|
|
| Línea 1: |
Línea 1: |
| ==Background==
| | #REDIRECT[[Lambert-Eaton myasthenic syndrome]] |
| *Disease of the neuromuscular junction leading to primary presentation of weakness
| |
| *Caused by auto-antibodies against the voltage-gated calcium channels at the NMJ
| |
| | |
| ==Clinical Features==
| |
| *Often presents with alteration in gait or difficulty rising from a chair
| |
| *Symmetrical muscle weakness and fatiguability often beginning in lower extremities
| |
| *Autonomic dysfunction (dry mouth, erectile dysfunction)
| |
| *Compared with myastenia gravis, ELS begins with lower extremities weakness and rarely begins with extraocular muscle weakness
| |
| *Respiratory failure can occur in late stages of the disease
| |
| *Paraneoplastic and autoimmune form of LEMS have similar signs and symptoms
| |
| | |
| ==Differential Diagnosis==
| |
| *Myasthenia Gravis
| |
| *Myositis
| |
| *Dermatomyositis
| |
| *Amyotrophic Lateral Sclerosis
| |
| *Limb-Girdle Muscle Dystrophy
| |
| *Inclusion Body Myositis
| |
| | |
| ==Diagnosis==
| |
| *Diagnosis is generally a clinical diagnosis
| |
| *Confirmation testing for VGCC antibodies
| |
| *Neurophysiologic testing
| |
| | |
| ==Management==
| |
| *Guanidine
| |
| *Aminopyridines
| |
| *Acetylcholinesterase inhibitors
| |
| *IVIG
| |
| *Glucocorticoids
| |
| *Plasma exchange
| |
| | |
| ==Disposition==
| |
| *Referral to rheumatology
| |
| *Any patients with risk factors for small cell lung cancer will need to be referred for evaluation of underlying malignancy
| |
| | |
| ==See Also==
| |
| | |
| ==External Links==
| |
| | |
| ==References==
| |
| <UpToDate>
| |