[Cavitary lesions in multiple sclerosis: multicenter study on twenty patients]. / Formes cavitaires de sclérose en plaques: étude multicentrique sur vingt patients.

INTRODUCTION: Cavitary white matter changes are mainly described in leukodystrophies and especially in vanishing white matter disease. Large cavitary lesions are not typical for multiple sclerosis (MS). METHODS: We studied MS patients with large cavitary brain lesions. Patient characteristics, disease onset/duration/subtype, expanded disability status scale (EDSS), mini mental state (MMS), vanishing white matter disease genetic analysis, and MRI characteristics of the cavitary lesions were analyzed. RESULTS: Twenty patients were analyzed (6 men and 14 women). Mean age at disease onset was 37.6 (range 17-58). Mean disease duration was 10 years (range 2-20). Five patients had initial relapsing-remitting MS and nine patients had primary-progressive MS. Mean EDSS was 5.5 (range 2-8). Mean MMS was 20/30. Vanishing white matter disease genetic analysis was performed and negative in seven patients. Inferior corpus callosum lesions were seen in all patients with available sagittal FLAIR sequences. Cavitary lesions were strictly supratentorial, and located inside the diffuse leukoencephalopathy, with often a posterior predominance. CONCLUSION: MS patients with large cavitary lesions seem to represent a MS subgroup, predominantly women, with relatively late disease onset, predominantly primary-progressive type, relatively high EDSS scores, and severe cognitive dysfunction.

Synthesis and biological evaluation of cyclic nitrogen mustards based on carnitine framework.

Two series of cyclic nitrogen mustards structurally related to L-carnitine have been prepared. The cytotoxic activity of these compounds was evaluated by using Chlorambucil as a reference. In accordance with earlier report, the cytotoxicity is in direct correlation with the lipophilicity of the introduced alkyl chains. Among the cyclic nitrogen mustards synthesized, the most cytotoxic compounds were the one acylated with a palmitoyl side chain, which showed activities comparable to that of Chlorambucil.

Contrast-to-noise ratios of liver lesions using subtraction imaging on multiphase 64-detector row CT.

AIM: To measure contrast-to-noise ratios of liver lesions on conventional enhanced and digitally subtracted multidetector row computed tomography (CT) images. MATERIALS/METHODS: This study was approved by our hospital internal review board (IRB) and all collected data were evaluated in a Health Insurance Portability and Accountability Act (HIPAA)-compliant manner. Subtracted datasets, using pixel-by-pixel subtraction of the post-contrast images from the pre-contrast images, were created from the 64 detector-row CT of patients undergoing three-phase examination of the liver (unenhanced, arterial phase, and portal venous phase). Regions of interest were used to calculate the contrast-to-noise ratios between the lesions and the background liver parenchyma on both the post-contrast and subtracted datasets using the following formula: (Lesion mean (HU) - Liver mean (HU))/standard deviation of mean outside patient (HU). These ratios were compared using a mixed linear statistical model. RESULTS: Contrast-to-noise ratios were calculated for 64 lesions in 50 consecutive patients. Of the 64 lesions, 42 were hypervascular and 22 were hypovascular. Subtracted datasets yielded statistically significant higher contrast-to-noise ratios of hypervascular lesions compared to normal liver parenchyma (p<0.0001). Subtraction did not yield a statistically significant improvement in contrast-to-noise ratios for hypovascular liver lesions (p=0.16). CONCLUSION: Post-processed subtraction CT images generate increased contrast-to-noise ratios for hypervascular liver lesions. As this technique is easy to perform and does not involve additional radiation exposure, it should be considered when evaluating for suspected hypervascular lesions.

Valvular heart disease in patients with Parkinson's disease treated with pergolide. Course following treatment modifications.

