MRI findings of hypoxic cortical laminar necrosis in a child with hemolytic anemia crisis

We present magnetic resonance imaging findings of a 5-year-old girl who had a rapidly installing hemolytic anemia crisis induced by trimethoprim-sulfomethoxazole, resulting in cerebral anoxia leading to permanent damage. Magnetic Resonance imaging revealed cortical laminar necrosis in arterial border zones in both cerebral hemispheres, ischemic changes in subcortical white matter of left cerebral hemisphere, and in the left putamen. Although cortical laminar necrosis is a classic entity in adulthood related to conditions of energy depletions, there are few reports available in children. A wide review of the literature is also presented.     

A case of severe Ebstein's anomaly with incompetent pulmonary valve

A case of Ebstein's anomaly with functional pulmonary atresia diagnosed in utero is presented. The diagnosis was confirmed by postnatal echocardiographic, angiographic, and postmortem pathologic findings. On echocardiography the septal leaflet of the tricuspid valve was displaced towards the right ventricular apex. The tricuspid valve was moderately regurgitant and the arterial duct was patent. Continuous wave or color Doppler revealed serious reduction in forward flow from right ventricle through the pulmonary arteries; however, massive pulmonary regurgitation was observed. Pulmonary circulation was dependent on the ductal flow due to functional pulmonary atresia. Angiography revealed the massively enlarged right atrium, the absence of forward flow through the tricuspid valve, transfer of contrast material through the atrial septal defect to the left atrium, and the retrograde inflow of the pulmonary arteries from the aorta via the patent arterial duct. Ebstein's anomaly accompanied by functional pulmonary atresia is very rare. The fetal and neonatal presentation of this anomaly is associated with poor outcome.     

Rupture of rudimentary horn pregnancy at the 15th week of gestation: a case report

A patient with acute abdomen at 15th week of gestation underwent an emergency laparatomy. A ruptured rudimentary horn pregnancy was diagnosed, and the rudimentary part including the fetus and the accessories was resected. The mother survived without any complications.     

Evaluation of patients with globus pharyngeus with barium swallow pharyngoesophagography

OBJECTIVE: The objective of this study was to determine the value of barium swallow pharyngoesophagography in the investigation of patients with globus pharyngeus who had no known disease, no operation history in the head and neck region, and no thyroid pathology. We compare the clinical presentation with the radiologic findings to assess the diagnostic accuracy of barium swallow in such patients. METHODS: This was a retrospective study of the clinical records and radiologic reports of 194 patients with globus pharyngeus, between October 1998 and October 2004, that were reviewed and analyzed for correlations between clinical signs and symptoms and barium swallow findings using the chi2 test. All the patients had normal ear, nose, and throat examination, including indirect laryngoscopy and/or nasoendoscopy. Patients with known pharyngeal and esophageal disease, neurologic disease known to cause pharyngeal or esophageal dysfunction, evidence of autoimmune disease or diabetes mellitus, with a thyroid operation in the past and thyroid nodules detected with radiologic investigation were excluded. RESULTS: There were 116 (59.8%) female and 78 (40.2%) male patients. In 114 patients (59%), globus was the only symptom. Sore throat was the most common associated complaint. Benign barium swallow findings were detected in 63.9% of the patients. Cervical osteophyte indentation was the most common finding. In 36.1% of the patients, the radiologic finding was normal. The statistical analysis using the chi2 test showed a significant relationship (P < .001) between the symptoms of globus pharyngeus and the barium swallow results. CONCLUSION: Barium swallow pharyngoesophagography is useful to detect underlying benign characteristic pathologies in patients with globus who have no known head and neck illness.     

Surgical Outcome of Epilepsy Patients Evaluated with a Noninvasive Protocol

Summary: Surgery is now an accepted treatment for some medically intractable epilepsies. Presurgical evaluation is particularly important for the localization of the epileptogenic zone, which may necessitate sophisticated imaging techniques and intracranial electroencephalogram (EEG) recordings. If patients are carefully selected, however, successful results can be achieved with noninvasive evaluation methods. Seventy-seven patients were operated on for intractable seizures. All patients underwent EEG, neuropsychological, psychiatric, and magnetic resonance imaging investigations. Ictal EEG-video recording was performed in all nonlesional and in some lesional cases that had discordant data. Selective amygdalo-hippocampectomy was performed on patients with mesial temporal lobe epilepsy (MTLE), an extended or a limited lesionectomy was performed on patients with structural lesions, and a lesionectomy with deafferentation was performed on two patients with West syndrome. Electrocorticography was not used. Temporal lobe directed surgery was performed in 63·6% of the cases. The pathological examinations of all cases showed hippocampal sclerosis (HS) in 43%, tumor or tumor-like lesions in 36%, and cortical dysplasia in 5% of patients. After a mean follow-up of 17 months (range, 2–53), 75% of the patients were seizure-free with or without aura and 15% had a marked improvement, whereas 10% did not benefit from surgery. Neuropsychological outcome of patients with MTLE and HS also showed worthwhile results. Our patients, who were evaluated without pre- and perioperative intracranial recordings and other sophisticated techniques, had an outcome comparable to those in other series from more experienced centers. Our experience indicates that successful results, especially for patients with MTLE-HS and lesion-related epilepsies, can be obtained at centers with limited resources if the diagnoses and evaluation procedures are performed carefully.     

Exenatide upregulates gene expression of glucagon‐like peptide‐1 receptor and nerve growth factor in streptozotocin/nicotinamide‐induced diabetic mice

Glucagon‐like peptide‐1 (GLP‐1) is an incretin hormone that has modulating effects on insulin release. GLP‐1 and receptors for GLP‐1 are widely expressed throughout the body including the brain. The expression of GLP‐1 receptors is very specific to large neurons in hippocampus, neocortex, and cerebellum. GLP‐1 receptor stimulation enhances glucose‐dependent insulin secretion and lowers blood glucose in type 2 diabetes mellitus. Studies on adipobiology of neurotrophins have focused on nerve growth factor (NGF) as an example of adipose‐derived neurotrophins. Compromised trophic factor signaling may underlie neurodegenerative diseases ranging from Alzheimer's disease to diabetic neuropathies. Exenatide, a potent and selective agonist for the GLP‐1 receptor, is currently approved for the treatment of type 2 diabetes mellitus. The aim of this study was to assess the effect of chronic exenatide treatment on the hippocampal gene expression levels of GLP‐1 receptor and NGF in diabetic mice. The effects of chronic exenatide treatment (0.1 μg/kg, s.c., twice daily for 2 weeks) on GLP‐1 receptor and NGF gene expression levels in the hippocampus of streptozotocin/nicotinamide (STZ–NA)‐induced diabetic mice were assessed by quantitative real‐time polymerase chain reaction (RT‐PCR). The results of this study revealed that hippocampal gene expression of GLP‐1 receptor and NGF were downregulated in diabetic mice. Importantly, a significant increase in the gene expression level of GLP‐1 receptor and NGF was determined after 2 weeks of exenatide administration. Increased gene expression level of GLP‐1 receptor and NGF may underlie the beneficial action of exenatide in STZ/NA‐induced diabetes.     

Rare cause of absence of femoral arterial pulse: Bilateral common iliac artery hypoplasia

Congenital anomaly of the iliac arteries is rare, and, given that patients are generally asymptomatic, diagnosis in the newborn period is difficult. Herein is presented the case of a newborn with bilateral hypoplasia of the common iliac arteries, seen on multi-slice computed tomography while investigating the absence of femoral pulse. This anomaly is a rare cause of absence of femoral arterial pulse.