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Selected AbstractsNeuromagnetic Source Localization of Epileptiform Activity in Patients with Graphogenic EpilepsyEPILEPSIA, Issue 11 2006Naoaki Tanaka Summary:,Purpose: To clarify the source localization of epileptiform activity by using magnetoencephalography (MEG) in patients with graphogenic epilepsy. Methods: MEG and simultaneous EEG were recorded with a 204-channel whole-head MEG system in two patients with graphogenic epilepsy. During the MEG recordings, the patients performed a set of tasks comprising mental arithmetic calculation, speaking, moving the right arm in a manner resembling writing, writing, and thinking of writing. Equivalent current dipoles (ECD) were calculated for epileptiform discharges on MEG by using a single-dipole model. The ECD were superimposed on the magnetic resonance images of the patients. Results: The task of writing provoked seizures, in which both patients jerked the right arms. Thinking of writing also induced these seizures. In both patients, EEG associated with the seizures showed bursts of spike-and-slow-wave complexes predominantly in the centroparietal region. MEG also showed epileptiform discharges corresponding to the EEG bursts. ECDs obtained from the discharges were clustered in the left centroparietal area. Conclusions: Thinking of writing was a trigger for the seizures, as well as the task of writing. The source of the epileptiform discharge associated with the seizures was localized in the unilateral centroparietal area. The findings suggest that the centroparietal region plays an important role in the pathophysiology underlying these two graphogenic epilepsy cases. [source] Movement-Induced Focal Motor Seizures and Choreoathetosis As- sociated with Nonketotic Hyperglycemia: A Case ReportEPILEPSIA, Issue 2000Hisashi Tanaka Case Report: We report the case of a diabetic woman who developed right-sided reflex seizures and bilateral choreoathetosis during an episode of nonketotic hyperglycemia. The patient was a 67-year-old woman with a 14-year history of HCV-related liver cirrhosis who experienced polydipsia and polyuria in January 1998. She began to have episodes of abnormal hyperkinetic movements of the right upper extremity and tonic-clonic seizures in the right arm triggered by voluntary movements of right or bilateral arms in the beginning of March 1998. The seizures increased in frequency and consequently left her disabled. She was admitted to our hospital with complaints of these abnormal motor phenomena on March 9, 1998. Neurological examinations revealed that she was alert, well-oriented, and that cranial nerve functions were normal. Slight motor weakness of the right upper limb and deep tendon hyporeflexes were observed in all extremities. Sensations and cerebellar functions were intact. Choreic or athetotic involuntary movements were seen in the bilateral upper limbs and neck. These involuntary movements were increased by voluntary movement or posturing of the upper limbs. The focal tonic-clonic seizures were easily triggered by voluntary movements such as knotting a cord. This seizure suddenly began by tonic movements in the right upper limb and gradually progressed to the right hemi-face and neck without loss of consciousness. The average duration of seizures was about one minute. The laboratory data demonstrated mild leukocytopenia, thrombocytopenia, hepatic dysfunction, and hyperglycemia without ketosis. Fasting blood glucose was 41 I mg/dl, and HbAlc was 14.5%. Blood ammonia was within normal levels. Cranial CT revealed no abnormalities. Brain MRI on T I-weighted images demonstrated bilateral high signal intensity in the putamen. An interictal EEG revealed a symmetrical slow background activity of 7,8 Hz. An ictal EEG recording showed a 2.5 4 Hz irregular sharp and slow wave discharge in the bilateral frontal-central regions. Treatment with carbamazepine was ineffective for the seizures. However, the seizures completely disappeared after the administration of insulin on March 17. Under good control of the hyperglycemia, the abnormal involuntary movements decreased gradually and then completely disappeared; the patient became neurologically asymptomatic by March 30. The follow-tip EEG demonstrated 9-Hz alpha background activity without any epileptic discharges. Conclusions: Nonketotic hyperglycemia has been rarely reported to cause stimulus-induced seizures or hyperkinetic involuntary movements such as hemichorea-ballism. To our knowledge, this is the first reported case of both induced seizures and involuntary movements simultaneously caused by hyperglycemia. Movement-induced seizures and choreoathetoid movements in this patient can be considered to