Which of the following statements about Lambe Eaten Myaesthenic syndrome is true:
Correct Answer: Incremental response to repeated electrical stimulation
Description: Answer is C (Incremental response to repeated electrical stimulation ): Lambe Eaten Myaesthenic syndrome presents with an incremental response to repeated electrical stimulation. Lambe Eaten Myasthenia Syndrome What it is & Associations Paraneoplastic syndrome Associated with - Small cell carcinoma lung - Ceain autoimmune diseases Defect Presynaptic disorder Antibodies to calcium channels (P/Q type) Decrease in presynaptic release of Acetylcholine at Neuromuscular junction is the fundamental defect Presentation Onset usually after 40 years Men > Women (5: 1) Proximal limb muscles and muscles of trunk are the most commonly involved muscles Proximal limb muscles >> extraocular & lid muscles No fatiguability / Incremental response Repeated stimulation causes increased muscle strength Reflexes : Hyporeflexia or absent reflexes Autonomic changes such as dry mouth and impotence are associated Diagnosis Repeated nerve stimulation : incremental response Radioimmune assay : Antibodies against P/Q Ca2+ channels Tensilon test : Negative ( mildly positive in some) Myasthenia Gravis What it is & Associations Autoimmune disorder Associated with - Thymomas - Ceain autoimmune diseases Defect Postsynaptic disorder Antibodies against ACH Receptors Decrease in number of ACH Receptors at post synaptic muscle membrane is the fundamental defect Presentation Onset Often < 40 years Women > Men (4: 3) Craniofascial muscles paicularly lids and extraocular muscles are the most commonly involved. Extraocular & lid muscles > proximal limb muscles Fatiguability / Decremental response Repeated stimulation causes decreased muscle strength Reflexes : Intact No associated autonomic changes Diagnosis Repeated nerve stimulation : Decremental Radioimmune assay : - Anti ACHR antibody response - Anti-Musk antibodies Tensilon test : Characteristically Positive
Category:
Medicine
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now