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.

Adult spinal deformity (ASD) is one of the most challenging spinal disorders associated with broad range of clinical and radiological presentation. Correct selection of fusion levels in surgical planning for the management of adult spinal deformity is a complex task. Several classification systems and algorithms exist to assist surgeons in determining the appropriate levels to be instrumented. In this study, we describe our new simple decision making algorithm and selection of fusion level for ASD surgery in terms of adult idiopathic idiopathic scoliosis vs. degenerative scoliosis.
Adult* ; Classification ; Congenital Abnormalities* ; Decision Making* ; Humans ; Scoliosis ; Spinal Fusion ; Surgeons

OBJECTIVE: To improve pedicle screw placement accuracy with minimal radiation and low cost, we developed specially designed K-wire with a marker. To evaluate the accuracy of thoracolumbar pedicle screws placed using the novel guide-pin and portable X-rays. METHODS: Observational cohort study with computerized tomography (CT) analysis of in vivo and in vitro pedicle screw placement. Postoperative CT scans of 183 titanium pedicle screws (85 lumbar and 98 thoracic from T1 to L5) placed into 2 cadavers and 18 patients were assessed. A specially designed guide-pin with a marker was inserted into the pedicle to identify the correct starting point (2 mm lateral to the center of the pedicle) and aiming point (center of the pedicle isthmus) in posteroanterior and lateral X-rays. After radiographically confirming the exact starting and aiming points desired, a gearshift was inserted into the pedicle from the starting point into the vertebral body through the center of pedicle isthmus. RESULTS: Ninety-nine percent (181/183) of screws were contained within the pedicle (total 183 pedicle screws : 98 thoracic pedicle screws and 85 lumbar screws). Only two of 183 (1.0%) thoracic pedicle screws demonstrated breach (1 lateral in a patient and 1 medial in a cadaver specimen). None of the pedicle breaches were associated with neurologic or other clinical sequelae. CONCLUSION: A simple, specially designed guide-pin with portable X-rays can provide correct starting and aiming points and allows for accurate pedicle screw placement without preoperative CT scan and intraoperative fluoroscopic assistance.
Cadaver ; Cohort Studies ; Humans ; Spine* ; Titanium ; Tomography, X-Ray Computed

We investigated the question of whether 7-oxygenated cholesterol derivatives could affect inflammatory and/or immune responses in atherosclerosis by examining their effects on expression of IL-23 in monocytic cells. 7alpha-Hydroxycholesterol (7alphaOHChol) induced transcription of the TLR6 gene and elevated the level of cell surface TLR6 protein in THP-1 monocytic cells. Addition of an agonist of TLR6, FSL-1, to TLR6-expressing cells by treatment with 7alphaOHChol resulted in enhanced production of IL-23 and transcription of genes encoding the IL-23 subunit alpha (p19) and the IL-12 subunit beta (p40). However, treatment with 7-ketocholesterol (7K) and 7beta-hydroxycholesterol (7betaOHChol) did not affect TLR6 expression, and addition of FSL-1 to cells treated with either 7K or 7betaOHChol did not influence transcription of the genes. Pharmacological inhibition of ERK, Akt, or PI3K resulted in attenuated transcription of TLR6 induced by 7alphaOHChol as well as secretion of IL-23 enhanced by 7alphaOHChol plus FSL-1. Inhibition of p38 MAPK or JNK resulted in attenuated secretion of IL-23. These results indicate that a certain type of 7-oxygenated cholesterol like 7alphaOHChol can elicit TLR6-mediated expression of IL-23 by monocytic cells via PI3K/Akt and MAPKs pathways.
Atherosclerosis ; Cholesterol ; Interleukin-12 ; Interleukin-23* ; Macrophages ; p38 Mitogen-Activated Protein Kinases ; Toll-Like Receptor 6