result from transiently-increased activity in the basal ganglia and/or cerebral cortex associated with metaholic disorders. [source] Effect of tourniquet pressure and intra-individual variability on plasma fibrinogen, platelet P-selectin and monocyte tissue factorINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 6 2000J. L. Ritchie Small differences in levels of certain haemostatic components may be clinically significant. It is important therefore to eliminate potential sources of confounding variability. This study investigated the effect of removing tourniquet pressure prior to sample collection on plasma fibrinogen levels, platelet P-selectin and monocyte tissue factor expression. Blood was collected from the right arm under maintained tourniquet pressure and from the left arm following the release of pressure once the vein was sufficiently inflated for insertion of a needle. Whole blood was labelled within one hour of venepuncture to allow analysis of platelet P-selectin and monocyte tissue factor by flow cytometry. Plasma fibrinogen levels were analysed in samples stored at ,70 °C, for all individuals at the end of the study using a method based on the Clauss technique. Intra-individual variability for each of the components was assessed by collecting samples under tourniquet pressure from four individuals on the same day on three consecutive weeks. Intra-individual variations were greater than assay CVs for all three components. There were no significant differences between the two tourniquet methods of collection for fibrinogen, P-selectin or tissue factor. In conclusion, there is no reason not to use a tourniquet during collection of blood for analysis of plasma fibrinogen, platelet P-selectin or monocyte tissue factor. [source] Ultrasonographic detection of spontaneous rupture in the Biceps tendon of a patient with rheumatoid arthritisINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 4 2007Hyun-Sook KIM Abstract Ultrasonography is a useful imaging modality for the diagnosis of pathologic conditions in the biceps tendon. We describe a 64-year old man with rheumatoid arthritis (RA) who suddenly developed a painless lump in the antecubital region of the right arm. The bulging was prominent when he flexed his forearm. Proximal loss of the long head in biceps tendon could be demonstrated by ultrasonography. The ultrasonographic features of spontaneous isolated biceps tendon rupture occurring in a patient with RA are described. [source] Primary Cutaneous Immunocytoma Presenting with Diffuse Subclinical Involvement and Demonstrating Kappa and Lambda Light Chain RestrictionsJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005C. Lorenzo A 37 year-old man with primary cutaneous immunocytoma with lambda light chain restriction involving the left shoulder was treated with Rituxan with clinical resolution. Four years later, he presented with a six-month history of diffuse asymptomatic erythema. Physical examination revealed three grouped papules on the right upper arm and an irregular sclerotic patch with slight erythema superiorly on the left shoulder at the site of the initial tumor. There was no diffuse erythema. Biopsies were obtained from a papule on the right upper arm, the sclerotic patch on the left shoulder, and clinically uninvolved skin on the right anterior thigh. All three specimens showed a variably dense, predominantly periadnexal and perivascular dermal infiltrate of plasma cells and lymphocytes. The specimen from the right arm demonstrated kappa light chain restriction. The specimen from the right thigh showed lambda light chain restriction. Physical examination six weeks later demonstrated mottled erythema on the anterior thighs. Two biopsies were obtained from the right thigh. One showed immunocytoma. The other was unremarkable. The patient's primary cutaneous immunocytoma demonstrated two unusual findings: (1) histologic presence of tumor in clinically uninvolved and minimally involved skin; and (2) the presence of two distinct monoclonal populations. [source] CD34+ Pigmented Fibrous Proliferations: The Morphologic Overlap Between Pigmented Dermatofibromas and Bednar TumorsJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005J. Chu McAllister Pigmented dermatofibrosarcoma protuberans (Bednar tumor) constitute 5,10% of all cases of dermatofibrosarcoma protuberans, and are usually considered mimics of melanocytic proliferations rather than fibrous lesions. We report two cases of pigmented fibrous proliferations that demonstrate features of both dermatofibromas and DFSP. The first case is a 19-year-old man with a three year history of a slowly growing pigmented lesion on the right arm. On clinical exam the lesion was a 7 mm firm