Amyotrophic lateral sclerosis and myasthenia gravis: association or chance occurrence?

AbstractVery few cases of patients with myasthenia gravis (MG) who later developed amyotrophic lateral sclerosis (ALS) have been described, although some studies showed that significantly more cases than expected have ALS associated with a prior diagnosis of autoimmune diseases. Our aim was to investigate whether the association of ALS and MG was higher than expected in a population-based study and to describe the clinical features characterizing these patients. In Emilia Romagna Region of Italy, a prospective registry has been collecting all incident ALS cases since 1.1.2009. For each patient, detailed clinical information is collected by caring physicians, including comorbidities. From 1.1.2009 to 31.12.2014, 671 patients were diagnosed with ALS; five patients (0.75%) were also affected by MG. Considering Western Countries incidence rates the occurrence of both the diseases should be a really exceptional event (7.5/109), compared to our findings (1.87/107) (p 
Source: Neurological Sciences - Category: Neurology Source Type: research

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, Casadó V Abstract Dopaminergic and purinergic signaling play a pivotal role in neurological diseases associated with motor symptoms, including Parkinson's disease (PD), multiple sclerosis, amyotrophic lateral sclerosis, Huntington disease, Restless Legs Syndrome (RLS), spinal cord injury (SCI), and ataxias. Extracellular dopamine and adenosine exert their functions interacting with specific dopamine (DR) or adenosine (AR) receptors, respectively, expressed on the surface of target cells. These receptors are members of the family A of G protein-coupled receptors (GPCRs), which is the largest protein superf...
Source: Advances in Pharmacology - Category: Drugs & Pharmacology Authors: Tags: Adv Pharmacol Source Type: research
Publication date: Available online 25 June 2019Source: Life SciencesAuthor(s): Mehdi Derakhshani, Hossein Abbaszadeh, Ali Akbar Movassaghpour, Amir Mehdizadeh, Majid Ebrahimi-Warkiani, Mehdi YousefiAbstractHematopoietic stem cells (HSCs) are a rare cell population in adult bone marrow, mobilized peripheral blood, and umbilical cord blood possessing self-renewal and differentiation capability into a full spectrum of blood cells. Bone marrow HSC transplantation has been considered as an ideal option for certain disorders treatment including hematologic diseases, leukemia, immunodeficiency, bone marrow failure syndrome, genet...
Source: Life Sciences - Category: Biology Source Type: research
Authors: Berth SH, Lloyd TE PMID: 31233365 [PubMed - as supplied by publisher]
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research
Mechanisms underlying motor neuron degeneration in amyotrophic lateral sclerosis (ALS) are yet unclear. Specific deletion of the ER-component membralin in astrocytes manifested postnatal motor defects and lethality in mice, causing the accumulation of extracellular glutamate through reducing the glutamate transporter EAAT2. Restoring EAAT2 levels in membralin-KO astrocytes limited astrocyte-dependent excitotoxicity in motor neurons. Transcriptomic profiles from mouse astrocytic membralin-KO motor cortex indicated significant perturbation in KEGG pathway components related to ALS, including downregulation of Eaat2 and upreg...
Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research
ConclusionWe present a detailed clinical and laboratory analysis of the patient. This case report will emphasize the importance of evaluating anti-MuSK and anti-LRP4 antibodies even in patients with anti-AChR antibodies.
Source: Clinica Chimica Acta - Category: Laboratory Medicine Source Type: research
CONCLUSION: We present a detailed clinical and laboratory analysis of the patient. This case report will emphasize the importance of evaluating anti-MuSK and anti-LRP4 antibodies even in patients with anti-AChR antibodies. PMID: 30006288 [PubMed - as supplied by publisher]
Source: International Journal of Clinical Chemistry - Category: Chemistry Authors: Tags: Clin Chim Acta Source Type: research
Abstract Myasthenia gravis (MG) is a common disorder that affects the neuromuscular junction. It is caused by antibodies against acetylcholine receptor and muscle‐specific tyrosine kinase; however, some MG patients do not have antibodies against either of the proteins. Recent studies have revealed antibodies against agrin and its receptor LRP4—both critical for neuromuscular junction formation and maintenance—in MG patients from various populations. Results from experimental autoimmune MG animal models indicate that anti‐LRP4 antibodies are causal to MG. Clinical studies have begun to reveal the significanc...
Source: Annals of the New York Academy of Sciences - Category: Science Authors: Tags: REVIEW Source Type: research
Abstract: The Guillain–Barré syndrome (GBS) is one of the few neuropathies well known to the general public, in part because of its association with swine flu vaccinations in 1976. GBS has again reached the general public with its possible association with Zika virus. The virus, borne by infected Aedes aegypti mosquitos, is being linked to birth defects when pregnant women are bitten and infected. There are early reports also linking GBS to Zika infection, which could expose a wider range of infected people to the neuropathy. This summer infected Aedes mosquitos will likely reach southern portions of the Unite...
Source: Journal of Clinical Neuromuscular Disease - Category: Neurology Tags: Literature Review Source Type: research
When evaluating a patient with progressive weakness for the first time, it is occasionally difficult to distinguish a motor neuron disease from an autoimmune neuromuscular disorder. Our neurological testing may even cloud the picture, as nearly 1/3 of patients with amyotrophic lateral sclerosis (ALS) may have decrement on repetitive nerve stimulation [1] and the risk of ALS is increased in people with concomitant autoimmune disease including myasthenia gravis (MG) [2]. In this context, the neurologist may wish to commence immunotherapy given the dismal alternative.
Source: Neuromuscular Disorders - Category: Neurology Authors: Tags: Editorial Source Type: research
Abstract: In this issue, we review clinical features associated with an elevated serum creatine kinase level found in a percentage of patients with amyotrophic lateral sclerosis (ALS). The treatment of ALS remains problematic, and issues with offerings on the internet for unregulated stem cell treatment and the movement for right-to-try experimental drugs are discussed. The last What's in the Literature? discussed information about recording from stimulating electrodes implanted in the diaphragm of ALS patients, and this issue discusses the results of a randomized trial using diaphragm pacing. Chronic inflammatory demyelin...
Source: Journal of Clinical Neuromuscular Disease - Category: Neurology Tags: Literature Review Source Type: research
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