A rare embryological malformation of brain - Dandy-Walker syndrome - and its association with Kallmann's syndrome.

BACKGROUND & AIM: Dandy-Walker malformation, a rare congenital brain malformation, is described as a triad of cystic dilatation of the fourth ventricle, complete or partial agenesis of the cerebellar vermis, and an enlarged posterior fossa with elevated tentorium. We aimed to report an association of Kallmann's syndrome and Dandy-Walker malformation. CASE: A fifteen years old boy was referred to endocrinology department due to delayed puberty. Stages of male genital development according to Marshall and Tanner, was stage G1 and P1 respectively. In the LHRH test, peak LH level was 40th min.:15.3 IU/ml. Peak growth hormone with insulin tolerance test was 14.5 microg/L. Olfactory test revealed light anosmia. With these findings the patient was accepted as isolated gonadotropin deficiency (Kalmann's syndrome). In computed tomography of the brain, cerebellar vermis was found to be hypoplastic and 4th ventricle was large and in posterior fossa broad hypodens area with cerebrospinal fluid density were seen (Dandy-Walker malformation). CONCLUSION: We reported an association of Kallmann's syndrome and Dandy-Walker malformation. This is second reported case probably.

An experimental Staphylococcus aureus meningitis model for investigating induced leptomeningeal and subpial inflammation in rats: a transmission electron microscopy study.

OBJECTIVE: To evaluate leptomeningeal and subpial inflammatory responses of experimental Staphylococcus aureus bacteriemia following intraperitoneal and intravenous applications and to compare the inflammatory reactions in different regions of central nervous system. MATERIAL AND METHODS: Forty anesthetized rats were divided into four groups equal in number. The rats in group-I were given 1 ml suspension of Staphylococcus aureus intraperitoneally. Group-II was the control group of group I; it was administrated 1 ml 0.9% NaCl in water intraperitoneally. The rats in group-III were given the same amount of bacteria intravenously. Group IV was the control group of the group-III; it was administrated 1 ml 0.9% NaCl solution intravenously. The rats were sacrificed on the 21st day. Inflammatory changes of different regions of the central nervous system were examined under transmission electron microscopy. Statistical analysis was done by using variance analysis, Bonferroni, Tamhane post hoc, Student's t and univariate tests. RESULTS: Thoracic and occipital regions were the most vulnerable zones. Increasing of collagen tissue was the most detected inflammatory change. CONCLUSION: This experimental model can be used for inducing subpial and leptomeningeal inflammations and it may be developed for investigations of pathogenesis of leptomeningitis during systemic infections.

Association of Polymorphisms within the Serotonin Receptor Genes 5-HTR1A, 5-HTR1B, 5-HTR2A and 5-HTR2C and Migraine Susceptibility in a Turkish Population.

OBJECTIVE: Migraine, a highly prevelant headache disorder, is regarded as a polygenic multifactorial disease. Serotonin (5-HT) and their respective receptors have been implicated in the patogenesis. METHODS: We investigated the 5-HT1A, 5-HT1B, 5-HT2A, and 5-HT2C receptor gene polymorphisms and their association with migraine in Turkish patients. The rs6295, rs1300060, rs1228814, rs6311, rs6313, rs6314, rs6318, rs3813929 (-759C/T) and rs518147 polymorphisms were analyzed in 135 patients with migraine and 139 healthy subjects, using a BioMark 96.96 dynamic array system. RESULTS: We found no difference in the frequency of the analyzed eight out of nine polymorpisms between migraine and control groups. However, a significant association was found between the rs3813929 polymorphism in the promoter region of 5-HTR2C gene and migraine. Also, the allele of rs3813929 was more common in the migraine group. CONCLUSION: This result suggests that the 5-HTR2C rs3813929 polymorphism can be a genetic risk factor for migraine in a Turkish population.

Facial diplegia: etiology, clinical manifestations, and diagnostic evaluation.

OBJECTIVE: Facial diplegia (FD) is a rare neurological manifestation with diverse causes. This article aims to systematically evaluate the etiology, diagnostic evaluation and treatment of FD. METHOD: The study was performed retrospectively and included 17 patients with a diagnosis of FD. RESULTS: Patients were diagnosed with Guillain-Barré syndrome (GBS) (11), Bickerstaff's brainstem encephalitis (1), neurosarcoidosis (1), non-Hodgkin's Lymphoma (1), tuberculous meningitis (1) herpes simplex reactivation (1) and idiopathic (1). In addition, two patients had developed FD during pregnancy. CONCLUSION: Facial diplegia is an ominous symptom with widely varying causes that requires careful investigation.

CT and MR imaging of chronic subdural hematomas: a comparative study.

UNLABELLED: QUESATIONS UNDER STUDY / PRINCIPLES: This study was designed to compare CT and MR appearances of chronic subdural haematomas as well as CT- and MR-guided measurements of haematoma thicknesses. METHODS: CT and MR images of 48 chronic subdural haematomas of 34 patients were reviewed retrospectively. The thickness measurements and imaging characteristics of haematomas were compared. RESULTS: Levelling was observed in 25% of haematomas, and most of them (60%) had intrahaematomal membranes. All membranes could be delineated by MR imaging, whereas only 27% were defined by CT. Mixed density (52%) and T1 hyperintensity (59%) were commonly observed in membraned haematomas, but the difference was not statistically significant. Haematomas were measured significantly thicker on MR images. All patients had been treated with burr-hole cranio-tomy and irrigation. CONCLUSIONS: MR imaging is more sensitive than CT in determining the size and internal structures of chronic subdural haematomas.

Facial diplegia: etiology, clinical manifestations, and diagnostic evaluation / Diplegia facial: etiologia, manifestações clínicas, e avaliação diagnóstica

ABSTRACT Objective Facial diplegia (FD) is a rare neurological manifestation with diverse causes. This article aims to systematically evaluate the etiology, diagnostic evaluation and treatment of FD. Method The study was performed retrospectively and included 17 patients with a diagnosis of FD. Results Patients were diagnosed with Guillain-Barré syndrome (GBS) (11), Bickerstaff’s brainstem encephalitis (1), neurosarcoidosis (1), non-Hodgkin’s Lymphoma (1), tuberculous meningitis (1) herpes simplex reactivation (1) and idiopathic (1). In addition, two patients had developed FD during pregnancy. Conclusion Facial diplegia is an ominous symptom with widely varying causes that requires careful investigation.

RESUMO Objetivo Diplegia facial (DF) é uma manifestação neurológica rara proveniente de diferentes causas. Este artigo visa avaliar sistematicamente a etiologia, avaliação diagnóstica e tratamento de DF. Método O estudo foi retrospectivo e incluiu 17 pacientes com diagnóstico de FD. Resultados Os pacientes foram diagnosticados como casos de síndrome de Guillain-Barré (SGB) (11), encefalite de tronco de Bickerstaff (1), neurosarcoidose (1), linfoma não-Hodgkin’s (1), meningite tuberculosa (1) reativação de herpes simplex (1) e causa idiopática (1). Além disto, duas pacientes haviam desenvolvido DF durante a gestação. Conclusão Diplegia facial é uma manifestação com diversas causas que requer investigação cuidadosa.