pigmented papulonodular lesion. The second case is a 31-year-old woman with a 4,5 year history of a slowly enlarging, asymptomatic ,dark area' on the right buttock. On clinical exam the lesion is a 2 cm darkly pigmented flat nodule. Morphologically both lesions are primarily dermal proliferations of spindled cells admixed with pigmented dendritic melanocytes. The lesional cells trap collagen fibers at the periphery and there is basal cell hyperpigmentation. Adnexal structures are effaced but significant trapping of subcutaneous fat is not present. By immunohistochemistry both lesions show focal CD34 positivity but are negative for Factor XIIIa and melanocytic markers. Although overlap between dermatofibromas and DFSP is well documented in the literature, pigmented fibrous lesions with features of both entities are not well described. [source] Bilateral common carotid occlusion without neurological deficitJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 4 2002Serdar Karaköse Summary A 40-year-old man presented with pain and numbness in his right arm. On his clinical examination, no neurological deficit was found. Bilateral common carotid artery duplex sonography scan demonstrated no flow in either lumen. No abnormality was recognized on brain CT. On cerebral digital substraction angiogram, total occlusion of the brachiocephalic trunk and left carotid artery were shown. There was a modest stenosis in the left vertebral artery. Collateral circulation feeding the intracranial carotid system mainly originated from the left vertebrobasilar system. Previous cases of bilateral carotid occlusion are reviewed and discussed. [source] Angiotensin-converting enzyme gene insertion/deletion polymorphism frequency in normotensive children with a positive family history of essential hypertensionJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 12 2009Lale Camci Aim: To evaluate the possible relationship between blood pressure (BP) and angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism in normotensive children with a positive family history of essential hypertension (EHT). Material and Methods: Three hundred seventy-six randomly selected normotensive schoolchildren (147 boys, 229 girls) between the ages of seven and 17 years were enrolled. Children were subdivided into a ,first-degree relative group' and a ,second-degree relative group' according to the presence of EHT in parents or grandparents, respectively. BP was measured twice from the right arm and the systolic BP, diastolic BP and mean BP were recorded. ACE gene I/D polymorphism was performed from all studied children and frequency od DD, ID and ID allele were analysed in each study group. Results: Allelic frequencies of the DD genotype of the ACE gene were higher in children with a positive history in the first- (36.2%) and second-degree (38.3%) relatives for EHT than the controls (30.7%) (P < 0.05 for both). Children with a positive family history of EHT and a DD genotype, had significantly higher SBP, DBP and MBP levels (P < 0.05) than the children with ID or II genotypes. Conclusion: We found that the ACE gene DD genotype was common and that BP levels were higher in Turkish children with a positive family history of EHT and DD genotype. Because the presence of DD allele might be the one of the potential contributor of EHT pathogenesis, further studies needed in large cohort for long term follow-up for EHT in children with DD allele. [source] Genetic analysis of the strong gyrase site (SGS) of bacteriophage Mu: localization of determinants required for promoting Mu replicationMOLECULAR MICROBIOLOGY, Issue 4 2000M. L. Pato The Mu strong gyrase site (SGS), located in the centre of the Mu genome, is required for efficient Mu replication, as it promotes synapsis of the prophage termini. Other gyrase sites tested, even very strong ones, were unable to substitute for the SGS in Mu replication. To determine the features required for its unique properties, a deletion analysis was performed on the SGS. For this analysis, we defined the 20 bp centred on the midpoint of the 4 bp staggered cleavage made by gyrase to be the ,core' and the flanking sequences to be the ,arms'. The deletion analysis showed that (i) ,,40 bp of the right arm is required, in addition to core sequences, for both efficient Mu replication and gyrase cleavage; and (ii) the left arm was not required for efficient Mu replication, although it was required for efficient gyrase cleavage. These observations implicated the right arm as the unique feature of the SGS. The second observation showed that strong gyrase cleavage and Mu replication could be dissociated and suggested that even weak gyrase sites, if supplied with the right arm of the SGS, could