Since the earliest attempt by Klinger in 1931 to describe the systemic vasculitis subsequently characterized in 1936 by Wegener as Wegener's granulomatosis, this disorder has been reported to present in a number of differing fashions. No previous description relates Wegener's presenting as a seizure disorder, and no reports of MRI of the brain in such patients exists. We relate such a case, alo...

Case report. A 14-year-old Caucasian female presented with a 3-month history of headaches, progressive rightsided convergent squint, dysarthria, gait abnormality with progressive difficulty in climbing up the stairs and being able to stand, and hyperesthesia involving her left arm and leg. She had a longstanding history of pervasive refusal disorder for which she was receiving psychiatric suppo...

In the last decade, multiple investigator groups have identified structural changes of various neuroanatomic structures in patients with idiopathic major depression and bipolar disorders. Using high-resolution MRI of the brain and functional neuroimaging studies (i.e., PET, SPECT), researchers have described decreases in the volume of hippocampal formation, amygdala, entorhinal cortex, various ...

BACKGROUND Antecedents to human immunodeficiency virus-dementia (HIV-D) are poorly understood. OBJECTIVE To identify risk factors for HIV-D. METHODS Subjects who are positive for HIV who have CD4+ counts either below 200/microL or below 300/microL with evidence of cognitive impairment were enrolled in this study. Neurologic, cognitive, functional, and laboratory assessments were done semian...

There is a complex relationship between seizures and encephalopathy. Seizures alone without any underlying neurologic or medical illness can be the sole cause of encephalopathy. Often these patients have a history of epilepsy, in which case accurate diagnosis is straightforward. Acute neurologic conditions often contribute to encephalopathy, but also increase the risk of seizures--many of which...

OBJECTIVE Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a newly recognized antineuronal antibody-mediated inflammatory brain disease that causes severe psychiatric and neurologic deficits in previously healthy children. The present study was undertaken to describe characteristic clinical features and outcomes in children diagnosed as having anti-NMDAR encephalitis. METHODS C...

OBJECTIVE The goal of this study was to describe reported influenza A (H1N1)pdm09 virus (pH1N1)-associated deaths in children with underlying neurologic disorders. METHODS The study compared demographic characteristics, clinical course, and location of death of pH1N1-associated deaths among children with and without underlying neurologic disorders reported to the Centers for Disease Control a...

BACKGROUND Inherited deficiency of phosophomannomutase (PMM2) causes a human glycosylation disorder known as Congenital Disorder of Glycosylation Ia. CASE PRESENTATION Herein, we describe a case of congenital disorder of glycosylation Ia, presented with recurrent pericardial effusion and unusual findings of inverted nipples, fat pads, reduced deep-tendon reflexes and multisystem involvement. ...

idiopathic hypereosinophilic syndrome represents a heterogeneous group of leukoprolifrative disorders associated with prolonged eosinophilia of an undetectable cause with multi organ system dysfunction. it is a rare group disorder in children, most cases are reported in adult age group. we report a child with this syndrome who along with the usual features of the syndrome also had the presentat...