164-10 Familial Myasthenia Gravis
Identifier: 164-10 Title: Familial Myasthenia Gravis Subject: Familial Myasthenia Gravis; External Ophthalmoplegia; Ocular Myasthenia Gravis; Unilateral Ptosis; Unilateral Myasthenia Gravis; Description: In 1999 Dr. A. G. Engel published a monograph on Myasthenia Gravis and Myasthenic Disorders with a chapter devoted to Congenital Myasthenic Syndromes. Dr. Engel systematically defined and classified hereditary and congenital myasthenic syndromes, delineated on the basis of their electrophysiological and ultra structural features. The congenital myasthenic syndromes are inherited defects in components of the presynaptic, synaptic, or post-synaptic junctional apparatus. It has been estimated that in three quarters of the cases the defect is post-synaptic.