Early- and late-state subacute sclerosing panencephalitis: chemical shift imaging and single-voxel MR spectroscopy

BACKGROUND AND PURPOSE: Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, inflammatory neurodegenerative disease. Our aim was to determine the metabolic abnormalities of brain in early- and late-stage SSPE by using MR spectroscopy and to assess areas of involvement in the early stages when MR imaging findings were normal. METHODS: Children with stage II (n = 3) or III (n = 3) SSPE and 10 healthy, age-matched children underwent MR imaging, multivoxel MR spectroscopy, and short-echo single-voxel MR spectroscopy (SVS). Areas of involvement in the brain were determined with chemical shift imaging. For SVS, 2 x 2 x 2-cm voxels were placed in the frontal subcortical white matter (FSWM) and parieto-occipital white matter (POWM). N-acetylaspartate (NAA)/creatine (Cr), choline (Cho)/Cr, myo-inositol (Ins)/Cr, and NAA/Cho ratios were calculated. RESULTS: Comparisons of NAA/Cr, Cho/Cr, Ins/Cr and NAA/Cho ratios between patients and control subjects showed significant differences in FSWM and POWM (P <.01). In patients with SSPE, NAA/Cr ratios in POWM were significantly less than those in FSWM (P <.01). NAA/Cr ratios in patients with stage II SSPE and those in the control group were not significantly different; this may reflect the absence of neuronal loss. Decreased NAA/Cr, increased Cho/Cr and Ins/Cr ratios, and increased lactate and lipid peaks were found in patients with stage III SSPE. CONCLUSION: MR spectroscopy showed findings suggestive of inflammation in stage II and findings of demyelination, gliosis, cellular necrosis, and anaerobic metabolism in stage III. MR spectroscopy could be a promising technique for early diagnosis and treatment planning in cases of SSPE.     

Temporary and Permanent Inferior Vena Cava Filter Combination in a Young Patient: To Implant or Not to Implant?

The decision to implant vena cava filters, either temporary or permanent, is difficult in young patients. We present the case of a young man with pulmonary embolism in whom temporary and permanent inferior vena cava filters were implanted. The decision process is discussed in relation to the current literature.     

Two patients with Kabuki syndrome presenting with endocrine problems

A 4 year-old boy with mental retardation and seizures presented to the pediatric endocrinology clinic because of a history of hypoglycemia; a 16 month-old girl with developmental delay presented with bilateral breast tissue enlargement; in both, a diagnosis of Kabuki syndrome was made because of typical facial features, neurodevelopmental delay and other stigmata consistent with Kabuki syndrome. Kabuki syndrome is a mental retardation-malformation syndrome affecting multiple organ systems with a broad spectrum of abnormalities. The facial features of the syndrome are specific and independent of ethnic origin. In addition to presenting with endocrine problems, the patients reported here exhibit some novel findings such as congenital alopecia areata and hyperpigmented skin lesion. The diagnosis of Kabuki syndrome should be considered in patients with hypoglycemia or premature thelarche when associated with developmental delay and a peculiar facies.     

Intermittent versus continuous sertraline therapy in the treatment of premenstrual dysphoric disorders

OBJECTIVE: Premenstrual dysphoric disorder (PMDD) is a combination of mood disturbances and physical symptoms that reduce the quality of individual life and the functionality of the individual. Many women do not consider the complaints arising in the luteal phase of the menstrual cycle as a psychiatric disorder and, thus, do not seek treatment. Those who take their complaints to doctors usually apply to gynecology clinics. The aim of the present study is to analyze the psychiatric disorders observed in patients with PMDD and to compare the continuous and intermittent administration of sertraline. MATERIALS AND METHODS: PMDD was investigated in the patients admitted to the Gynecology Clinic of the Medical School of Fatih University. The patients were asked to fill out forms designed in accordance with PMDD diagnosis criteria as defined in DSM IV. RESULTS: Among the 267 patients who filled out the forms, 162 (60.7%) were PMDD positive. Of the PMDD-positive patients, 133 accepted a psychiatric interview; 36 (27%) of them had depression, obsessive-compulsive disorder, anxiety and somatoform disorders as an accompanying disorder. Out of 162 PMDD-positive patients 94 accepted medical treatment; 71 patients were given sertraline on a continuous basis, and 23 patients took sertraline intermittently in the luteal phase of the cycle. Because of side effects, 44 (62%) of the continuous therapy and 22 (96%) of the intermittent therapy group stopped medication. At the end of 6-month follow-up, continuous use of sertraline was found to be significantly more tolerated than intermittent therapy in the treatment of PMDD (chi2 = 7.88, p = 0.005). CONCLUSION: In patients with the symptoms of PMDD, psychiatric evaluation should be encouraged by gynecology clinics, and continuous administration of sertraline should be the choice because of patients' greater acceptance of the therapy.     

