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Archivum Immunologiae Et Therapiae... Feb 2012Myasthenia gravis (MG) is an autoimmune disease caused by an immunological response against the acetylcholine receptor (AChR) at the neuromuscular junction. Anti-AChR... (Review)
Review
Myasthenia gravis (MG) is an autoimmune disease caused by an immunological response against the acetylcholine receptor (AChR) at the neuromuscular junction. Anti-AChR antibodies induce degradation of the receptor, activation of complement cascade and destruction of the post-synaptic membrane, resulting in a functional reduction of AChR availability. The pathophysiological role of autoantibodies (auto-Abs) and T helper lymphocytes has been studied in the experimental autoimmune MG (EAMG) models. EAMG models have been employed to investigate the factors involved in the development of MG and to suggest new therapies aimed to preventing or modulating the ongoing disease. EAMG can be induced in susceptible mouse and rat strains, which develop clinical symptoms such as muscular weakness and fatigability, mimicking the human disease. Two major types of EAMG can be induced, passive and active EAMG. Passive transfer MG models, involving the injection of auto-Abs, are helpful for studying the role of complement molecules and their regulatory proteins, which can prevent neuromuscular junction degradation. Active models, induced by immunization, are employed for the analysis of antigen-specific immune responses and their modulation in order to improve disease progression. In this review, we will concentrate on the main pathogenic mechanisms of MG, focusing on recent findings on EAMG experimental models.
Topics: Animals; Autoantibodies; Complement System Proteins; Disease Models, Animal; Humans; Immune Tolerance; Immunotherapy; Myasthenia Gravis; Myasthenia Gravis, Autoimmune, Experimental; Neuromuscular Junction; Receptors, Cholinergic
PubMed: 22159475
DOI: 10.1007/s00005-011-0158-6 -
Muscle & Nerve Mar 2013
Topics: Adult; Electromyography; Female; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Myasthenia Gravis; Neurologic Examination; Prognosis; Speech Disorders
PubMed: 23322599
DOI: 10.1002/mus.23645 -
Revue de L'infirmiere Apr 1991
Topics: Clinical Protocols; Humans; Myasthenia Gravis
PubMed: 1853079
DOI: No ID Found -
Meditsinskaia Sestra May 1990
Topics: Combined Modality Therapy; Humans; Myasthenia Gravis
PubMed: 2381294
DOI: No ID Found -
Il Policlinico. Sezione Pratica Nov 1971
Topics: Diagnosis, Differential; Humans; Methods; Myasthenia Gravis
PubMed: 5128032
DOI: No ID Found -
Archives of Neurology Feb 1980Familial infantile myasthenia is a rare type of myasthenia that usually occurs in connection with respiratory depression. The condition is characterized by (1) absence...
Familial infantile myasthenia is a rare type of myasthenia that usually occurs in connection with respiratory depression. The condition is characterized by (1) absence of myasthenia in the mother, (2) occurrence of a similar disorder among siblings, (3) respiratory depression at birth, (4) episodic weakness and apnea during the first two years of life, and (5) improvement with age. Since the condition responds to anticholinesterase medication, early diagnosis is important. Familial infantile myasthenia is a potential cause of sudden infant death and should be considered in infants with unexplained respiratory distress.
Topics: Adolescent; Apnea; Child; Child, Preschool; Edrophonium; Follow-Up Studies; Humans; Infant; Male; Myasthenia Gravis; Neostigmine; Physical Exertion; Synaptic Transmission
PubMed: 6243929
DOI: 10.1001/archneur.1980.00500510075018 -
South African Medical Journal =... Aug 2015
Topics: Autoantibodies; Humans; Incidence; Myasthenia Gravis; Receptors, Cholinergic
PubMed: 26543935
DOI: 10.7196/samjnew.8328 -
The Veterinary Record Nov 1974
Topics: Action Potentials; Animals; Dog Diseases; Dogs; Electromyography; Esophageal Achalasia; Female; Male; Myasthenia Gravis; Neostigmine; Physical Exertion
PubMed: 4446286
DOI: 10.1136/vr.95.20.452 -
Scottish Medical Journal Jul 1977
Topics: Antibodies; Humans; Myasthenia Gravis; Neuromuscular Junction; Synaptic Transmission; Thymectomy
PubMed: 196330
DOI: 10.1177/003693307702200305 -
Neurology India 2008We present the findings from the largest hospital-based studies on myasthenia gravis from India, using data collected over a period of 43 years from the Neurology...
BACKGROUND
We present the findings from the largest hospital-based studies on myasthenia gravis from India, using data collected over a period of 43 years from the Neurology Department in a tertiary referral center in India.
OBJECTIVES
To study the clinical presentation, age at onset, gender distribution, serological status and thymic pathology in patients with myasthenia gravis.
MATERIALS AND METHODS
A retrospective study was carried out using records of patients with myasthenia gravis from the years 1965 to 2008.
RESULTS
Of 841 patients, 836 (611 males and 225 females) had acquired myasthenia (myasthenia gravis) and five congenital myasthenia. The median age at onset was 48 years (males 53 years and females 34 years). The peak age at onset for males was in the sixth and seventh decade and in females, in the third decade. Two hundred and twenty-two (26.31%) patients had ocular and 616 (73.68%) generalized myasthenia. Serological studies were done in 281 patients with myasthenia gravis for Acetylcholine receptor (AchR) antibodies of which 238 (84.70%) were seropositive. The most common histopathology was thymoma and the second most common was thymic hyperplasia.
CONCLUSION
Myasthenia gravis in our study was more common in males (M:F of 2.70:1). There was a single peak of age at onset (males sixth to seventh decade; females third decade). The higher prevalence of thymomas in this series is in all probability related to selection bias as patients with thymic enlargement or more severe disease underwent thymectomy. Thymoma was more common in males; hyperplasia in females.
Topics: Adolescent; Adult; Age Distribution; Age Factors; Age of Onset; Aged; Aged, 80 and over; Antibodies; Child; Child, Preschool; Electrophysiology; Female; Humans; India; Infant; Male; Middle Aged; Myasthenia Gravis; Receptors, Cholinergic; Retrospective Studies; Sex Distribution; Thymectomy; Young Adult
PubMed: 18974563
DOI: 10.4103/0028-3886.43455