PURPOSE: To evaluate whether intraoperative neurophysiologic monitoring (IONM) with combined muscle motor evoked potentials (mMEPs) and somatosensory evoked potentials is useful for more aggressive and safe resection in intramedullary spinal cord tumour (IMSCT) surgery. MATERIALS AND METHODS: We reviewed data from consecutive patients who underwent surgery for IMSCT between 1998 and April 2012. The patients were divided into two groups based on whether or not IONM was applied. In the monitored group, the procedures were performed under IONM using 75% muscle amplitude decline weaning criteria. The control group was comprised of patients who underwent IMSCT surgery without IONM. The primary outcome was the rate of gross total excision of the tumour on magnetic resonance imaging at one week after surgery. The secondary outcome was the neurologic outcome based on the McCormick Grade scale. RESULTS: The two groups had similar demographics. The total gross removal tended to increase when intraoperative neurophysiologic monitoring was used, but this tendency did not reach statistical significance (76% versus 58%; univariate analysis, p=0.049; multivariate regression model, p=0.119). The serial McCormick scale score was similar between the two groups (based on repeated measure ANOVA). CONCLUSION: Our study evaluated combined IONM of trans-cranial electrical (Tce)-mMEPs and SEPs for IMSCT. During IMSCT surgery, combined Tce-mMEPs and SEPs using 75% muscle amplitude weaning criteria did not result in significant improvement in the rate of gross total excision of the tumour or neurologic outcome.
Adult ; Evoked Potentials, Motor/physiology ; Evoked Potentials, Somatosensory/*physiology ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Spinal Cord Neoplasms/*surgery

Granular cell tumors (GrCTs) of the spinal cord are rare benign tumors with a high rate of local recurrence. Only 6 cases of spinal GrCTs have been reported. GrCT is difficult to distinguish from other benign tumors such as schwannoma using imaging. A radiological "speckled dots" sign may be a useful differentiating feature of GrCT based upon experience with two cases and a review of the literature.
Diagnosis, Differential ; Granular Cell Tumor ; Magnetic Resonance Imaging ; Neurilemmoma ; Recurrence ; Spinal Cord

Detection of cerebrospinal fluid leakage or exact localization of leakage site after spinal surgery is difficult on conventional imaging studies. We report two patients with surgery-related spinal CSF leakage detected on magnetic resonance (MR) myelography. They presented with severe headache after spinal surgeries, lumbar discectomy and excision of spinal meningioma, respectively. The sites of spinal CSF leakage in the patients were detected accurately on MR myelography, and the patients recovered from the postoperative CSF leakage after being treated with an epidural blood patch or reoperation. MR myelography may be effective in demonstrating the exact site of surgery-related spinal CSF leakage.
Blood Patch, Epidural ; Diskectomy ; Headache ; Humans ; Magnetic Resonance Spectroscopy ; Magnetics ; Magnets ; Meningioma ; Myelography ; Reoperation

Giant spinal schwannoma of the cauda equine involving many nerve roots is rare, and ossification is usually not observed in the schwannoma. A 21-year-old man presented with a 12-month history of urinary dysfunction and numbness below the buttocks. Plain radiography showed scalloping of the posterior surface of the vertebral bodies from L4 to the sacrum, and magnetic resonance imaging and computed tomography revealed a giant cauda equina tumor with dystrophic calcification. The tumor was completely removed, with intraoperative neurophysiologic monitoring. Histopathologic examination showed that the tumor was a schwannoma. The patient's postoperative course was uneventful, with urinary function and numbness gradually improving. Although a giant schwannoma accompanied by dystrophic calcification is extremely rare, such a tumor can be removed safely and completely by meticulous dissection and careful neuromonitoring of the cauda equina spinal nerves involved in the tumor.
Buttocks ; Cauda Equina ; Humans ; Hypesthesia ; Magnetic Resonance Imaging ; Neurilemmoma ; Pectinidae ; Sacrum ; Spinal Nerves ; Young Adult

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

Liposarcomas are malignant tumors of the soft tissue, with myxoid liposarcoma being the second most common subtype, tending to occur in the limbs, particularly in the thighs. Myxoid liposarcomas have an intermediate prognosis between well-differentiated and pleomorphic tumors. Spinal metastasis is usual but intradural involvement is extremely rare. We present an unusual case of a multicentric myxoid liposarcoma with intradural involvement. A 41-year-old woman complained of tingling sensation on her left arm. Radiological evaluation revealed multiple masses in her cervical spine, abdominal wall, liver, heart and right thigh, all of which were resected. She was histologically diagnosed with small round cell myxoid sarcoma and underwent adjuvant chemotherapy. However, magnetic resonance imaging analysis after 1 year revealed a large metastatic mass with bony invasion at the C6-T1 level. This mass consisted of extradural and intradural components causing severe compression of the spinal cord. She underwent resection via a posterior facetectomy of C6-7 and an anterior C7 corpectomy. However, the patient died of multiple metastases 18 months after the first diagnosis.
Abdominal Wall ; Adult ; Arm ; Chemotherapy, Adjuvant ; Extremities ; Female ; Heart ; Humans ; Liposarcoma ; Liposarcoma, Myxoid ; Liver ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Prognosis ; Sarcoma ; Sensation ; Spinal Cord ; Spine ; Thigh