We have experienced a rare case of progressive supranuclear palsy with hallucination and delusion. The common manifestations of progressive supranuclear palsy are gait disturbance, mental change and sign of vertical ophthamoplegia but no efficacious therapy has been known. Neuropsychiatric symptom clusters include cognitive impairment, affective and behavioral changes, sleep disturbance, and psychotic symptoms. Though schizophrenia-like psychosis has been reported but this is certainly rare. Addressing a case of progressive supranuclear palsy, in whom parkisonian symptoms appeared with concurrent psychotic symptoms, we emphasize accurate diagnosis.
Delusions ; Diagnosis ; Gait ; Hallucinations ; Psychotic Disorders ; Supranuclear Palsy, Progressive*

Hashimotos thyroiditis and Graves disease have been thought to be the autoirnmune diseases having their distinct and separate clinical and pathologic features. Because of the partial and complete simi1arity in histologic feature, it has been hypothesized that Hashimoto's thyroiditis and Oraves disease may be interrelated. Several patients who had been diagnosed as Graves disease showed features of hypothyroidism, and were proven to have histologie features of Hashimotos thyroiditis in their thyroid tissue. Two meehanisms had been suggested to explain tbis phenomenon. One is that the combined Hashimotos thyroiditis in Graves' disease may become predominant with time, The other is that the amount of TSH receptor blocking antibody may increase in the course of Graves disease. Early recognition of these patients would be impartant to estabilish therapeutic plan. Futhermcee, extensive study of these patients would give more understanding of the mechanism of these diseases. Here we report 5 cases of clinically diagnosed Graves disease with pathologic features of Hashimotos thyroiditis or focal lymphocytic thyroiditis.
Graves Disease ; Humans ; Hypothyroidism ; Receptors, Thyrotropin ; Thyroid Gland ; Thyroiditis ; Thyroiditis, Autoimmune

It is uncommon that anesthesiologists experience patients with thyroid storms. In our case, the patient had been medicated for 5 years, however, she developed agranulocytosis. Anti-thyroid drugs were stopped and hyperthyroidism progressed. Her symptoms and laboratory results revealed manifestation of thyroid storm: TSH of < 0.005 IU/L, free T4 of > 7.77 ng/dl, T3 of 403.1 ng/dl, and T4 of 22.15 microg/dl. The euthyroid state had not been achieved before the surgery. From the judgment of difficulty controls of hyperthyroidism, the surgeon requested for an emergency operation. We report a case of total intravenous anesthesia with propofol and remifentanil which achieved hemodynamic stability.
Agranulocytosis ; Anesthesia, Intravenous* ; Emergencies ; Hemodynamics ; Humans ; Hyperthyroidism ; Judgment ; Propofol ; Thyroid Crisis ; Thyrotoxicosis*

Achondroplasia is the most common non-lethal skeletal dysplasia. Underdevelopment and premature ossification of bones result in characteristic craniofacial and spinal abnormalities. Achondroplastic dwarfs have low fertility rates and require delivery by cesarean section due to their cephalo-pelvic disproportion. Controversy exists regarding the ideal anesthesia for an achondroplastic parturient dwarf for urgent cesarean section. Anesthesia, whether general or regional, presents many potential problems during cesarean section. We report the experience of the epidural anesthesia in an achondroplastic dwarf undergoing elective cesarean section.
Achondroplasia ; Anesthesia ; Anesthesia, Epidural ; Birth Rate ; Cesarean Section ; Female ; Pregnancy

One of the predominant methods for preserving the internal iliac artery (IIA) in aortoiliac aneurysms is to use an iliac bifurcated device (IBD). However, there are a few limitations to the use of IBD in Korea. Our study aims to present the technical aspects of these devices, and to provide a mid-term analysis of IBD. Since 2013, 4 IBDs were used in 4 patients, with a mean follow-up of 14 months (range, 6-22 months). A 100% technical success rate was achieved without mortality and morbidity in our cases. The mean procedural time was 176 min (range, 145-240 min), and the mean contrast dose used was 184 mL (range, 135-220 mL). Type I or III endoleaks and postoperative expansion of the aneurysms weren't observed. Our cases demonstrates that IBD is a relatively safe repair method of aortoiliac aneurysms with preservation of the IIAs. However, a longer follow-up is needed to review the midterm results.
Aneurysm* ; Aortic Aneurysm, Abdominal ; Endoleak ; Endovascular Procedures ; Follow-Up Studies ; Humans ; Iliac Artery ; Korea ; Mortality

PURPOSE: The purpose of this study was to evaluate the early and midterm results of superficial femoral artery (SFA) stenting with self-expanding nitinol stents and to identify the factors affecting patency. MATERIALS AND METHODS: SFA stenting was performed in 165 limbs of 117 patients from January 2009 to December 2013. Patients were followed-up for the first occurrence of occlusion or stenosis based on computed tomography and duplex scan results and a decrease in ankle brachial index of >15%. RESULTS: During the follow-up period (mean, 15.3+/-3.2 months), no early thrombotic reocclusions occurred within 30 days, but in-stent restenosis developed in 78 limbs. The primary patency rates at 6, 12, 18, and 24 months were 78%, 66%, 42%, and 22%, respectively, and the secondary patency rates were 85%, 72%, 58%, and 58%, respectively. TASC II C or D lesions, stent length >8 cm, number of patent tibial arteries and diabetes were significantly associated with reintervention. CONCLUSION: The midterm results of stenting for SFA occlusive disease were disappointing because the primary and secondary patency rates at two years were 22% and 58%, respectively. Reintervention after SFA stenting remains a major problem, particularly in patients with diabetes mellitus or long TASC II C or D lesions.
Ankle Brachial Index ; Constriction, Pathologic ; Diabetes Mellitus ; Endovascular Procedures ; Extremities ; Femoral Artery* ; Follow-Up Studies ; Humans ; Stents* ; Tibial Arteries

