Physicians awareness on primary immunodeficiency disorders in Iran.

Primary immunodeficiency diseases (PIDs) consist of a group of genetic disorders that predispose the patients to immune-mediated complications. The aim of this study was to assess the knowledge of Iranian general practitioners and pediatricians about PIDs.A questionnaire consisting 52 closed questions on clinical symptoms, laboratory data, associated syndromes and management of PIDs patients was made valid and reliable by a pair pilot study. Then the questionnaire was filled by pediatricians, general practitioners and pediatric residents from different regions of Iran. Totally, 333 physicians (50 general practitioners, 52 pediatric residents, 182 pediatric specialists, and 49 pediatric sub specialists) participated in this study. The mean total score was 55.9±14.3 (i.e. about 29 correct answers out of 52 questions). One hundred and five participants (31.9%) answered correctly more than two third of all questions. In order to qualitatively compare the groups a ranking system was used. Total scores was significantly different between physicians groups (p<0.01). Pediatric subspecialties gained the highest rank, which was significantly over the other participants (p<0.05). This study showed that there is a considerable lack of awareness on PIDs in physicians. This may be one of the major reasons in late diagnosis and the delay in adequate treatment deteriorating patients' morbidity and mortality. Retraining classes and reconsidered educating schedules are needed as an efficient strategies and improving physicians' knowledge about PIDs.

The effects of voxel localization and time of echo on the diagnostic accuracy of cystic brain tumors in 3 tesla magnetic resonance spectroscopy.

BACKGROUND: Although magnetic resonance spectroscopy (MRS) has been shown as an effective diagnostic tool in distinguishing inflammation from neoplasm in cystic brain lesions, the optimum approach in selecting the portions of lesions in MRS and the possible effects of different times of echoes (TEs) remains unknown. OBJECTIVES: To determine the most effective TE in diagnosing neoplastic lesions based on detecting choline (Cho), N acetyl aspartate (NAA) and creatinine (Cr). Moreover, the role of voxel localization on the diagnosis of the neoplastic nature of the lesions is assessed through comparing the abovementioned metabolite ratios in the rim and center of each lesion with the same TE. PATIENTS AND METHODS: In 16 patients with brain cystic tumors, MRS was performed at TEs of 30, 135 and 270 ms for detection of Cho, NAA and Cr metabolites using a 3 tesla MRI unit. The percentage of analyzed ratios greater than a cut-off point of 1.3 for Cho/Cr and 1.6 for Cho/NAA were calculated. RESULTS: Cho/Cr and Cho/NAA ratio means at all TEs were more at the central area in comparison with the periphery, although none of the differences were statistically significant. There was no statistically significant difference among the compared TEs. The percentages of ratios above the cut-off point at all TEs were more in the rim compared to the center and in the union of both compared to the rim or center. All the patients had at least one voxel with a Cho/Cr ratio of more than 1.3 when the voxel was chosen according to the hotspots shown in the chemical shift imaging map, regardless of their location at all examined TEs. CONCLUSIONS: Selection of voxels with the guide of chemical shift imaging map yields to 100% diagnostic sensitivity. If not accessible, the use of the union of peripheral and central voxels enhances the sensitivity when compared to usage of peripheral or central voxels solely.

Cortical representation of Persian word production: an fMRI study.

BACKGROUND: Neural correlates of single word reading with the use of a functional MRI (fMRI) scan have been widely studied in different languages. These study patterns of cortical activation differ in different languages. In this report we used a similar technique to study cortical activation when reading single Persian words. METHODS: The subjects were comprised of nine healthy right-handed bilingual individuals who performed three consecutive fMRI paradigms. RESULTS: Our study showed activation of the inferior frontal gyrus (IFG) when single Persian words were read. These results revealed that the pattern of brain activation during word production in Persian has a similar topography to that of English equivalents. CONCLUSION: The paradigms selectively activate word production areas and are useful in neurological assessment of the Persian population.

Non-invasive brain mapping of motor-related areas of four limbs in patients with clinically isolated syndrome compared to healthy normal controls.

Functional MRI studies on patients with multiple sclerosis (MS) have demonstrated widespread cortical reorganization of the motor network. However, few functional studies have addressed cortical plasticity in patients with clinically isolated syndrome (CIS). The activity of the lower limb motor system, despite its highlighted involvement in patients with CIS and MS, has been little studied. Thus, brain activation was compared in CIS patients with clinically intact motor systems with that in healthy control participants while they were performing motor tasks with four limbs. A total of 26 right-handed patients with CIS with clinically intact motor systems and 28 right-handed age and sex-matched controls participated in the functional MRI (fMRI) motor task. Patients with CIS showed greater activation in the ipsilateral secondary somatosensory cortex, cingulate gyrus and precuneus cortex while performing the ankle movement task compared to healthy controls. In the finger-tapping task, patients with CIS showed greater activity in the contralateral thalamus, ipsilateral premotor and superior temporal gyrus. In addition, the left inferior frontal gyrus was activated more in patients with CIS, regardless of the hand used. Therefore, despite having clinically intact motor systems, patients with CIS had different motor networks. All novel recruited regions were adjacent to the somatotopy of the primary motor areas of the limbs. Our finding confirm that brain reorganization precedes clinical manifestation, as no patient had any clinical manifestation that suggested involvement of the motor system.

The antiepileptic activity of Vitex agnus castus extract on amygdala kindled seizures in male rats.

The antiepileptic activity of hydrophilic extract of Vitex agnus castus fruit (Vitex) was evaluated by the kindling model of epilepsy. Intact male rats (250-300 g) were stereotaxically implanted with a tripolar and two monopolar electrodes in amygdala and dura, respectively. The afterdischarge (AD) threshold was determined in each animal and stimulated daily until fully kindled. The animals were administered different doses (60, 120 or 180 mg/kg) of Vitex or 0.1 ml of hydro alcoholic solvent intra-peritoneally (i.p.) and kindling parameters including AD threshold, seizure stages (SS), afterdischarge duration (ADD), stage 4 latency (S4L) and stage 5 duration (S5D) were recorded 30 min post-injection. The obtained data showed that even low dose (60 mg/kg) of Vitex could significantly increase the AD threshold and decrease the ADD and S5D (P<0.05). These changes were more significant with higher doses (120 or 180 mg/kg) for ADD (P<0.01) and S5D (P<0.001). Vitex at the dose of 120 mg/kg, induced significant increment in S4L (P<0.05). This effect was more prominent at the dose of 180 mg/kg (P<0.001). The latter dose could significantly reduce seizure stage (P<0.01) and most of the animals did not show S5. These results indicate that Vitex can reduce or prevent epileptic activity as demonstrated by reduction of ADD and S5D (length of convulsion) in a dose dependent manner. In conclusion, Vitex at appropriate dose can probably reduce or control epileptic activities.