Accuracy of somatosensory evoked potentials in diagnosis of mild idiopathic carpal tunnel syndrome

OBJECTIVE: Concerning prevalence of carpal tunnel syndrome (CTS) and the difficulties with electromyography (EMG) and nerve conduction studies (NCS), this study was designed to evaluate the power of somatosensory evoked potential (SSEP) in CTS diagnosis among Iranian patients. PATIENTS AND METHODS: SSEP was performed on 100 asymptomatic hands of 50 healthy participants (40 female, age range 38-59 years) and on 61 hands of 46 patients (39 female, age range 34-58 years). Mean difference between N(20) latency of the middle finger and the wrist (median nerve innervation) as well as N(20) latency of the third finger and the fifth finger (ulnar nerve innervation) were measured. Using receiver operating characteristic (ROC) curve analysis, the upper limits of these variables were defined as 6.0 and 1.5 ms, respectively. Higher amounts in either of these variables were considered as positive SSEP for diagnosis of CTS. Measures of accuracy for SSEP were measured getting clinical diagnosis by two separate neurologists as the reference standard. In the patients' group who underwent both techniques of SSEP and EMG-NCS, kappa statistic as the agreement coefficient between two procedures was calculated. RESULTS: Sensitivity, specificity, and likelihood ratios for positive and negative results of SSEP in diagnosis of CTS were 70.4%, 91.0%, 7.83 and 0.32, respectively. Sensitivity of EMG-NCS in diagnosis of CTS was measured as 81.9%. Measure of agreement between two procedures (kappa) was calculated as 0.42. CONCLUSION: This study showed that positive results of SSEP might have a role in diagnosis of CTS. However, larger studies to demonstrate diagnostic power of SSEP in comparison with EMG-NCS seem necessary.     

Elevated endothelial microparticle-monocyte complexes induced by multiple sclerosis plasma and the inhibitory effects of interferon-beta 1b on release of endothelial microparticles, formation and transendothelial migration of monocyte-endothelial microparticle complexes

Monocyte migration through the disrupted cerebral endothelial cell (EC) junctions plays an essential role in formation of multiple sclerosis (MS) demyelinating lesions. During pathogenesis of MS, activated ECs release endothelial microparticles (EMP), which possibly facilitate transendothelial migration (TEMIG) of monocytes. To assess functional roles of EMP in MS, specifically, their (i) interaction with monocytes, (ii) effect on monocyte TEMIG in an in vitro model of the brain microvascular endothelial cells (BMVEC), (iii) phenotypic profiles of EMP elicited by MS plasma and (iv) the effects of IFN-beta 1b on release of EMP and on TEMIG of monocytes (mono) and monocytes:EMP complexes (mono:EMP) through the BMVEC. The effect of IFN-beta 1b on the release of EMP and the TEMIG of mono and mono:EMP was assessed by preincubating BMVEC cultures of IFN-beta 1b prior to addition of plasma. Three EMP phenotypes, CD54, CD62E and CD31 were assayed. Plasma specimens from 20 patients with relapsing remitting MS (11 in exacerbation, MS-E, and 9 in remission, ME-R) and 10 healthy controls were studied. Incubation of BMVEC with MS-E plasma yielded elevated levels of EMPCD54, EMP62E and EMPCD31 relative to MS-R and control plasmas. MS-E but not MS-R or control plasma also augmented TEMIG of monocytes, respectively. Mono:EMP complexes further augmented TEMIG relative to mono alone, but only in the presence of MS-E plasma; there was no significant effect with MS-R or control plasmas. The presence of IFN-beta 1b inhibited TEMIG of mono and mono:EMP by 20% and 30%, respectively. MS-E but not MS-R plasma elicited release of activation-derived EMP and enhanced TEMIG of mono and mono:EMP. IFN-beta 1b inhibited TEMIG and release of EMP, suggesting a role of EMP and a novel therapeutic mechanism for IFN-beta 1b in MS.     

