Neonatal lupus can occur in infants born to mother with autoimmune disorders through transplacental auto-antibodies. Clinical manifestations in neonatal lupus include cutaneous lesions and hematologic or hepatobiliary findings resembling those seen in systemic lupus erythematosus. In autoimmune state, macrophage activation syndrome (MAS) represent a critical and potentially fatal complication that can result in mortality if not immediately identified and managed with the appropriate care. Here we present a 33-day-old girl diagnosed with neonatal lupus and serious MAS. She was delivered by a primipara mother who did not exhibit any autoimmune symptoms. The patient visited the hospital due to fever and pancytopenia. Laboratory data were compatible with MAS, including pancytopenia, high level of ferritin, soluble interleukin-2, and decreased natural killer cell activity. In addition, autoimmune study showed positive results for anti-nuclear antibody (ANA), anti-Sjogren syndrome antigen A (SSA), and SSB, The autoimmune study for mother also showed positive results for ANA, anti-SSA, and SSB. The patient recovered after she received high dose steroid and supportive care. Our case indicates that neonatal lupus should be taken into consideration when fever, erythematous skin rash, and pancytopenia are observed in infants, even if their mothers have no prior history of autoimmune conditions.

Anterior communicating artery (ACoA) aneurysms may rarely lead to oculomotor nerve palsy. We present here interesting cases in which isolated unilateral adduction paresis mimicking internuclear ophthalmoplegia (INO) was one of the symptoms of suspicious impending ruptured aneurysm of the ACoA. Careful neurologic examination is crucial for early discrimination with INO and oculomotor palsy.

OBJECTIVE: Proximal junctional kyphosis (PJK) is radiologic finding, and is defined as kyphosis of >10° at the proximal end of a construct. The aim of this study is to identify factors associated with PJK after segmental spinal instrumented fusion in adults with spinal deformity with a minimum follow-up of 2 years. METHODS: A total of 49 cases of adult spinal deformity treated by segmental spinal instrumented fusion at two university hospitals from 2004 to 2011 were enrolled in this study. All enrolled cases included at least 4 or more levels from L5 or the sacral level. The patients were divided into two groups based on the presence of PJK during follow-up, and these two groups were compared to identify factors related to PJK. RESULTS: PJK was observed in 16 of the 49 cases. Age, sex and mean follow-up duration were not statistically different between two groups. However, mean bone marrow density (BMD) and mean back muscle volume at the T10 to L2 level was significantly lower in the PJK group. Preoperatively, the distance between the C7 plumb line and uppermost instrumented vertebra (UIV) were no different in the two groups, but at final follow-up a significant intergroup difference was observed. Interestingly, spinal instrumentation factors, such as, receipt of a revision operation, the use of a cross-link, and screw fracture were no different in the two groups at final follow-up. CONCLUSION: Preoperative BMD, sagittal imbalance at UIV, and thoracolumbar muscle volume were found to be strongly associated with the presence of PJK.
Adult ; Back Muscles ; Bone Marrow ; Congenital Abnormalities ; Follow-Up Studies* ; Hospitals, University ; Humans ; Kyphosis* ; Osteoporosis ; Risk Factors* ; Sarcopenia ; Spine

Thoracic pedicle screw fixation techniques are still controversial for thoracic deformities because of possible complications including neurologic deficit. Methods to aid the surgeon in appropriate screw placement have included the use of intraoperative fluoroscopy and/or radiography as well as image-guided techniques. We describe our technique for free hand pedicle screw placement in the thoracic spine without any radiographic guidance and present the results of pedicle screw placement analyzed by computed tomographic scan in two human cadavers. This free hand technique of thoracic pedicle screw placement performed in a step-wise, consistent, and compulsive manner is an accurate, reliable, and safe method of insertion to treat a variety of spinal disorders, including spinal deformity.
Cadaver ; Congenital Abnormalities ; Fluoroscopy ; Hand ; Humans ; Neurologic Manifestations ; Spine

