Cerebellar gangliocytoma presenting with hemifacial spasms: clinical report,literature review and possible mechanisms

Summary  Cerebellar lesions have classically been considered not to cause epilepsy. However, previous reports have attributed seizures, beginning as hemifacial spasms to lesions of the cerebellar peduncles. We report an example of paroxysmal facial contractions associated with a cerebellar gangliocytoma. The seizures began on the first day of life and consisted of paroxysmal contractions involving the left orbicularis oculi, often the left forehead and lower facial muscles, sometimes accompanied by nystagmoid eye movements to the right and by head deviation to the left. Video-EEG monitoring showed only artifacts from muscle contractions. Magnetic resonance imaging showed a mass arising from the left superior cerebellar peduncle and partially occupying the fourth ventricle. The lesion was removed subtotally and partial seizure control was achieved. The neuropathological findings were consistent with a gangliocytoma. The literature in the association of cerebellar lesions with hemifacial spasms is reviewed and its possible mechanisms discussed. Correspondence: Krasimir Minkin, M.D., Department of Neurosurgery, Medical University of Sofia, University Hospital Saint Ivan Rilski, 15 Boulevard Akademik Ivan Geshov, 1431 Sofia, Bulgaria.     

The association of pet keeping at home with symptoms in airways,nose and skin among Bulgarian children

The role of pet exposure in early childhood for allergy/ asthma later in life is still controversial. Recently it was shown that ‘avoidance behaviour’ is an important factor for the pet distribution in the population. The aim of the present work is to study the association between self-reported pet keeping at home and symptoms in airways, nose and skin among children 2–7 years of age, in a country where primary prevention strategies regarding allergies are not common. A cross-sectional survey on the association between allergy and asthma symptoms and home enviromental factors was conducted in two towns in Bulgaria in spring 2004 (the ALLHOME-1 study). Data for 4479 out of 12982 children was obtained. 21.3% of the parents reported having pets at the time of the questionnaire, and 23.3% for pet keeping at index child's birth. Parents of 3.3% of the children got rid of some of the pets and 10.6% refrained from having pets, due in both cases to allergic illness in the family. Keeping dogs or cats at the time of the survey or during the child's first years was associated with most of the symptoms (aOR 1.1–2.2).     

Ependymoma of the cauda equina starting with communicating hydrocephalus: a case report

The presence of concomitant hydrocephalus in cases with spinal cord tumors is relatively rare. Here, we describe a case of myxopapillary ependymoma of the cauda equina starting with communicating hydrocephalus in a 14-year-old boy. The patient presented to the clinic without underlying causes of hydrocephalus on the neuroimaging data. After ventriculoperitoneal shunt placement, despite the numerous malfunction incidents, the patient was doing well. Eight years later, he developed progressive weakness and coldness of the lower limbs. Multiple cystic lesions in the cervicothoracic area were found on magnetic resonance imaging. Decompressive hemilaminectomy of the cervicothoracic region was performed with temporary improvement of the patient's condition. Because of persisting complaints, the sacral area of the spine was also observed and neuroimaging data for a tumoral lesion in the cauda equina region were found. The lesion was surgically removed and the histological result was myxopapillary ependymoma. Therefore, cases presenting with internal hydrocephalus without clear-cut intracranial etiology should have detailed neuroimaging of the whole central nervous system.     