Cervical carcinosarcoma: a case report

BACKGROUND: Carcinosarcomas are rare neoplasms of the female genital tract. They tend to be highly aggressive and are generally associated with a poor prognosis. Carcinosarcomas of the uterine cervix are extremely rare, with only approximately 35 cases previously reported in English. CASE: A 68-year-old woman presented with cervical carcinosarcoma. She remained without evidence of recurrent disease for 18 months after surgical resection and pelvic radiation treatment. CONCLUSION: In a review of all cases reported in the literature, it appears that cervical carcinosarcomas tend to present at an earlier stage than carcinosarcomas of the uterine corpus, therefore allowing early diagnosis and treatment. They may therefore be associated with a better overall prognosis than their counterparts in the corpus. Some studies have shown improved survival of patients of carcinosarcoma of the uterine corpus whose treatment included postoperative radiation and chemotherapy. Due to the better prognosis of cervical carcinosarcomas, we suggest studies to evaluate the role of aggressive, multimodal therapy, with the intent of obtaining a cure of cervical carcinosarcomas.     

Identification of a nonsense mutation in the very low-density lipoprotein receptor gene (VLDLR) in an Iranian family with dysequilibrium syndrome

We have investigated a consanguineous Iranian family with eight patients who suffer from mental retardation, disturbed equilibrium, walking disability, strabismus and short stature. By autozygosity mapping we identified one region with a significant LOD score on chromosome 9(p24.2-24.3). The interval contains the VLDLR gene, which codes for the very low-density lipoprotein receptor. This protein is part of the reelin signalling pathway, which is involved in neuroblast migration in the cerebral cortex and cerebellum. A homozygous deletion encompassing VLDLR has previously been found to cause a syndrome of cerebellar ataxia and mental retardation associated with cerebellar hypoplasia in the Hutterite population known as dysequilibrium syndrome (DES). The reported deletion however, contains an additional brain expressed gene of unknown function, whose involvement in the aetiology of the phenotype could so far not be excluded. We screened the coding region of VLDLR for mutations in our patients and found a homozygous c.1342C>T nucleotide substitution, which leads to a premature stop codon in exon 10. This is the first report of a mutation in patients with DES that affects VLDLR exclusively, confirming the central role of the very low-density lipoprotein receptor in the aetiology of this condition.     

Surgical treatment of metopic synostosis

Metopic synostosis is characterized by keel-shaped forehead (trigonocephaly), prominent midline ridge of the forehead, bitemporal narrowing, bilateral retrusion of supraorbits, egg-shaped orbits, low nasal dorsum, and reduced volume of the anterior cranial fossa. The mainstay treatment is early surgical intervention before the age of 12 months, which usually consists of bifrontal craniotomy with bilateral recontouring, lateral advancement, and lateral displacement of the superior orbital rims. Here, we have developed a new simplified technique for surgical treatment of trigonocephaly. A total of 60 cases of trigonocephaly were operated on between January 1995 and January 2010 by the first author. Surgical outcomes were evaluated 6 months after surgery using postoperative photographs and clinical examination notes, and scaling was made using the Whitaker classification. The evaluation showed that 85% of them were in class I, 11.6% were in class II, and 3.3% were in class III. No case was in class IV. Only the last 10 cases received the new surgical technique, and all were in class I. Complication rate was 38.3% for all cases and was only 20% for the last 10 cases, that is, the new technique group. Revision rate for trigonocephaly surgery was 13.3%, and the most common reason was hardware removal. None of the patients from the new technique group underwent revision surgery. We believe that our new technique is fast and easy, can provide sufficient bone graft, and is more useful for older patients (>1 y). Early postoperative results have been promising.     

Effects of GABAergic inhibition on neocortical long-term potentiation in the chronically prepared rat

In this study we investigated the hypothesis whether P2-related differences tested in a visual priming paradigm are associated with theta phase-locking. We recorded the EEG from 31 electrodes and calculated phase-locking index and total power differences for frequencies between 2 and 20 Hz. ERPs (event-related potentials) were analyzed for P1, N1 and P2 components. P2 showed strongest task-related amplitude differences between congruent and incongruent targets. A source analyses was performed for the P2 component using sLoreta that revealed local generators of the P2 in parieto-occipital regions. Phase-locking analyses showed specific effects in the theta range (4-6 Hz) appearing in time windows at around the P2 component. We draw the conclusion that phase-locked theta reflect top-down regulation processes mediating information between memory systems and is in part involved in the modulation of the P2 component.