Familial partial epilepsy with variable foci: a new partial epilepsy syndrome with suggestion of linkage to chromosome 2

Traditional T2-based imaging techniques are geared toward imaging long-T2 species. Traditional techniques are, therefore, not optimal in clinical situations where the information of interest lies in the short-T2 species. T2-selective RF excitation (TELEX) is a technique for obtaining a T2-based contrast that highlights short-T2 values while suppressing long-T2 values-opposite to traditional T2 contrast. Previously, TELEX has been demonstrated qualitatively to highlight only very short-T2 values (T2 approximately 0.001 s). When applied to longer T2 values (T2 > or = 0.01 s), TELEX becomes sensitive to deltaB0 non-uniformities. This restricts its application to problems in which the T2 of interest is very short. In this study, TELEX is characterized quantitatively. Furthermore, a bandwidth broadening scheme is developed that reduces the deltaB0 sensitivity of TELEX. This permits the technique to be applied to longer T2 values. The capabilities and limitations of a practical implementation of TELEX are discussed.     

Spinal anesthesia for elective lumbar spine surgery

STUDY OBJECTIVE: To evaluate a large series of elective lumbar spine surgical procedures by a single surgeon whose patients were all offered spinal anesthesia. DESIGN: Retrospective chart review. SETTING: Tertiary-care teaching hospital. MEASUREMENTS AND MAIN RESULTS: The records of all elective lumbar spine procedures between 1984 and 1995 performed by one surgeon (GRB) were obtained, and 803 were identified. Of those 803 patients, 611 accepted spinal anesthesia. Data collected included patient demographics, details of the spinal and general anesthesia, perioperative complications, and impact of the spinal anesthetic options on the outcome of spinal anesthesia. General and spinal anesthesia patients were comparable for age, gender, height, and ASA physical status. Patients who received spinal anesthesia were significantly heavier than the general anesthesia patients. Among perioperative complications, nausea and deep venous thrombosis occurred significantly more often in the general than spinal anesthesia patients. Mild hypotension and decreased heart rate (HR) were the most common hemodynamic changes with spinal anesthesia, whereas hypertension and increased HR were the result of general anesthesia. Among spinal anesthetic drugs, plain bupivacaine was associated with the lowest incidence of supplemental local anesthetic use intraoperatively compared to hyperbaric bupivacaine or hyperbaric tetracaine. CONCLUSION: Spinal anesthesia is an effective alternative to general anesthesia for lumbar spine surgery and has a reduced rate of minor complications.     

Fatal cyst formation after fetal mesencephalic allograft transplant for Parkinson''s disease

PURPOSE: The aim of this study was to investigate the characteristics of the structural transitions and changes in ligand binding properties of different albumins during the pH-dependent structural transition, often referred to as the N-B transition. METHODS: Structural transitions were evaluated by means of spectrometry, differential scanning calorimetry and chemical modification. In addition, ligand binding properties were investigated using typical site-specific bound drugs (warfarin, phenylbutazone, ibuprofen and diazepam). RESULTS: Conformational changes, including N-B transition, clearly occurred in albumins from all species used in this study. The conformational stabilities of all the albumins were clearly lost in the weakly alkaline pH range. This was probably the result of the destruction of salt bridges between domain I and domain III in the albumin molecule. In addition, the profiles of the ANS-induced fluorescence were different and could be classified into two patterns, suggesting that hydrophobic pockets in the albumin molecules were different for the different species. The data suggest that the amino acid residues responsible for the transitions were some of the His residues located in domain I. Further, the ligand binding properties of the albumins were slightly different but statistically significant. CONCLUSIONS: The overall mechanisms of the N-B transition may be similar for all the albumins, but its impact is considerably different among the species in terms of both structural characteristics and ligand binding properties. Furthermore, the transitions appear to be multi-step transitions.     

Treatment of advanced gastroesophageal reflux disease with Collis gastroplasty and Belsey partial fundoplication