promote Mu replication. Hybrid sites were constructed with gyrase sites that could not support efficient Mu replication. The SGS right arm was used to replace one arm of the strong pSC101 gyrase site or the weaker pBR322 site. The pSC101 hybrid site allowed efficient Mu replication, whereas the pBR322 hybrid site allowed substantial, but reduced, replication. Hence, it appears that optimal Mu replication requires a central strong gyrase site with the properties imparted by the right arm sequences. Possible roles for the SGS right arm in Mu replication are addressed. [source] Complete Recovery From Intractable Complex Regional Pain Syndrome, CRPS-Type I, Following Anesthetic Ketamine and MidazolamPAIN PRACTICE, Issue 2 2007Ralph-Thomas Kiefer MD Abstract Objective: To describe the treatment of an intractable complex regional pain syndrome I (CRPS-I) patient with anesthetic doses of ketamine supplemented with midazolam. Methods: A patient presented with a rapidly progressing contiguous spread of CRPS from a severe ligamentous wrist injury. Standard pharmacological and interventional therapy successively failed to halt the spread of CRPS from the wrist to the entire right arm. Her pain was unmanageable with all standard therapy. As a last treatment option, the patient was transferred to the intensive care unit and treated on a compassionate care basis with anesthetic doses of ketamine in gradually increasing (3,5 mg/kg/h) doses in conjunction with midazolam over a period of 5 days. Results: On the second day of the ketamine and midazolam infusion, edema, and discoloration began to resolve and increased spontaneous movement was noted. On day 6, symptoms completely resolved and infusions were tapered. The patient emerged from anesthesia completely free of pain and associated CRPS signs and symptoms. The patient has maintained this complete remission from CRPS for 8 years now. Conclusions: In a patient with severe spreading and refractory CRPS, a complete and long-term remission from CRPS has been obtained utilizing ketamine and midazolam in anesthetic doses. This intensive care procedure has very serious risks but no severe complications occurred. The psychiatric side effects of ketamine were successfully managed with the concomitant use of midazolam and resolved within 1 month of treatment. This case report illustrates the effectiveness and safety of high-dose ketamine in a patient with generalized, refractory CRPS. [source] Hypermelanocytic guttate and macular segmental hypomelanosisBRITISH JOURNAL OF DERMATOLOGY, Issue 3 2004W. Westerhof Summary We report two sisters, 27 and 30 years of age, with a cutaneous pigmentary anomaly, which seems to be a new entity. At the age of 26 years the elder sister developed an asymptomatic and persistent rash consisting of discrete, grouped, round to oval, guttate and nummular, hypopigmented macules, 0·2,5 cm in diameter. The distribution of the lesions was unilateral. They were located on the right side of the thorax with a moderately sharp demarcation in the mid-line and ran in a segmental distribution over the right arm, hand and fingers. Microscopic examination of lesional skin scrapings was negative for fungi. Examination with Wood's light accentuated the lesions from the surrounding normal skin. The younger sister had experienced identical, mostly guttate, skin lesions for many years, which at examination were distributed on all extremities and buttocks, and to a lesser degree on the trunk, but here in a segmental distribution. Histological examination (Masson,Fontana staining) of lesional skin of both sisters was identical. A slightly thinned epidermis and a marked decrease in pigmentation of the epidermal basal layer was seen. Electron microscopic examination of lesional skin showed an overall linear increase of morphologically and cytologically normal melanocytes just above the epidermal basal membrane. At many places the density of melanocytes was so high that the keratinocytes were displaced from the basal layer. The melanocytic dendrites extended into the suprabasal layer. The keratinocytes of lesional skin showed a decreased number of melanosomes. It is paradoxical that a hypomelanotic macule shows a histological picture of an increase in normal functioning melanocytes. In all probability a deficient melanosome transfer is responsible for this unexpected phenomenon. [source] Neuromagnetic Source Localization of Epileptiform Activity in Patients with Graphogenic EpilepsyEPILEPSIA, Issue 11 2006Naoaki Tanaka Summary:,Purpose: To clarify the source localization of epileptiform activity by using magnetoencephalography (MEG) in patients with graphogenic epilepsy. Methods: MEG and simultaneous EEG were recorded with a 204-channel