Pericentric inversion of chromosome 4 can give rise to 2 alternate recombinant (rec) chromosomesby duplication or deletion of 4p. The deletion of distal 4p manifests as Wolf-Hirschhorn syndrome (WHS). Here, we report the molecular cytogenetic findings and clinical manifestations observed in an infant with 46,XX,rec(4)dup(4q)inv(4)(p16q31.3)pat. The infant was delivered by Cesarean section at the 33rd week of gestation because pleural effusion and polyhydramnios were detected on ultrasonography. At birth, the infant showed no malformation or dysfunction, except for a preauricular skin tag. Array comparative genomic hybridization analysis of neonatal peripheral blood samples showed a gain of 38 Mb on 4q31.3-qter and a loss of 3 Mb on 4p16.3, and these results were consistent with WHS. At the last follow-up at 8 months of age (corrected age, 6 months), the infant had not achieved complete head control.
*Chromosome Deletion ; *Chromosome Duplication ; *Chromosome Inversion ; *Chromosomes, Human, Pair 4 ; Comparative Genomic Hybridization ; Female ; Gestational Age ; Humans ; Infant ; Pleural Effusion/ultrasonography ; Polyhydramnios/ultrasonography ; Pregnancy ; Wolf-Hirschhorn Syndrome/*genetics

No abstract available.
Humans ; Prader-Willi Syndrome ; Temporal Arteries

BACKGROUND: Several factors, such as compromised cardiopulmonary function, anticoagulative therapy, or anatomical deformity in the elderly, prevent general anesthesia and neuraxial blockade from being conducted for total knee replacement arthroplasty (TKRA). We investigated the efficacy of femoral/sciatic nerve block with lateral femoral cutaneous nerve block (FSNB) as an alternative procedure in comparison with combined spinal epidural nerve block (CSE) in patients undergoing TKRA. METHODS: In this observational study, 80 American Society of Anesthesiologists physical status I-III patients scheduled for elective unilateral TKRA underwent CSE (n = 40) or FSNB (n = 40). Perioperative side effects, intraoperative medications, duration and remaining amount of intravenous patient-controlled analgesia, rate of satisfaction with the surgical anesthesia and postoperative analgesia, willingness to recommend the same surgical anesthesia and postoperative analgesia to others, and postoperative visual analog scale pain scores were assessed. Statistical analysis was done using Chi-square test, Student's t-test, and repeated-measures analysis of variances. RESULTS: There was significantly more use of antihypertensives, analgesics, and sedatives in the FSNB group. There were no significant differences of perioperative side effects, duration and remaining amount of intravenous patient-controlled analgesia, rate of satisfaction with the surgical anesthesia and postoperative analgesia, willingness to recommend the same surgical anesthesia and postoperative analgesia to others, and postoperative visual analog scale scores between the two groups. CONCLUSIONS: FSNB with a sophisticated use of antihypertensives, analgesics, and sedatives to supplement insufficient block offers a practical alternative to CSE for TKRAs.
Aged ; Analgesia ; Analgesia, Patient-Controlled ; Analgesics ; Anesthesia ; Anesthesia, Epidural ; Anesthesia, General ; Anesthesia, Spinal ; Antihypertensive Agents ; Arthroplasty ; Arthroplasty, Replacement, Knee ; Congenital Abnormalities ; Femoral Nerve ; Humans ; Hypnotics and Sedatives ; Nerve Block ; Sciatic Nerve

Insulin autoimmune syndrome (IAS) is characterized by spontaneous hypoglycemia caused by insulin autoantibodies in the absence of exogenous insulin administration. Some drugs containing sulfhydryl compounds are known to initiate the onset of IAS. A 67-year-old female who had diabetes for 5 years visited the outpatient clinic at our institution due to diabetic peripheral polyneuropathy. She was prescribed alpha-lipoic acid (ALA), which contains two sulfur atoms. Two weeks later, she complained of recurrent hypoglycemic symptoms. We detected a high level of insulin and high titers of insulin autoantibodies. Her human leukocyte antigen (HLA) genotype included the DRB1*0406 allele, which indicates a high level of susceptibility to IAS. She was treated with prednisolone. After this episode, she experienced two more hypoglycemic events after taking ALA for diabetic neuropathy in other hospitals. As ALA can be used to treat diabetic peripheral polyneuropathy, physician discretion is advised based on the possibility of IAS due to ALA in diabetic patients.
Aged ; Alleles ; Ambulatory Care Facilities ; Autoantibodies ; Diabetic Neuropathies ; Female ; Genotype ; Humans ; Hypoglycemia ; Insulin Antibodies ; Insulin* ; Leukocytes ; Polyneuropathies ; Prednisolone ; Sulfhydryl Compounds ; Sulfur ; Thioctic Acid*