UNLABELLED: Valvular heart abnormalities have been reported in patients with Parkinson's disease (PD) treated with pergolide. However, the incidence and severity of these abnormalities vary from study to study and their course after drug withdrawal has not been systematically assessed. OBJECTIVES: To estimate the frequency and severity of valvular heart abnormality and its possible reversibility after drug withdrawal in a case-control study. METHODS: All PD patients in the Amiens area treated with pergolide were invited to attend a cardiologic assessment including transthoracic echocardiography. Thirty PD patients participated in the study. A second echocardiography was performed (median interval: 13 months) after pergolide withdrawal (n=10 patients). Controls were age- and sex-matched non-PD patients referred to the cardiology department. RESULTS: Compared to controls, aortic regurgitation (OR: 3.1; 95% IC: 1.1-8.8) and mitral regurgitation (OR: 10.7; 95% IC: 2.1-53) were more frequent in PD patients (tricuspid: NS). The number of affected valves (n=2.4+/-0.7) and the sum of regurgitation grades (n=2.8+/-1.09) were higher (p=0.008 and p=0.006, respectively) in the pergolide group. Severity of regurgitation was not correlated with pergolide cumulative dose. A restrictive pattern of valvular regurgitation, suggestive of the role of pergolide, was observed in 12/30 (40%) patients including two with heart failure. Pergolide was discontinued in 10 patients with valvular heart disease, resulting in a lower regurgitation grade (p=0.01) at the second transthoracic echocardiography and the two patients with heart failure returned to nearly normal clinical examination. This study supports the high frequency of restrictive valve regurgitation in PD patients treated with pergolide and reveals that a significant improvement is usual when the treatment is converted to non-ergot dopamine agonists.

Solitary fibrous tumor of the buccal space: treatment with percutaneous cryoablation.

Solitary fibrous tumors are rare spindle cell neoplasms that typically occur in the thorax but have been described in various locations within the abdomen and head and neck region. The most common extrapleural site is the oral cavity, but these tumors have been also described in the orbit, nasopharynx, paranasal sinuses, salivary glands, and larynx. We describe a case of a solitary fibrous tumor of the buccal space successfully treated with percutaneous CT-guided cryoablation.

Biotinylation of glycan chains in beta2 glycoprotein I induces dimerization of the molecule and its detection by the human autoimmune anti-cardiolipin antibody EY2C9.

Binding of beta2GPI (beta2 glycoprotein I), a human plasma protein, to AnPLs (anionic phospholipids) plays a key role in the formation of antiphospholipid antibodies involved in autoimmune diseases like antiphospholipid syndrome or systemic lupus erythematosus. We recently showed that binding of beta2GPI to AnPLs was enhanced by biotinylation of its glycan chains with biotin-hydrazide. In the present study, we investigated why this chemical modification of beta2GPI increased both its affinity for AnPLs and its recognition by anti-cardiolipin antibodies. Electrophoretic analysis showed that: (i) high molecular mass beta2GPI (dimers and other oligomers) covalently coupled by imine bonds, were present in variable amounts in oxidized beta2GPI and in beta2GPI-bh (beta2GPI-biotin-hydrazide), but were absent in native beta2GPI; (ii) binding of beta2GPI-bh to phosphatidylserine-coated microtitre plates generated high molecular mass polymers in a time-dependent manner. Native beta2GPI did not polymerize in these conditions. These polymers did not bind more strongly to AnPLs than the monomer beta2GPI. However, in solution at 1 microM beta2GPI-bh essentially appeared as a dimer as revealed by light-scattering analysis. SPR (surface plasmon resonance) analysis showed that the increased affinity of beta2GPI-bh for AnPL monolayers was due to a lower dissociation rate constant compared with native beta2GPI. Finally, the monoclonal human aCL (auto-immune anti-cardiolipin antibody) EY2C9 bound to beta2GPI-bh but did not bind to monomeric native and oxidized beta2GPI. It is likely that the dimeric quaternary structure of beta2GPI-bh is in fact responsible for the appearance of the epitopes targeted by the EY2C9 antibody.

Radiofrequency ablation of bony metastatic disease.

Image-guided radiofrequency (RF) ablation is an emerging alternative therapy for treatment of cancer in patients who can not undergo surgical resection. We have performed RF ablation on patients to locally treat and palliate painful osseous metastatic disease for several years. We present an overview of various applications of RF ablation for treating osseous metastatic disease.

Radiofrequency ablation of regional recurrence from well-differentiated thyroid malignancy.

