Pathological laughter and crying: A case series and proposal for a new classification.

Background: Disorders of laughter and crying (DLC) are seen in several neuropsychiatric conditions. Their nomenclature remains under debate.

Methods: We present the clinical and imaging findings of 17 patients with DLC and introduce a new classification based on phenomenology and pathogenesis.

Vagal stimulation in brain dead donor rats decreases chronic allograft nephropathy in recipients.

Background: Although it has been shown that a vagus nerve stimulation of brain dead (BD) donors leads to an improvement of renal function in recipients in an acute allograft rejection model, its influence on chronic allograft nephropathy is still unknown. In the present study, we assessed the influence of donor vagus nerve stimulation on survival, renal function and histology in a chronic allograft model.

Methods: Brain death was induced in Fisher rats, and electro-stimulation of the vagus nerve was applied in one group (BD + vagus) during the whole course of BD (6 h).

Modulation of brain dead induced inflammation by vagus nerve stimulation.

Because the vagus nerve is implicated in control of inflammation, we investigated if brain death (BD) causes impairment of the parasympathetic nervous system, thereby contributing to inflammation. BD was induced in rats. Anaesthetised ventilated rats (NBD) served as control.

Neurologic presentations of fungal infections.

Fungal infections of the central nervous system (CNS) have a high rate of morbidity and mortality caused by several factors. Most importantly, the last three decades have witnessed a rising prevalence of susceptible hosts from the growing numbers of organ transplants, chemotherapy patients, and intensive care unit hospitalizations. Knowledge of CNS fungal infections including their symptoms and signs, required diagnostic studies, and treatment methods is imperative for all neurologists.

Ondansetron and seizures.

Experimental studies suggest that 5-hydroxytryptamine (5-HT) receptors play a role in epileptogenesis and seizure propagation. Ondansetron, a 5-HT(3) receptor antagonist, has been reported to have proconvulsant and anticonvulsant effects in animals. We describe three patients who developed seizures after receiving ondansetron.

The optic nerve.

The optic nerve is our most important cranial nerve. As it courses from the eyeball to the brain, it is divided into four segments: intraocular, intraorbital, intracanalicular, and intracranial. Four tests are primarily used to assess its functional integrity and detect optic nerve disorders.

The additional detrimental effects of cold preservation on transplantation-associated injury in kidneys from living and brain-dead donor rats.

Background: Brain death and cold preservation are major alloantigen-independent risk factors for transplantation outcome. The present study was conducted to assess the influence of these factors on transplantation-associated injury independently or in combination.

Methods: Brain death was induced in F344 rats.

Clinical benefit of a short term dietary oatmeal intervention in patients with type 2 diabetes and severe insulin resistance: a pilot study.

Aims/hypothesis: To evaluate the potential effectiveness of 'carbohydrate days' as a dietary intervention to overcome insulin resistance in type 2 diabetes.

Materials And Methods: Patients (n=14) with uncontrolled type 2 diabetes and insulin resistance as defined by a dosage of more than 1 IU/day (*)kg BW were consecutively enrolled in this prospective study. Primary outcomes were daily insulin requirement and mean blood glucose levels which were evaluated before, after, and 4 weeks after the intervention.

IM interferon beta-1a delays definite multiple sclerosis 5 years after a first demyelinating event.

Background: The Controlled High Risk Subjects Avonex Multiple Sclerosis Prevention Study (CHAMPS) showed that IM interferon beta-1a (IFNbeta-1a) significantly slows the rate of development of clinically definite multiple sclerosis (CDMS) over 2 years in high-risk patients who experience a first clinical demyelinating event. This report highlights the primary results of a 5-year, open-label extension of CHAMPS (the Controlled High Risk Avonex Multiple Sclerosis Prevention Study in Ongoing Neurologic Surveillance [CHAMPIONS Study]).

Objective: To determine if the benefits of IFNbeta-1a observed in CHAMPS are sustained for up to 5 years.

Subclavian artery dissection and triple infarction of the nervous system.

Background: Subclavian artery dissection is a rare entity. It is usually associated with anomalous aortic vasculature. Only with trauma or catheterization procedures is subclavian artery dissection with normal aortic vasculature reported.

Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage.

ATP-binding cassette (ABC) transporters facilitate unidirectional translocation of chemically diverse substances, ranging from peptides to lipids, across cell or organelle membranes. In peroxisomes, a subfamily of four ABC transporters (ABCD1 to ABCD4) has been related to fatty acid transport, because patients with mutations in ABCD1 (ALD gene) suffer from X-linked adrenoleukodystrophy (X-ALD), a disease characterized by an accumulation of very-long-chain fatty acids (VLCFAs). Inactivation in the mouse of the abcd1 gene leads to a late-onset neurodegenerative condition, comparable to the late-onset form of X-ALD [Pujol, A.