Thrombotic thrombocytopenic purpura associated with multiple myeloma

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic disease. Associations with collagen vascular diseases, pregnancy, some drugs, surgical intervention, and infections are documented (known). Malignancy is also one of the known factors associated with thrombotic thrombocytopenic purpura. These malignancies are usually the disseminated solid organ tumors. Hematological malignancies constitute a rare association. Here, we present a patient with thrombotic thrombocytopenic purpura associated with multiple myeloma and discuss the pathogenesis. To our knowledge, this is the first case report of thrombotic thrombocytopenic purpura associated with multiple myeloma.     

Quantitative evaluation of brain edema by radionuclide imaging technique

The objective of the present investigation was to quantify metabolic cerebral edema in dogs with impaired monoamine oxidase (MAO) function and liver disease using dynamic imaging of the brain with 99mTc-diethylenetriamine pentacetic acid (99mTc DTPA). Data acquisition was started following a rapid intracarotid injection of 99mTc DTPA (5 mCi), and sixty 0.5-s images of the left or right hemisphere were taken. First-pass time-activity curves were obtained by selecting regions of interest for the appropriate brain hemisphere. The results of this study demonstrated that in phenelzine-treated animals there was a 6 to 38% reduction in brain washout slopes of 99mTc DTPA (24.0 +/- 11.5% reduction). These washout slopes were further reduced (range 24.0-86.0; 50.6 +/- 18.5% reduction) following the oral administration of tyramine (1 mg/kg). A significant correlation was noted between changes in washout slopes and the development of coma in these animals.     

Permanent transfemoral pacemaker implantation in a child with Maroteaux Lamy syndrome

Permanent transfemoral pacing has been described as an alternative route in patients in whom the superior venous approach is not feasible. This report describes the use of the femoral venous approach to insert a permanent pacemaker in a child with Maroteaux Lamy syndrome who has complete atrioventricular block and abnormal subclavian venous anatomy. Transfemoral pacing may be a suitable alternative in children with short stature.     

Quantitative Doppler evaluation of the splenoportal venous system in various stages of cirrhosis: differences between right and left portal veins

PURPOSE: The purpose of our study was to evaluate the relationship between the splenoportal hemodynamics in patients with cirrhosis and the stage of the disease. METHODS: Patients with cirrhosis were grouped according to modified Child-Pugh scoring into stages A, B, and C of cirrhosis. A control group of healthy volunteers was included. After gastroenterologic clinical and laboratory examinations, all participants underwent a splenoportal Doppler sonographic evaluation in which the vessels' diameter, area, and blood flow velocity were measured and blood flow rate and the congestion index in the splenoportal venous system were calculated. RESULTS: Seventy-five patients with cirrhosis (25 women and 50 men) were enrolled; the control group consisted of 30 healthy volunteers (15 women and 15 men) with no liver disease. The mean age (+/- standard deviation) of the patients was 54.4 +/- 14.8 years (range, 13-80 years) and of the control subjects was 47.3 +/- 14.5 years (range, 18-72 years). No significant differences in vessel diameter, blood flow velocity, and blood flow rate were found in the main and left portal veins between the study group and the control group. In the right portal vein, we found decreases in the vessel diameter, blood flow velocity, and blood flow rate, and in the splenic vein, we found increases in vessel diameter and blood flow rate. The congestion index was increased in the main portal and splenic veins but was unchanged in the left portal vein. CONCLUSIONS: Although our data indicate that there is no difference in Doppler sonographic parameters of the main portal vein according to Child-Pugh scores, the hemodynamic differences between the left and right branches of the portal vein may be clinically useful in patients with cirrhosis.     

The Effect of Pulmonary Hypertension on Left Ventricular Diastolic Function in Chronic Obstructive Lung Disease: A Tissue Doppler Imaging and Right Cardiac Catheterization Study

Background

Pulmonary hypertension (PH) caused by chronic obstructive lung disease (COLD) essentially involves the right heart. Also left ventricular (LV) systolic and diastolic functions may be affected.

Objectives

The aim of this study was to investigate the effect of on LV diastolic function in patients with COLD.

Methods

A total of 47 patients with COLD and 20 controls were included in this study. All patients underwent Doppler echocardiography, tissue Doppler imaging examinations and right cardiac catheterization. The patients were divided into 2 subgroups according to mean pulmonary arterial pressure (mPAP): patients without PH (group1, n = 25) and with PH (group 2, n = 22). The following measurements were taken: peak velocity of early diastolic filling (E), peak late filling with atrial contraction (A), E/A ratio, deceleration time (DT) of E, isovolumic relaxation time (IVRT), early (Em) and late (Am) diastolic mitral lateral annulus velocity.

Results

Mitral E/A < 1 and Em < 8 cm/sec were higher in group 2 than in group 1 and the control group. There were significant correlations between mPAP and both mitral E/A (r:? 0.60) and Em (r:? 0.45). In multivariate model, mPAP was not found to be significant on mitral E/A ratio < 1, but there was a significant effect on mitral Em < 8 cm/sec (odds ratio [OR]:1.14, P < 0.05).

Conclusion

This study shows that LV diastolic dysfunction in COLD is closely correlated to PH levels. Although increased mPAP may affect the mitral E/A ratio, it seems to have no effect on mitral E/A < 1, whereas it has an independent effect on Em < 8 cm/sec. Copyright © 2010 Wiley Periodicals, Inc.