whole-head MEG system in two patients with graphogenic epilepsy. During the MEG recordings, the patients performed a set of tasks comprising mental arithmetic calculation, speaking, moving the right arm in a manner resembling writing, writing, and thinking of writing. Equivalent current dipoles (ECD) were calculated for epileptiform discharges on MEG by using a single-dipole model. The ECD were superimposed on the magnetic resonance images of the patients. Results: The task of writing provoked seizures, in which both patients jerked the right arms. Thinking of writing also induced these seizures. In both patients, EEG associated with the seizures showed bursts of spike-and-slow-wave complexes predominantly in the centroparietal region. MEG also showed epileptiform discharges corresponding to the EEG bursts. ECDs obtained from the discharges were clustered in the left centroparietal area. Conclusions: Thinking of writing was a trigger for the seizures, as well as the task of writing. The source of the epileptiform discharge associated with the seizures was localized in the unilateral centroparietal area. The findings suggest that the centroparietal region plays an important role in the pathophysiology underlying these two graphogenic epilepsy cases. [source] Paradoxical Vasodilation During Lower Body Negative Pressure in Patients with Vasodepressor Carotid Sinus SyndromeJOURNAL OF AMERICAN GERIATRICS SOCIETY, Issue 6 2003Arduino A. Mangoni MD OBJECTIVES: To elucidate the pathophysiological mechanism of the vasodepressor form (VD) of carotid sinus syndrome (CSS) by maneuvers designed to induce generalized sympathetic activation after baroreceptor unloading (lower body negative pressure, LBNP) or direct peripheral adrenoreceptor stimulation via local administration of norepinephrine (NA). DESIGN: Subjects were identified with VD of CSS through diagnostic testing. SETTING: Research laboratory. PARTICIPANTS: Eleven young controls (YC) (mean age ± standard error of mean = 22.8 ± 0.7), eight elderly controls (EC) (72.6 ± 0.6), and eight elderly patients with VD (78.7 ± 1.7). MEASUREMENTS: Forearm arterial blood flow (FABF) was measured in the left and right arms by venous occlusion plethysmography. Measurements were performed during baseline conditions, LBNP (,20 mmHg), and intra-arterial NA infusion in the left brachial artery at three progressively increasing rates (60, 120, and 240 pmol/min). RESULTS: During LBNP, FABF significantly decreased in YC (baseline 3.61 ± 0.30 vs ,20 mmHg 2.96 ± 0.24 mL/100 g/min, P = .030) and EC (4.05 ± 0.74 vs 3.69 ± 0.65 mL/100 g/min, P = .033) but increased in elderly patients with VD (3.65 ± 0.60 vs 4.54 ± 0.80 mL/100 g/min, P = .020). During NA infusion, a significant forearm vasoconstriction occurred in YC (FABF left:right ratio 1.00 ± 0.05 at baseline; 0.81 ± 0.08 at 60 pmol/min, P = .034; 0.81 ± 0.05 at 120 pmol/min, P < .001; 0.72 ± 0.04 at 240 pmol/min, P < .001), whereas no significant FABF changes were observed in EC (1.04 ± 0.06; 0.96 ± 0.07, P = .655; 0.89 ± 0.10, P = .401; 0.94 ± 0.10, P = .590) or elderly patients with VD (1.04 ± 0.06; 1.16 ±0 .10, P = .117; 1.04 ± 0.08, P = .602; 1.11 ± 0.10, P = .305). CONCLUSION: VD of CSS is associated with a paradoxical vasodilatation during LBNP and an impairment of peripheral ,-adrenergic responsiveness, which may be age-related. [source] MukB colocalizes with the oriC region and is required for organization of the two Escherichia coli chromosome arms into separate cell halvesMOLECULAR MICROBIOLOGY, Issue 6 2007Olessia Danilova Summary The circular Escherichia coli chromosome is organized by bidirectional replication into two equal left and right arms (replichores). Each arm occupies a separate cell half, with the origin of replication (oriC) at mid-cell. E. coli MukBEF belongs to the ubiquitous family of SMC protein complexes that play key roles in chromosome organization and processing. In mukBEF mutants, viability is restricted to low temperature with production of anucleate cells, reflecting chromosome segregation defects. We show that in mukB mutant cells, the two chromosome arms do not separate into distinct cell halves, but extend from pole to pole with the oriC region located at the old pole. Mutations in topA, encoding topoisomerase I, do not suppress the aberrant positioning of chromosomal loci in mukB cells, despite suppressing the temperature-sensitivity and production of anucleate cells. Furthermore, we show that MukB and the oriC region generally colocalize throughout the cell cycle, even when oriC localization is aberrant. We propose that MukBEF initiates the normal bidirectional organization of the chromosome from the oriC region. [source] | ||||||||||||||||||||||