OBJECTIVE: To examine the factors affecting outcome in patients with advanced gastroesophageal reflux disease. DESIGN: Retrospective analysis. SETTING: University tertiary referral center. PATIENTS: Thirty-seven patients with advanced gastroesophageal reflux disease and no previous antireflux surgery. INTERVENTIONS: Thirty patients underwent Collis gastroplasty for esophageal lengthening and Belsey partial fundoplication. Seven patients with esophageal stricture and global loss of esophageal body motility who underwent primary esophagectomy and reconstruction were used as a comparison group. OUTCOME MEASURES: Symptomatic outcome in all 37 patients was assessed by questionnaire at a median of 25 months (range, 5-156 months) after surgery. In a subset of 11 patients undergoing the Collis-Belsey procedure, outcome was measured using 24-hour pH and results of motility studies. RESULTS: The Collis-Belsey procedure was successful in relieving symptoms of gastroesophageal reflux in 21 (70%) of the 30 patients. The outcome was excellent or good in 16 (89%) of 18 patients who presented with symptoms other than dysphagia, but only in 5 (42%) of 12 patients with dysphagia (P = .01). The outcome was particularly poor if dysphagia was associated with a previously dilated esophageal stricture. Persistent or induced dysphagia was the reason for failure in all but 1 patient. Results of 24-hour esophageal pH studies were returned to normal in 8 (73%) of 11 patients undergoing postoperative evaluation. Contraction amplitudes in the distal esophagus and the prevalence of simultaneous contractions in these segments did not change after the operation. All 7 patients who underwent primary esophagectomy were classified as having an excellent or good outcome and were relieved of their reflux symptoms, including dysphagia. Six of these could eat 3 meals per day and enjoyed an unrestricted diet. CONCLUSIONS: The outcome of the Collis-Belsey procedure in patients with advanced gastroesophageal reflux disease without dysphagia is excellent. It is less so in patients with dysphagia as a preoperative symptom. Esophagectomy can provide a good outcome in patients who have a combination of dysphagia stricture and a profound loss of esophageal motility.     

DNA loop domains in a 1.4-Mb region around the human hprt gene mapped by cleavage mediated by nuclear matrix-associated topoisomerase II

The aim of the study was the definition of the clinical features and survival of 27 resected cases of distal bile duct carcinoma. This neoplasm accounted for 14% of all periampullary malignancies treated by pancreaticoduodenectomy between 1990 and 1996. Jaundice was present in 96% of patients, but was the first symptom only in 78%. Preoperative investigations allowed to recognize distal bile duct cancer in a minority of patients (41%). Operative mortality and morbidity were 3.7 and 44%, respectively. Most patients (88%) were assigned to UICC stage IV-A. Postoperative survival was not significantly better than survival of 101 patients undergoing pancreaticoduodenectomy for pancreatic ductal carcinoma; median survival was 22 months, with a 13% 5-year survival rate. Determinants of a better prognosis were UICC stage 相似文献   

Imaging of spinal intradural arachnoid cysts: MRI, myelography and CT

Imaging studies in eight patients with surgically-confirmed spinal arachnoid cysts were analyzed retrospectively. All patients had preoperative MRI of the spine and seven preoperative myelography with postmyelographic CT. In all cases the correct diagnosis could be made preoperatively on the basis solely of MRI. The diagnosis could also be established from myelography and postmyelographic CT in six of the seven cases. In one case myelography and CT simply demonstrated an intradural extramedullary mass.     

Crystal structure of DCoH, a bifunctional, protein-binding transcriptional coactivator

DCoH, the dimerization cofactor of hepatocyte nuclear factor-1, stimulates gene expression by associating with specific DNA binding proteins and also catalyzes the dehydration of the biopterin cofactor of phenylalanine hydroxylase. The x-ray crystal structure determined at 3 angstrom resolution reveals that DCoH forms a tetramer containing two saddle-shaped grooves that comprise likely macromolecule binding sites. Two equivalent enzyme active sites flank each saddle, suggesting that there is a spatial connection between the catalytic and binding activities. Structural similarities between the DCoH fold and nucleic acid-binding proteins argue that the saddle motif has evolved to bind diverse ligands or that DCoH unexpectedly may bind nucleic acids.     

In vivo behaviour of rat band 3 cross-linked carrier erythrocytes

Inhibitory pathways in the spinal cord play an important role in establishing the pattern of motor discharge. In the wallaby spinal cord preparation, disruption of glycinergic and gamma-amino butyric acid (GABA)ergic neurotransmission abolished the alternation between antagonistic motor pools during fictive locomotion. A new pattern of motor discharge also appeared when both glycine and GABA(A) receptors were blocked simultaneously. This discharge pattern was biphasic, characterized by a distinct pause between two bursts of motoneurone firing during each cycle of motor activity. Whole cell patch recordings showed that the second burst of motor discharge was not caused by a separate inward current at a delayed time course. Furthermore, local injection of an N-methyl-D-aspartic acid (NMDA) specific antagonist converted the biphasic discharge to a continuous burst pattern. The result suggests an NMDA-mediated mechanism, which causes a suppression of motoneurone firing when glutamate release from interneurones is enhanced in the absence of glycinergic and GABAergic inhibition.