Abstract
Introduction: Charcot-Marie-Tooth disease (CMT) is a group of inherited sensorymotor neuropathies with variable age of onset, clinical and neurophysiological patterns but often with a chronic slow progression. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy with a relapsing clinical course and typically good response to corticosteroids or other therapies. The distinction between these two conditions can be made with aid of clinical history, neurophysiological studies and genetic testing in the vast majority of cases. However, an overlap between them can occur. Methods: We describe four Brazilian patients under clinical follow-up at our service with genetic diagnosis of CMT and clinical and neurophysiological features compatible with a concurrent CIDP diagnosis. Results: Four cases of different CMT subtypes with co-occurrence of an immunemediated neuropathy compatible with CIDP were reported. The patients were all unrelated, two males and two females, age range from 3 to 45 years. The genetic mutations were the following: hemizygous pathogenic variant c.514C>T (p.Pro172Ser) in GJB1 gene (CMT1X), duplication of PMP22 gene (CMT1A), simple heterozygous pathogenic variant c.188_190delCCT (p.Ser64del) in MPZ gene (CMT1B) and homozygous pathogenic variant c.122T>C (p.Ile41Thr) in FIG4 gene (CMT4J). All four patients presented with relapsing or subacute worsening of neurological symptoms, demyelinating non-uniform features in neurophysiological studies including conduction blocks and elevated cerebrospinal fluid (CSF) protein levels without pleocytosis. Three patients (3/4) improved after treatment with corticosteroids, immunoglobulin or cyclophosphamide with variable clinical response. Conclusion: CMT and CIDP are different conditions involving the peripheral nervous system and the distinction between them usually is possible with the appropriate assessment. The overlap between them is possible and we report four cases with this association.
Highlights
Capsaicin is able to induce mast cell degranulation, an event probably related to the pathophysiology of a migraine attack
Objectives: The present review study aimed to address the mechanisms of action of capsaicin and other chemical inducers in mast cell degranulation and an interaction of nerves and events that happen in the dura mater with the activation of mast cells
The analyses showed significantly higher frequency of the genotype VV in those who had depression, compared with the allele A
Summary
Capsaicin is able to induce mast cell degranulation, an event probably related to the pathophysiology of a migraine attack. Neuroinfections are pathologies that affect the CNS, for example, we have Murcomycosis, a progressive infection caused by opportunistic fungi of the order Mucorales, with high frequency in Immunodepressed patients, Diabetes Mellitus (DM) is the main underlying pathology associated with the development of Rhinocerebral Murcomycosis, which represents 50% of the cases, with a mortality rate of 70% (Sidrim, 2012, p.168). The COVID-19 pandemic has been alarming the world since its first outbreak in December 2019 In this scenario, the presence of aggravating factors such as the elevation of the D-dimer and the reduction of the angiotensin-converting enzyme 2 (ACE2) during the clinical course of the disease, collaborated in the appearance of thromboembolic events derived from inflammatory processes and extensive intravascular coagulation, contributing to the emergence of diseases such as Hemorrhagic Stroke (ICH), leading the patient to have a worse clinical prognosis and a consecutive worsening of their health. Despite being classically associated with this etiology, the finding may be present in other diseases, especially infiltrative ones
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