-Lambert-Eaton syndrome.

Lambert-Eaton syndrome
or
Myasthenic-myopayhic syndrome.

*Pathophysiology:-

• a myasthenic syndrome due to autoimmune orocess targeting the mechanism of releasing A.CH from nerve terminals.
• It often associated with bronchogenic carcinoma or other malignancies.

*Clinical picture:-
Proximal weakness,wasting and fatigue but with less common ocular and bulbar affection than Myasthenia.

*Diagnosis:-

• EMG:- shows paradoxical increase  in successive muscle contractions.
• No significant response to prostigmine,but respond to guanidine HCL 20 mg/Kg/day or prednisolone+imuran.
• No acetylecholine receptor antibodies.
• CXR to determine if associated with bronchogenic carcinoma or not.

*Treatment:-

• Treatment of the underlying neoplasm.
• Plasmapheresis.
• Immunosuppression.

Dr Ibrahim

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