Differences between acute-onset chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyneuropathy in adult patients.

Acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A-CIDP diagnosed at our institution between January 2006 and July 2017 were retrospectively reviewed.

Door-to-Needle Time in Acute Stroke Treatment and the "July Effect".

Intravenous thrombolysis improves outcomes in acute ischemic stroke in a time-dependent fashion. As in teaching hospitals, clinical outcomes may worsen due to the arrival of new inexperienced house staff early in the academic year (July effect, JE), we evaluated the impact of the "JE" on the door-to-needle time for intravenous thrombolysis and other stroke outcomes. In this retrospective cohort study, we assessed all acute ischemic strokes treated with intravenous thrombolysis between July 2003 and June 2016.

Spontaneous Cystic Arteriovenous Malformation-A Novel Treatment Alternative.

Background: Brain arteriovenous malformation (BAVM) is a life-threatening vascular congenital malformation due to the risk of intracerebral hemorrhage. The formation of a spontaneous cyst within a BAVM, without history of hemorrhage or gamma knife surgery, is rare and has an unknown pathophysiology. We suggest a novel theory of spontaneous cystic BAVM formation, subsequently treated with endovascular embolization followed by surgery with a favorable long-term outcome.

Biallelic mutations in the ferredoxin reductase gene cause novel mitochondriopathy with optic atrophy.

Iron-sulfur (Fe-S) clusters are ubiquitous cofactors essential to various cellular processes, including mitochondrial respiration, DNA repair, and iron homeostasis. A steadily increasing number of disorders are being associated with disrupted biogenesis of Fe-S clusters. Here, we conducted whole-exome sequencing of patients with optic atrophy and other neurological signs of mitochondriopathy and identified 17 individuals from 13 unrelated families with recessive mutations in FDXR, encoding the mitochondrial membrane-associated flavoprotein ferrodoxin reductase required for electron transport from NADPH to cytochrome P450.

A multifactorial study on nutritional status, binge eating and physical activity as main factors directly influencing body weight in Parkinson's disease.

Weight lossisa multifactorial disorder commonly affecting Parkinson's disease patients. The aim of this study was to investigate the relationship between body weight, nutritional status, physical activity, and Parkinson's disease-related factors. A total of 114 consecutive Parkinson's disease patients without dietary restrictions were evaluated prospectively with respect to: nutritional status (Mini Nutritional Assessment), physical activity level (Yale Physical Activity Survey), MDS-UPDRS score, olfactory function, depression, cognitive functionand impulse-control disorders, among other variables.

Environmental factors influencing multiple sclerosis in Latin America.

It is generally accepted that autoimmune diseases like multiple sclerosis (MS) arise from complex interactions between genetic susceptibility and environmental factors. Genetic variants confer predisposition to develop MS, but cannot be therapeutically modified. On the other hand, several studies have shown that different lifestyle and environmental factors influence disease development, as well as activity levels and progression.

Age may contribute to the increased frequency of axonal Guillain-Barré syndrome.

Introduction: The frequency of axonal Guillain-Barré syndrome (GBS) varies among countries. Previous studies supporting the high frequency of axonal GBS in South America have been carried out with pediatric populations. We seek to determine the frequency of axonal GBS in both children and adults in South America.

Headache and Treatment of Unruptured Intracranial Aneurysms.

Background And Purpose: The relationship between unruptured intracranial aneurysms (UIAs) and chronic headache and the impact of aneurysm treatment on headache outcome are controversial. The aim of this study was to determine clinical features of a supposedly primary headache in patients with UIA. We also assessed changes in headache characteristics after UIA treatment.

Self-Assessment of Disability in Parkinson's Disease: The MDS-UPDRS Part II Versus Clinician-Based Ratings.

Background: Parkinson's disease (PD) is characterized by motor and nonmotor symptoms that progress with time, causing disability. The performance of a disease-specific, self-applied tool for assessing disability, the MDS-UPDRS Part II, is tested against generic and rater-based rating scales.

Methods: An international, cross-sectional, observational study was performed.

How Do I Examine Postural Disorders in Parkinson's Disease?

Postural abnormalities in Parkinson's disease (PD) are considered the rule more than the exception and are disabling complications of the disease. These deformities include camptocormia, antecollis, Pisa syndrome, and scoliosis. Evidence to date suggests that postural deformities have a multifactorial pathophysiology, including muscular rigidity, axial dystonia, weakness due to myopathy, body scheme defects due to centrally impaired proprioception, and structural changes in the spine.

Anti-inflammatory effects of melatonin in multiple sclerosis.

Melatonin is a hormone with complex roles in the pathogenesis of autoimmune disorders. Over the years, it has become clear that melatonin may exacerbate some autoimmune conditions, whereas it alleviates others such as multiple sclerosis. Multiple sclerosis is an autoimmune disorder characterized by a dysregulated immune response directed against the central nervous system.

Facial tremors in patients with and without parkinsonism.

Facial (lip and jaw) tremors can be an early sign of Parkinson's disease (PD), essential tremor and other parkinsonisms. Its response to acute dopaminergic therapy and further predictive clinical diagnosis has not been previously addressed. The aim of this study was to evaluate facial tremors response to acute dopaminergic therapy and further predictive value for clinical diagnosis.

Motor features in Parkinson's disease with normal olfactory function.

Background: Normosmic Parkinson's disease (PD) might be a unique clinical phenotype with a more benign course when compared with hyposmic PD.

Objective: The objective of this study was to evaluate motor features and the acute levodopa response according to olfactory function.

Methods: A total of 169 de novo PD patients that underwent olfactory testing and acute levodopa challenge for clinical prediction of sustained long-term dopaminergic response were evaluated.

Analysis of four scales for global severity evaluation in Parkinson's disease.

Global evaluations of Parkinson's disease (PD) severity are available, but their concordance and accuracy have not been previously tested. The present international, cross-sectional study was aimed at determining the agreement level among four global scales for PD (Hoehn and Yahr, HY; Clinical Global Impression of Severity, CGIS; Clinical Impression of Severity Index, CISI-PD; and Patient Global Impression of Severity, PGIS) and identifying which of them better correlates with itemized PD assessments. Assessments included additional scales for evaluation of the movement impairment, disability, affective disorders, and quality of life.

The TAM family receptor tyrosine kinase TYRO3 is a negative regulator of type 2 immunity.

Host responses against metazoan parasites or an array of environmental substances elicit type 2 immunity. Despite its protective function, type 2 immunity also drives allergic diseases. The mechanisms that regulate the magnitude of the type 2 response remain largely unknown.

Influence of white matter MRI hyper-intensities on acute l-dopa response in patients with Parkinson's disease.

Objective: To determine whether brain white matter hyperintensities (WMH) influence l-dopa response in Parkinson's disease (PD) patients.

Methods: We prospectively evaluated 60 PD patients with an acute l-dopa challenge test, and assessed motor performance with the Movement Disorders Society revised Unified Parkinson's Disease Rating Scale (MDS-UPDRS) during "ON" and "OFF" medication states. Magnetic resonance images were examined using a visual semi-quantitative rating scale for quantification and distribution analysis of WMH.

Sphingosine 1-phosphate signaling in astrocytes: Implications for progressive multiple sclerosis.

Multiple sclerosis is an autoimmune disorder characterized by recurrent attacks against the central nervous system. After many years, certain patients enter a progressive disease phase, characterized by steady clinical deterioration. However, in 10-15% of patients, the disease is progressive from the beginning, and thus diagnosed as Primary Progressive Multiple Sclerosis.