Although the apoptosis of chondrocytes plays an important role in endochondral ossification, its mechanism has not been elucidated. In this study, we show that guanosine induces chondrocyte apoptosis based on the results of acridine orange/ ethidium bromide staining, caspase-3 activation, and sub-G1 fraction analysis. The potent inhibitory effect of dipyridamole, a nucleoside transporter blocker, indicates that extracellular guanosine must enter the chondrocytes to induce apoptosis. We found that guanosine promotes Fas-Fas ligand interaction which, in turn, leads to chondrocyte apoptosis. These findings indicate a novel mechanism for endochondral ossification via metabolic regulation.
Tumor Necrosis Factors/metabolism ; Signal Transduction/drug effects ; Receptors, Tumor Necrosis Factor/*metabolism ; Rats, Sprague-Dawley ; Rats ; Nucleoside Transport Proteins/metabolism ; Membrane Glycoproteins/metabolism ; Guanosine/*pharmacology/physiology ; Fas Ligand Protein ; Chondrocytes/*drug effects/metabolism ; Apoptosis/*drug effects ; Antigens, CD95 ; Animals

OBJECTIVE: Gait pattern in patients with lumbar degenerative kyphosis (LDK) is disturbed because trunk bends forward due to decreased lumbar lordosis. Surgical therapy in LDK is required when conservative management fails. We investigated kinematic and kinetic changes of the pelvis, hip, and knee joints on the sagittal plane in patients with LDK before and after operation. METHOD: Fifteen patients underwent operations between March 1999 and September 2003. Gait analysis was performed for all patients. RESULTS: Total lumbar lordotic angle increased from 10.50 degrees +/-11.22 degrees to 26.71 degrees +/-8.80 degrees postoperation. In gait analysis, anterior pelvic tilting angle increased from maximum 7.86 degrees +/-9.69 degrees, minimum 4.40 degrees +/-9.82 degrees to maximum 12.61 degrees +/-5.36 degrees, minimum 9.68 degrees +/-5.63 degrees (p<0.05). Maximum hip flexion angle changed from 31.39 degrees +/-11.71 degrees to 35.83 degrees +/-5.84 degrees (p<0.05). Maximum knee flexion angle in terminal stance phase decreased from 13.32 degrees +/-7.34 degrees to 8.30 degrees +/-6.38 degrees (p<0.05). CONCLUSION: After corrective operation, an increase of lumbar spine lordosis and anterior pelvic tilt with decrease of knee flexion were observed. However, an increase of maximum hip flexion secondary to increased anterior pelvic tilting influenced ambulation negatively. Therefore, stretching of the hip flexor and strengthening of the hip extensor are required before and after operation.
Animals ; Decompression Sickness ; Gait ; Hip ; Humans ; Knee ; Knee Joint ; Kyphosis* ; Lordosis ; Lower Extremity* ; Pelvis* ; Spine ; Walking

Rolandic epilepsy(or benign epilepsy with centrotemporal spikes) and childhood absence epilepsy are idiopathic epilepsies and are the most common forms of age-related epilepsies, occurring in previously neurologically normal children. Benign epilepsy with centrotemporal spikes is the most common partial childhood epilepsy, beginning between 2 and 13 years of age and characterized by typical EEG focal discharges and a self- limited course with recovery in or before puberty. Childhood absence epilepsy is one of generalized epilepsies with bilateral synchronous and symmetrical spike-wave paroxysms of 3 Hz and it has a good prognosis under a correct antiepilepsy treatment. These two epilepsies share some common features : similar age at onset, overall good prognosis and marked hereditary predisposition. A coincidence of these two epilepsies in a patient is very rare. We experienced a case of a coincidence of rolandic and childhood absence epilepsy in a 6 year-old female. The presence of an absence focus in rolandic epilepy, however, makes the coincidence of these entirely distinct phenomena, even if very rare, not excluded. We present the case with a review of related literature.
Adolescent ; Child ; Electroencephalography ; Epilepsy ; Epilepsy, Absence* ; Epilepsy, Generalized ; Epilepsy, Rolandic ; Female ; Humans ; Prognosis ; Puberty