Vascular endothelial growth factor (VEGF) affects processing of amyloid precursor protein and β-amyloidogenesis in brain slice cultures derived from transgenic Tg2576 mouse brain

The up-regulation of the angiogenic vascular endothelial growth factor (VEGF) in brains of Alzheimer patients in close relationship to β-amyloid (Aβ) plaques, suggests a link of VEGF action and processing of the amyloid precursor protein (APP). To reveal whether VEGF may affect APP processing, brain slices derived from 17-month-old transgenic Tg2576 mice were exposed with 1 ng/ml VEGF for 6, 24, and 72 h, followed by assessing cytosolic and membrane-bound APP expression, level of both soluble and fibrillar Aβ-peptides, as well as activities of α- and β-secretases in brain slice tissue preparations.Treatment of brain slices with VEGF did not significantly affect the expression level of APP, regardless of the exposure time studied. In contrast, VEGF exposure of brain slices for 6 h reduced the formation of soluble, SDS extractable Aβ(1–40) and Aβ(1–42) as compared to brain slice cultures incubated in the absence of any drug, while the fibrillar Aβ peptides did not change significantly. This effect was less pronounced 24 h after VEGF exposure, but was no longer detectable when brain slices were exposed by VEGF for 72 h, which indicates an adaptive response to chronic VEGF exposure. The VEGF-mediated reduction in Aβ formation was accompanied by a transient decrease in β-secretase activity peaking 6 h after VEGF exposure. To reveal whether the VEGF-induced changes in soluble Aβ-level may be due to actions of VEGF on Aβ fibrillogenesis, the fibrillar status of Aβ was examined using the thioflavin-T binding assay. Incubation of Aβ preparations obtained from Tg2576 mouse brain cortex, in the presence of VEGF slightly decreased the fibrillar content with increasing incubation time up to 72 h. The data demonstrate that VEGF may affect APP processing, at least in vitro, suggesting a role of VEGF in the pathogenesis of Alzheimer's disease.     

Does Aliskiren Influence the Changes of Global Longitudinal Strain in Patients with Diastolic Dysfunction？

The aim of our study was to investigate the effects of Aliskiren on the blood pressure values as well as on the myocardial function, assessed by GLS （global longitudinal strain） in patients with uncontrolled AH （arterial hypertension） and diastolic dysfunction. Forty five nondiabetic patients （29 men and 16 women, mean age 58.7 ± 12.4 years） with BP （blood pressure） 〉 140/90 mm Hg despite of combined antihypertensive therapy （diuretic, calcium channel blockers, beta-blockers） were evaluated. All of them had echocardiographic data for diastolic dysfunction： E/E＇ ratio _〈 8, E/A ratio 〈 0.8, DT （deceleration time） 〉 200 ms. Aliskiren 2 x 150 mg per day was added to the previous therapy. The follow-up period was 1 year, including monthly clinical visits. Echocardiographic assessment of the left ventricular function by longitudinal strain and Doppler analysis of the transmitral blood flow was performed at months 1, 6 and 12. The baseline mean measurements of systolic and diastolic BP values showed （153.4 ± 14.4）/（99.2 ±6.7） mmHg for males and （157.6 ±12.5）/（97.3±8.2） mmHg for females. The systolic and diastolic BP values at the end of the 1st month were （131.7±7.4）/（83.6± 5.2） mmHg in men and （132.4± 5.3）/（81.8± 6.9） mmHg in women （P 〈 0.05, compared to the initial BP values）. The baseline ratio of E/E＇ was found to be 6.5 ± 0.9, E/A-0.6±0.01 and DT--258 ± 32.7 ms. The values of these parameters at month 12 were as follows： E/E＇--7.0± 0.64, E/A--0.7± 0.05, DT--239 ± 16.5 ms （P = NS）. Baseline global longitudinal strain in men was -10.4 ±0.7% and -11.0 ± 0.9% in women, whereas GLS at month 12 showed values of： （-16.3± 0.9%） and （-17.5 ± 0.7%） for males and females respectively （P 〈 0.05）. During the whole period of treatment with Aliskiren and follow-up no adverse effects were registered. Aliskiren, added to a combined antihypertensive therapy （without RAAS--renin-angiotensin-aldosterone system blocker） in patients with uncontrolled AH and diastolic dysfunction provides not only a better BP control, but also a significant improvement of the myocardial function, assessed by global longitudinal strain. The 12 months treatment with Aliskiren was safe and well tolerated.