BACKGROUND: Regional recurrence of well-differentiated thyroid cancer (WTC) is primarily detected with ultrasonography (US), and current treatment is surgical. Radiofrequency ablation (RFA) has been used primarily for liver tumors as an alternative to a surgical procedure. We have applied RFA to a group of patients with locally recurrent WTC. METHODS: Eight patients underwent percutaneous RFA for biopsy-proven recurrent WTC in the neck (mean size, 2.4 cm; range, 0.8-4.0 cm) while under intravenous conscious sedation and with US guidance. The RF electrode was inserted into the site of recurrence and treated with the maximum allowable current for between 2 and 12 minutes. Follow-up consisted of US in 8 patients, thyroglobulin levels in 6 patients, biopsy in 4 patients, and surgical treatment in 2 patients. RESULTS: All 8 patients with no bleeding or infectious complications were treated as outpatients. A minor skin burn and 1 vocal cord paralysis occurred. No recurrent disease at the treatment site was detected, with a mean follow-up of 10.3 months. Histological examination showed no evidence of a tumor in the treated lymph nodes in 6 patients. Follow-up US examinations showed disappearance of previously detected color Doppler flow, as well as mass shrinkage and internal cystic change, or both. CONCLUSIONS: US-guided RFA is an exciting new treatment modality that appears to have a future role in treating locally recurrent WTC.

CT of the duodenum: an overlooked segment gets its due.

Abdominal computed tomography (CT) is frequently performed to evaluate gastrointestinal pathologic conditions, and the majority of the gastrointestinal radiology literature has concentrated on the colon, stomach, and distal small bowel. In a description of CT findings of duodenal pathologic conditions, congenital, traumatic, inflammatory, and neoplastic diseases are presented. Congenital duodenal anomalies such as duplications and diverticula are usually asymptomatic, while annular pancreas and malrotation may manifest in the 1st decade of life. CT plays a vital role in the diagnosis of traumatic duodenal injury. Primary inflammatory processes of the duodenum such as ulcers and secondary involvement from pancreatitis can reliably be diagnosed at CT. Infectious diseases of the duodenum are difficult to diagnose, as the findings are not specific. While small bowel malignancies are relatively rare, lipoma, adenoma, and adenocarcinoma, as well as local extension from adjacent malignancies, can be diagnosed at CT. Careful CT technique and attention to the duodenum can result in reliable prospective diagnoses.

Radiofrequency treatment of hepatic neoplasms in patients with permanent pacemakers.

Clinicians who provide care for patients with implantable devices for rhythm management, ie, pacemakers and internal cardioverter defibrillators, must be aware of sources of interference that could affect device function. Intracardiac radiofrequency is a recognized source of potential interference. However, radiofrequency to extracardiac sites that are relatively close to the implanted device has not been investigated thoroughly. We present 2 patients with permanent pacemakers undergoing intrahepatic radiofrequency for the treatment of metastatic disease. No interference was documented in either patient. Additional in vitro and in vivo studies are needed to determine definite clinical guidelines for such patients.

Pre-operative localization of parathyroid adenomas: a comparison of power and colour Doppler ultrasonography with nuclear medicine scintigraphy.

AIM: To compare power and colour Doppler ultrasonography (US) with nuclear medicine scintigraphy (NM) in the preoperative localization of parathyroid adenomas in patients with primary hyperparathyroidism (PHPT). MATERIALS AND METHODS: Thirty-one patients with biochemical evidence of PHPT underwent pre-operative US and NM for parathyroid adenoma localization. Both studies were interpreted independently without prior knowledge of the other study's findings. All patients had surgical removal of the parathyroid adenoma utilizing standard neck exploration or minimally invasive unilateral surgical techniques with rapid serum assay of circulating parathyroid hormone levels. RESULTS: All patients had single parathyroid adenomas at surgery. Prospective sensitivities for US, NM and both studies combined were 65%, 68%, and 74%, respectively, with a positive predictive value of 100% each. The adenoma was localized by only one imaging modality in 16% of cases. CONCLUSIONS: US and NM provide complementary roles in the pre-operative localization of parathyroid adenomas in patients with PHPT.

Mapping, characterization, and expression analysis of the SM-20 human homologue, c1orf12, and identification of a novel related gene, SCAND2.

Major psychosis was shown to segregate with a balanced translocation (1q42.1; 11q14.3) in a multigenerational family. This study describes the identification of a human SM-20 homologue gene that lies at about 400 kb on the centromeric side of the 1q42.1 breakpoint. The full-length cDNA sequence and gene structure were determined. Expression analysis was performed, showing high expression levels in skeletal and cardiac muscles; in the central nervous system, expression was restricted to dopaminergic neurons and spinal motoneurons. A second gene displaying high sequence similarity with SM-20 was also identified by BLAST. This gene, located on chromosome 15, is likely to have evolved by retroposition of SM-20 mRNA and an exon-shuffling mechanism. It encodes a 306-amino-acid protein harboring strong homology with an N-terminal motif found in some zinc-finger proteins. This gene was named SCAND2 (SCAN domain-containing 2).