolled clinical investigation we studied retrospectively the effects of tamoxifen on biochemical measures of bone mineral metabolism in 112 women with axillary-node-negative breast cancer. RESULTS: In all four subgroups, neither the serum calcium nor the phosphorus level showed any fluctuation beyond the reference range. Serum alkaline phosphatase (ALP) decreased one year after the operation in all subgroups, and then increased afterwards in the reference range. However, women in the under 40-years-old subgroup and in the both estrogen and progesterone receptor negative subgroup showed a prominent rise in the upper value of the reference range up to 436 IU/l. In the 60 or older subgroup, ALP values showed some fluctuations similar to those for the both estrogen and progesterone receptor positive subgroup. CONCLUSIONS: We think that treatment with tamoxifen may be associated with preservation of bone minerals in women after menopause, in women 60 and older, and in women with a positive estrogen and progesterone receptor. Also, this effect probably can help to prevent or to delay the development of osteoporosis in these women with breast cancer, but a possible relation with a decrease in the risk of fractures still remains to be evaluated.
Alkaline Phosphatase ; Breast Neoplasms* ; Breast* ; Calcium ; Estrogen Receptor Modulators ; Estrogens ; Female ; Humans ; Korea ; Menopause ; Metabolism* ; Minerals ; Osteoporosis ; Phosphorus ; Receptors, Progesterone ; Reference Values ; Retrospective Studies ; Tamoxifen*

BACKGROUND: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder that is characterized by involuntary eye movements and myoclonus. OMS exhibits various etiologies, including paraneoplastic, parainfectious, toxic-metabolic, and idiopathic causes. The exact immunopathogenesis and pathophysiology of OMS are uncertain. CASE REPORT: We report the case of a 19-year-old male who developed opsoclonus and myoclonus several days after a flu-like illness. Serological tests revealed acute mumps infection. The findings of cerebrospinal fluid examinations and brain magnetic resonance imaging were normal. During the early phase of the illness, he suffered from opsoclonus and myoclonus that was so severe as to cause acute renal failure due to rhabdomyolysis. After therapies including intravenous immunoglobulin, the patient gradually improved and had fully recovered 2 months later. CONCLUSIONS: This is the first report of OMS associated with mumps infection in Korea. Mumps infection should be considered in patients with OMS.
Acute Kidney Injury ; Brain ; Cerebrospinal Fluid ; Eye Movements ; Humans ; Immunoglobulins ; Korea ; Magnetic Resonance Imaging ; Male ; Mumps ; Mumps virus* ; Myoclonus ; Nervous System Diseases ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome* ; Rhabdomyolysis ; Serologic Tests ; Young Adult

A 32-year-old man was transferred to an intensive care unit due to respiratory difficulties with a 4-day history of progressive areflexic quadriparesis following acute hepatitis A. A nerve-conduction study revealed inexcitability of most nerves. The cerebrospinal fluid showed albuminocytologic dissociation, suggesting Guillain-Barre syndrome (GBS). The patient appeared brain dead on day 4, showing absent brainstem reflexes, respiratory failure, and fully dilated and fixed pupils. This case is an example of how GBS can evolve and simulate a brain-dead state from fulminant deafferentation following acute hepatitis A.
Adult ; Brain Death ; Brain Stem ; Cerebrospinal Fluid ; Guillain-Barre Syndrome* ; Hepatitis A* ; Hepatitis* ; Humans ; Intensive Care Units ; Pupil Disorders ; Quadriplegia ; Reflex ; Respiratory Insufficiency

PURPOSE: Couinaud described segment IV as being equivalent to segments II and III, as the umbilical portion of the portal vein (PV), and its equal branch of segment II, originated from the transverse portion of the PV. On the contrary, Healey suggested the presence of left lateral and medial segments, on the basis of umbilical fissure. Recently, some author have claimed the branch of segment II originated from the distal portion of the ligamentum venosum (LV), and that this branch was not equal to, only a branch of, the umbilical portion. Our goal was to evaluate the surgical anatomy of the left lobe of the liver through dissecting Korean cadavers. METHODS: The number of cadavers dissected totaled 10. PV, its branches, and the LV were dissected and the length of the transverse portion measured. The distance between the origin of the transverse portion and that of the segment II branch were also measured. RESULTS: The branch of segment II originated from the distal portion of the LV in all 10 cases. The length of the transverse portion was 18.8+/-5.8 mm, and the distance between the origins of the LV and segment II branch was 7.0+/-3.1 mm. CONCLUSION: Considering the embryology of the liver, as well as the above result, the umbilical portion and segment II branch were not equal anatomic structures. The umbilical portion and LV are equal anatomic structures. The branch of segment II is only one of the branches of the umbilical portion. We think Healey's classification is more accurate for the left lobe of the liver.
Cadaver* ; Classification ; Embryology ; Hepatectomy ; Liver* ; Portal Vein

PURPOSE: To understand the modulation of genes by atrophy, differential expression of genes in normal and denervated skeletal muscle was investigated by DNA chip technology. MATERIALS AND METHODS: Sciatic nerve and femoral nerve were resected in right leg of rat to make the muscle atrophy model. Muscle tissues from the gastrocnemius of normal and denervated legs were homogenized and RNA were extracted. Dyes were labelled during reverse transcription and hybridization was done into the DNA chip which is consisted of about 5,000 probes. RESULTS: By statistical analysis, 39 genes were selected as differentially expressed genes by atrophy. 15 known genes up-regulated by atrophy were genes related to immune response, extracellular matrix, andsignal transduction in plasma membrane. 7 known genes down-regulated by atrophy were genes related to cell growth and proliferation, intracellular signal transduction, and energy metabolism. Some unknown gene functions were analysed by bioinformatics analysis and they were highly homologous genes with McKusick-Kaufman syndrome protein, ADP-ribosylation factor-like 4, and component X of pyruvate dehydrogenase complex. CONCLUSION: These results suggest that reduction of energy metabolism, activation of cholesterol exclusion, and changes on signal transduction pathway are involved in the process of atrophy by denervation in skeletal muscle of rat.
Abnormalities, Multiple ; Animals ; Atrophy ; Cell Membrane ; Chimera ; Cholesterol ; Coloring Agents ; Computational Biology ; Denervation ; Energy Metabolism ; Extracellular Matrix ; Femoral Nerve ; Gene Expression ; Heart Defects, Congenital ; Hydrocolpos ; Leg ; Muscle, Skeletal ; Muscles ; Muscular Atrophy ; Oligonucleotide Array Sequence Analysis ; Oxidoreductases ; Polydactyly ; Pyruvic Acid ; Rats ; Reverse Transcription ; RNA ; Sciatic Nerve ; Signal Transduction ; Uterine Diseases