Neuromuscular junction disorders mimicking myopathy
نویسندگان
چکیده
منابع مشابه
The neuromuscular junction disorders.
Neuromuscular junction (NMJ) disorders result from destruction, malfunction or absence of one or more key proteins involved in neuromuscular transmission, illustrated diagrammatically in fig 1. The most common pathology is antibody mediated damage or down regulation of ion channels or receptors, resulting in myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and acquired neuromyo...
متن کاملAutoimmune disorders of the neuromuscular junction.
The neuromuscular junction lies beyond the protection of the blood-brain barrier and is particularly vulnerable to antibody-mediated attack. In myasthenia gravis, the expression of acetylcholine receptors (AChRs) in the thymus is under the control of the autoimmune regulator protein (AIRE), and polymorphisms in the AChR correlate with early onset of disease. In some 'AChR seronegative' patients...
متن کاملMyasthenia gravis and other neuromuscular junction disorders.
Correspondence to: Dr Saiju Jacob, Department of Neurology, Queen Elizabeth Neurosciences Centre, University Hospitals of Birmingham, B15 2TH, UK; [email protected] Myasthenia gravis is the most common autoimmune disease affecting the neuromuscular junction and is characterised by painless fatigable muscle weakness. It is caused by autoantibodies against neuromuscular junction proteins, eithe...
متن کاملSynaptic Homeostasis and Its Immunological Disturbance in Neuromuscular Junction Disorders
In the neuromuscular junction, postsynaptic nicotinic acetylcholine receptor (nAChR) clustering, trans-synaptic communication and synaptic stabilization are modulated by the molecular mechanisms underlying synaptic plasticity. The synaptic functions are based presynaptically on the active zone architecture, synaptic vesicle proteins, Ca2+ channels and synaptic vesicle recycling. Postsynapticall...
متن کاملNeuromuscular Junction Voice Disorders. Proposal of a Diagnostic Protocol
Submit Manuscript | http://medcraveonline.com Abbreviations: NMJD: Neuromuscular Junction Disorders; ENG: Electro Neurography; EMG: Electro Myography; RNS: Repetitive Nerve Stimulation; SFEMG: Single Fiber ElectroMyoGraphy; AChR Ab: AcetylCholine Receptor Antibody; anti-MuSk Ab: antiMuscle Specific kinase Antibody; TA: Tyro Arythenoid muscle; CMAP: Compound Motor Action Potential; Lrp4 Ab: Lipo...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Muscle & Nerve
سال: 2014
ISSN: 0148-639X
DOI: 10.1002/mus.24300