Anesthetic management for foramen magnum decompression in a patient with Morquio syndrome: a case report

Morquio syndrome is a hereditary mucopolysaccharide disorder presenting with an abnormality of the craniocervical junction from childhood. We describe an adult patient who presented with Morquio syndrome who had subglottic narrowing of the airway, restrictive pulmonary disease, and mild mitral regurgitation and trivial aortic regurgitation. The anesthetic management of this patient for atlantoaxial stabilization is presented.     

AGI-1067, a novel antioxidant and anti-inflammatory agent,enhances insulin release and protects mouse islets

The antioxidant and anti-inflammatory compound AGI-1067 (succinobucol) has potential as an oral anti-diabetic agent. AGI-1067 reduces H_bA1c, improves fasting plasma glucose, and reduces new-onset diabetes. We investigated AGI-1067 for possible effects on mouse pancreatic islets in vitro. Pretreatment with 10 μM AGI-1067 increased glucose-stimulated insulin secretion (11 mM) without affecting secretion in basal (3 mM) glucose. AGI-1067 enhanced the intracellular calcium response to glucose stimulation in 7 mM and 11 mM glucose, but had no effect in 28 mM or basal glucose. AGI-1067-pretreated islets also showed enhanced calcium responses to methyl pyruvate and alpha-ketoisocaproate at low doses, but not high doses. The AGI-1067-mediated effects on glucose-stimulated calcium were maintained during continuous diazoxide exposure, suggesting effects on the K_ATP-channel-independent pathway. AGI-1067 also reduced cytokine-induced islet cell death and expression of iNOS, a key component in cytokine signaling. This is the first report of direct stimulatory and protective effects of a first-in-class potential anti-diabetic agent on pancreatic islets.     

Ceramide transfer protein function is essential for normal oxidative stress response and lifespan

Ceramide transfer protein (CERT) transfers ceramide from the endoplasmic reticulum to the Golgi complex, a process critical in synthesis and maintenance of normal levels of sphingolipids in mammalian cells. However, how its function is integrated into development and physiology of the animal is less clear. Here, we report the in vivo consequences of loss of functional CERT protein. We generated Drosophila melanogaster mutant flies lacking a functional CERT (Dcert) protein using chemical mutagenesis and a Western blot-based genetic screen. The mutant flies die early between days 10 and 30, whereas controls lived between 75 and 90 days. They display >70% decrease in ceramide phosphoethanolamine (the sphingomyelin analog in Drosophila) and ceramide. These changes resulted in increased plasma membrane fluidity that renders them susceptible to reactive oxygen species and results in enhanced oxidative damage to cellular proteins. Consequently, the flies showed reduced thermal tolerance that was exacerbated with aging and metabolic compromise such as decreasing ATP and increasing glucose levels, reminiscent of premature aging. Our studies demonstrate that maintenance of physiological levels of ceramide phosphoethanolamine by CERT in vivo is required to prevent oxidative damages to cellular components that are critical for viability and normal lifespan of the animal.     

Focal neuronal loss, reversible subcortical focal T2 hypointensity in seizures with a nonketotic hyperglycemic hyperosmolar state

Introduction Neuroimaging in seizures associated with nonketotic hyperglycemia (NKH) is considered normal. We report magnetic resonance imaging (MRI) abnormalities in four patients with NKH and seizures. Methods We prospectively evaluated clinical and radiological abnormalities in four patients with NKH during the period March 2004 to December 2005. Results All patients presented with seizures, either simple or complex partial seizures or epilepsia partialis continua. Two of them had transient hemianopia. MRI showed subcortical T2 hypointensity in the occipital white matter and in or around the central sulcus (two patients each), T2 hyperintensity of the overlying cortex (two patients), focal overlying cortical enhancement (three patients) and bilateral striatal hyperintensity (one patient). Diffusion-weighted imaging (DWI) performed in three patients showed restricted diffusion. The ictal semiology and electroencephalographic (EEG) findings correlated with the MRI abnormalities. On clinical recovery, the subcortical T2 hypointensity and striatal hyperintensity reversed in all patients. The initial cortical change evolved to FLAIR hyperintensity suggestive of focal cortical gliosis. The radiological differential diagnosis considered initially included encephalitis, malignancy and hemorrhagic infarct rendering a diagnostic dilemma. Conclusion We identified subcortical T2 hypointensity rather than hyperintensity as a characteristic feature of seizures associated with NKH. Only very few similar reports exist in literature. Reversible bilateral striatal T2 hyperintensity in NKH has not been reported to the best of our knowledge.     