Moraxella catarrhalis--infected alveolar epithelium induced monocyte recruitment and oxidative burst.

The recruitment of monocytes appears to be a crucial factor for inflammatory lung disease. Alveolar epithelial cells contribute to monocyte influx into the lung, but their impact on monocyte inflammatory capacity is not entirely clear. We thus analyzed the modulation of monocyte oxidative burst by A549 and isolated human alveolar epithelial cells.

Analysis of very long-chain fatty acids using electrospray ionization mass spectrometry.

Elevated levels of very long-chain fatty acids (VLCFA) in plasma and tissues are the biochemical hallmark for patients with X-linked adrenoleukodystrophy (X-ALD). Current methods for the determination of VLCFA levels are laborious and time-consuming. We describe a rapid and easy method using electrospray ionization mass spectrometry (ESI-MS) with deuterated internal standards.

Interferon beta-1a for early multiple sclerosis: CHAMPS trial subgroup analyses.

The objective of this work was to assess the effect of interferon beta-1a (Avonex) on the rate of development of clinically definite multiple sclerosis and brain magnetic resonance imaging changes in subgroups based on type of presenting event, baseline brain magnetic resonance imaging parameters, and demographic factors in the Controlled High-Risk Subjects Avonex Multiple Sclerosis Prevention Study (CHAMPS) trial. After the onset of a first demyelinating event, 383 patients with brain magnetic resonance imaging evidence of subclinical demyelination were treated with corticosteroids and randomly assigned to receive weekly intramuscular injections of 30 microg interferon beta-1a or placebo. The treatment effect within subgroups was assessed in proportional hazards models both for the development of clinically definite multiple sclerosis and for a combined outcome of development of clinically definite multiple sclerosis or >1 new or enlarging T2 lesions on brain magnetic resonance imaging.

Characterization of plasma acylcarnitines in patients under valproate monotherapy using ESI-MS/MS.

Objectives: The effect of administration of the antiepileptic drug valproate (VPA), on the composition of the plasma acylcarnitine profile (including free carnitine) was investigated.

Design And Methods: Plasma samples were obtained from 18 individuals (13 males:5 females; 15-65 y) on long-term treatment with VPA (resulting in plasma levels of 14.6-135.

Light-induced amaurosis fugax.

Purpose: To describe an important symptom of bilateral amaurosis precipitated by exposure to bright light.

Methods: Case report. Clinical analysis of transient, painless, asymmetrical visual loss occurring in bright light in a 54-year-old man.

Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature.

Objective: The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options.

Design: Retrospective, noncomparative case series.

Retinol-binding protein in idiopathic intracranial hypertension (IIH).

Objective: We postulated that an alteration in endogenous vitamin A (retinol) metabolism plays a causal role in the pathogenesis of idiopathic intracranial hypertension (IIH).

Materials And Methods: Serum retinol was determined by a fluorometric method from 40 control subjects and 58 patients with idiopathic intracranial hypertension (IIH). Retinol binding protein (RBP) was also assayed by quantitative radial immunodiffusion in 17 control subjects and 30 patients with IIH.

Monocyte migration through the alveolar epithelial barrier: adhesion molecule mechanisms and impact of chemokines.

Alveolar monocyte influx requires adherence and transmigration through the vascular endothelium, extracellular matrix, and alveolar epithelium. For investigating the monocyte migratory process across the epithelial barrier, we employed both the A549 cell line and isolated human alveolar epithelial cells. Under baseline conditions, spontaneous bidirectional transepithelial monocyte migration was noted, which was dose-dependently increased in the presence of the monocyte chemoattractant protein-1.

MRI in Lhermitte-Duclos disease.

Lhermitte-Duclos disease (LDD) is a disorder sometimes referred to as a dysplastic gangliocytoma of the cerebellum. This is a focally indolent growth of the cerebellar cortex in which the folia enlarge due to a profusion of dysplastic cortical neurons and a thickening of the molecular layer. Loss of Purkinje cells and thinning of medullary white matter results.

Optic neuropathy, headache, and diplopia with MRI suggestive of cerebral arteritis in relapsing polychondritis.

The pathogenesis of central nervous system disease in relapsing polychondritis (RPC) is unknown but may be related to cerebral arteritis. Previous reports have described clinical and histopathologic evidence of cerebral vasculitis in RPC; however, a neuroimaging correlate has not been reported. We present a 36-year-old man with neuro-ophthalmic features of RPC whose magnetic resonance imaging revealed multifocal gray- and white-matter high intensities.