Chronic inflammatory demyelinating polyneuropathy associated with active systemic lupus erythematous: Anifrolumab as a potentially successful add-on therapy to intravenous immunoglobulins.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, autoimmune peripheral neuropathy, rarely reported as being associated with systemic lupus erythematous (SLE). We report the case of 53-year-old women with a long history of SLE, diagnosed with CIDP during a lupus flare beginning with progressive muscle weakness of lower and upper limbs, without affection of the central nervous system. She received treatment with steroids, intravenous immunoglobulins and anifrolumab and showed clinical and immunological improvement.

Multisystemic -Related Disorder: Expanding the Phenotype Beyond Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome.

Background And Objectives: The spectrum has become considerably expanded as multisystemic features beyond the triad of cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) have started to be unveiled, although many still require clinical replication. Here, we aimed to clinically characterize a cohort of -positive patients by addressing both classic and multisystemic features. In a second part of this study, we prospectively assessed small nerve fibers (SNF) and autonomic function in a subset of these -related patients.

[Guidelines for the Diagnosis and Treatment of Spontaneous Intracranial Hypotension].

Spontaneous intracranial hypotension (SIH) is a syndrome characterized by disabling orthostatic headache, due to reduced cerebrospinal fluid (CSF) volume probably caused by a CSF fistula. It affects mostly women of working-age, although it is probably underdiagnosed. The aim of this article is to present a practical approach to the diagnosis and treatment of SIH.

Oculomotor nerve palsy, an unusual onset of polyarteritis nodosa.

Introduction: Cranial nerve involvement in polyarteritis nodosa(PAN) is underrecognized and rarely reported. The aim of this article is to review the available literature and present an example of oculomotor nerve palsy in the course of PAN.

Material And Methods: Evaluation of texts describing the analyzed problem using the terms "polyarteritis nodosa", "nerve", "oculomotor", "cranial nerve" and "cranial neuropathy" for searching the PubMed database was done.

Congenital myopathies in adults: A diagnosis not to overlook.

Background: Congenital myopathies (CM) were traditionally classified according to the muscle histopathological features, but in recent years, molecular diagnosis has become increasingly important. CM may present a wide phenotype variability, and while adult-onset CM have been increasingly recognized, substantial diagnostic delays are still reported.

Objectives: To describe a cohort of adult CM patients, including clinical, genetic, and histopathological features, and further characterize the subgroup of adult-diagnosed patients.

Risk factors for idiopathic myelitis at admission and predictors for late diagnostic change.

Immune-mediated myelopathy (IMM) diagnosis is challenging, and its etiology may remain unclear despite extensive investigation. We evaluated diagnostic changes in IMM patients during follow-up. We included 80 patients, 61.

Neuromuscular Junction Abnormalities in Mitochondrial Disease: An Observational Cohort Study.

Objective: To determine the prevalence of neuromuscular junction (NMJ) abnormalities in patients with mitochondrial disease.

Methods: Eighty patients with genetically proven mitochondrial disease were recruited from a national center for mitochondrial disease in the United Kingdom. Participants underwent detailed clinical and neurophysiologic testing including single-fiber electromyography.

Optic neuropathy: A 15-year retrospective observational study.

Background: Optic neuropathies (ON) have several aetiologies and sometimes the diagnosis established ab initio is redefined after further investigations and/or new neurological events. We aim with this study to report clinical, paraclinical findings, treatment choices and disease course in patients admitted with a suspicion of acute or subacute optic neuropathy and to explore the diagnosis redefinition during follow-up and evaluate possible predictive factors that may influence that change.

Methods: We retrospectively reviewed the medical records of 156 patients with ON admitted to the ward of our Neurology Department, between January 2004 and August 2019.

A multicenter, non-interventional study to evaluate the disease activity in Multiple Sclerosis after withdrawal of Natalizumab in Portugal.

Objectives: Natalizumab (NTZ) is very effective for treatment of relapsing-remitting multiple sclerosis (RRMS), its use is mainly limited by safety issues. Discontinuation of NTZ is associated with recurrence of disease activity (reactivation and rebound). The best strategy for subsequent therapy and the predictive factors for recurrence in such patients are areas of active research.

Brody disease: when myotonia is not myotonia.

A 56-year-old man presented with painless impairment of muscle relaxation on vigorous contraction (eg, eyelid closure, hand grip, running). There were no episodes of paralysis, symptom progression, weakness or extramuscular symptoms. Five of his fifteen siblings had similar complaints.

Functional magnetic resonance imaging of human motor unit fasciculation in amyotrophic lateral sclerosis.

A novel diffusion-weighted magnetic resonance imaging protocol sensitive to contraction of individual skeletal motor units was developed. We applied this technique to the lower limb muscles of 4 patients with confirmed amyotrophic lateral sclerosis (ALS) and 6 healthy controls. A 3-minute scan revealed florid fasciculation in ALS patients, involving both superficial and deep muscles, and at a frequency higher than in healthy controls.

Synthesis and characterization of Locust Bean Gum derivatives and their application in the production of nanoparticles.

The development of LBG-based nanoparticles intending an application in oral immunization is presented. Nanoparticle production occurred by mild polyelectrolyte complexation, requiring the chemical modification of LBG. Three LBG derivatives were synthesized, namely a positively charged ammonium derivative (LBGA) and negatively charged sulfate (LBGS) and carboxylate (LBGC) derivatives.

Chitosan/sulfated locust bean gum nanoparticles: In vitro and in vivo evaluation towards an application in oral immunization.

This work proposes the design of nanoparticles based on locus bean gum (LBG) and chitosan to be used as oral immunoadjuvant for vaccination purposes. LBG-based nanoparticles were prepared by mild polyelectrolyte complexation between chitosan (CS) and a synthesized LBG sulfate derivative (LBGS). Morphological characterization suggested that nanoparticles present a solid and compact structure with spherical-like shape.

Monitorization of drug content in furosemide and lorazepam tablets stored in multidose pill boxes.

Background: Therapeutic nonadherence is a major health problem, particularly when therapeutic regimens are complex and long-lasting. Therefore, tools such as multidose pill boxes have been designed to provide the means for higher therapeutic compliance. However, no studies are available reporting on their capacity to keep the drug content of the stored tablets unaltered.