Baclofen in the management of inhalant withdrawal: a case series

Introduction: Abuse of inhalants and solvents is a significant public health problem. There is no specific treatment for inhalant withdrawal.Objective: To study the effect of baclofen in treating craving and withdrawal symptoms in patients with inhalant dependence.Case Reports: Case studies of 3 young male patients with DSM-IV diagnoses of inhalant dependence treated in an inpatient setting with baclofen are presented. All patients had nonspecific withdrawal symptoms in the form of irritability, insomnia, and craving. Baclofen was given in doses up to 50 mg/day and was continued throughout the period of hospitalization.Discussion: All patients reported significant reduction in withdrawal symptoms within 48 hours of treatment and were free of symptoms for the duration of their hospital stay. One patient continued the medication as an outpatient and has remained abstinent to date. Baclofen was well tolerated by all patients. Our results suggest that baclofen may be an effective treatment modality in this patient population. These effects are possibly due to the agonistic action of baclofen at gamma-aminobutyric acid B receptors in the ventral tegmental area.     

Role of magnetic resonance imaging in distinguishing fungal from nonfungal multiple brain abscesses

Cladophialophora bantiana is a neurotropic dematiaceous fungus known for affecting immunocompromised and immunocompetent hosts. We report a case of 24 year old immunocompetent male presenting with headache, fever and vomiting. MRI was suggestive of multiple fungal brain abscesses. He underwent total excision of abscesses. Pus culture was suggestive of brain abscess caused by C. bantiana. We report a culture proven case of C. bantiana emphasizing on specific MRI features which are critical in differentiating fungal from nonfungal brain abscesses.     

Evaluation of preanalytical stability of the sample for complete blood count

ObjectiveTo evaluate the effect of time of incubation on complete blood count (CBC) by using HmX analyzer.MethodsA cross sectional study was conducted at Manipal Acunova Limited, Bangalore. Ten blood samples which were stored at room temperature for CBC and differential count by using HmX analyzer were analyzed within one hour of draw and on 24, 48 and 72 hours of draw.ResultsDifferential count measured in an automated instrument changed over time.ConclusionsThe finding of this study shows that some of CBC parameters can be changed with the incubation, therefore it is better to perform on a fresh sample.     

Developmental and regional changes in the neurochemical profile of the rat brain determined by in vivo 1H NMR spectroscopy.

Sixteen metabolites were quantified from 11-24 micro l volumes in three different brain regions (hippocampus, striatum, and cerebral cortex) during postnatal development. Rat pups from the same litter were repeatedly measured on postnatal days 7, 10, 14, 21, and 28 using a completely noninvasive and longitudinal study design. Metabolite quantification was based on ultra-short echo-time (1)H NMR spectroscopy at 9.4 T and LCModel processing. Most of the brain metabolites were quantified with Cramer-Rao lower bounds (CRLB) less than 20%, which corresponded to an estimated concentration error <0.2 micro mol/g. Taurine and total creatine were quantified with CRLB < or = 5% from all 114 processed spectra. The resulting high reliability and reproducibility revealed significant regional and age-related changes in metabolite concentrations. The most sensitive markers for developmental and regional variations between hippocampus, striatum, and cerebral cortex were N-acetylaspartate, myo-inositol, taurine, glutamate, and choline compounds. Absolute values of metabolite concentrations were in very good agreement with previously published in vitro results based on chromatographic measurements of brain extracts. The current data may serve as a reference for studies focused on developmental defects and pathologies using neonatal rat models.     

Strong larvicidal activity of three species of Spilanthes (Akarkara) against malaria (Anopheles stephensi Liston, Anopheles culicifacies, species C) and filaria vector (Culex quinquefasciatus Say)

A system for biocontrol of malaria and filarial mosquito vectors has been developed using herbal extracts of three Spilanthes species, S. acmella L.var oleraceae Clarke, S. calva L. and S. paniculata Wall ex DC. Cent percent mortalities was achieved against the late third/early fourth instar larvae of A. stephensi Liston, A. culicifacies species C and C. quinquefasciatus Say using crude hexane extract obtained from flower heads of Spilanthes spp. Of the three plant species, S. acmella extract proved to be the most effective in inducing complete lethality at minimum doses, the respective LC50 and LC90 values being 4.57 and 7.83 (A. stephensi), 0.87 and 1.92 (A. culicifacies) and 3.11and 8.89 ppm (C. quinquefasciatus). This was followed by S. calva and S. paniculata extracts, respectively. This is the first report of achieving cent percent lethality against these mosquito larvae using minimal doses of plant extracts from this or any other plant species.