OBJECTIVE: We analyze pain relief, deformity correction and complication rate after percutaneous kyphoplasty for osteoporotic vertebral compression fractures. METHODS: The authors retrospectively reviewed medical records and radiological findings of 32 patients who underwent percutaneous kyphoplasty for osteoporotic vertebral compression fractures. RESULTS: The patients had significant pain improvement with the procedure. The visual analogue scale score reduced from 8.6 to 3.4 significantly after the procedure. The midline vertebral body height significantly increased postoperatively, but mean kyphotic angle did not. There was no serious complication except one case of epidural cement leakage without neurological impairment. CONCLUSION: Balloon kyphoplasty safely can reduce severe back pain and returned geriatric patients to higher activity levels. The midline vertebral height is restored significantly. However kyphotic deformity correction is not significant as contrary to what we expected from the present study before it was carried out.
Back Pain ; Body Height ; Congenital Abnormalities ; Fractures, Compression* ; Humans ; Kyphoplasty* ; Kyphosis ; Medical Records ; Retrospective Studies

PURPOSE: We wanted to evaluate the efficacy and safety of using ultrasound guided percutaneous radiofrequency ablation for the benign nodules of the thyroid gland. MATERIALS AND METHODS: We studied 148 patients with benign thyroid nodules (200 total nodules) that were confirmed histopathologically, and we performed ultrasound guided radiofrequency ablation. The radiofrequency ablation was done 1 to 5 times per one nodule, and follow-up ultrasonography was performed one to nineteen months after the ablation procedures. The physical changes and the decrease of volume of the nodules were evaluated, and the complications related to radiofrequency ablation were observed. RESULTS: The mean initial nodule volume was 0.01-95.61 ml (mean; 6.83+/-SD of 10.63 ml) and the nodule volume after radiofrequency ablation was decreased to 0.00-46.56 ml (mean; 1.83+/-SD of 4.69 ml). The mean volume reduction rate was 73.2%. Reduction of more than 50% was noted in 90% of all cases. For 180 nodules (90%), the decrease was 50% or more, in 20 nodules (10%), the decrease was 49% or less. On gray-scale ultrasonogram obtained after ablation, the echogenicity of the nodules changed to darker, and on the doppler-sonogram, the vascular flow within the nodules disappeared in all cases. Most patients complained pain during or right after the procedure, but the pain was transient and subsided after medication. Two patients developed hoarseness that was improved in 1 week and 2 months, respectively. CONCLUSION: Sonoguided percutaneous radiofrequency ablation can be one of the treatments for benign nodules of the thyroid gland.
Catheter Ablation* ; Follow-Up Studies ; Hoarseness ; Humans ; Thyroid Gland* ; Thyroid Nodule ; Ultrasonography

PURPOSE: To evaluate the physiologic background of aneurysms poorly visualized during 3D-TOF MRA, contrast-enhanced MRA (CEMRA) and DSA due to hemodynamic isolation. MATERIALS AND METHODS: Using handmade elastic silicon phantoms to represent terminal basilar tip aneurysms, 3D-TOF MRA, CEMRA and DSA were used to determine blood turnover. Aneurysmal neck size was 2 mm and 10 mm, and the use of a pulsatile pump also helped recreate human physiologic parameters. We compared the results with those of computational fluid dynamics. RESULTS: DSA images of the narrow-necked aneurysm showed that a small volume of contrast medium washed into it during the systolic phase. As the width of its neck increased, the turnover volume of fragments of contrast bolus also increased. At CEMRA, the broad-necked aneurysm was visualized as the main bolus of Gd-DT PA passed through it, and at delayed CEMRA, the narrow-necked aneurysm was visualized faintly after the passage of bolus Gd-DT PA. The results correlated closely with those of 3DTOF MRA and computational fluid dynamics. CONCLUSION: The visualization of intracranial aneurysms at 3D-TOF MRA, CEMRA and DSA was greatly dependent upon blood turnover, which varied according to aneurismal neck size. A narrow-necked aneurysm might be missed at 3D-TOF MRA, CEMRA and DSA due to hemodynamic isolation.
Aneurysm ; Angiography, Digital Subtraction ; Hemodynamics ; Humans ; Hydrodynamics ; Intracranial Aneurysm* ; Neck* ; Silicones