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Neuromuscular disorder characterized by muscle weakness and rapid muscle fatigue. Usually apparent during adulthood; onset may occur at any age. Most individuals present eyelid ptosis, diplopia, and excessive muscle fatigue following exercise. Other clinical features commonly include dysarthria, dysphagia, and proximal limb weakness. Approximately 10% may develop potentially life-threatening complications as a consequence of severe respiratory depression (myasthenic crisis).

Goldflam-Erb Disease; Erb Syndrome; Erb-Goldflam Disease; Erb-Oppenheim-Goldflam Syndrome; Hoppe-Goldflam Syndrome; Hoppe-Goldflam Syndrome Complex; Asthenic Bulbar Paralysis; Bulbospinal Paralysis; Myasthenia Gravis Pseudoparalytica.

  • Classic Generalized Myasthenia Gravis
  • Ocular Myasthenia Gravis
  • Transitory Neonatal Myasthenia Gravis
  • Juvenile Myasthenia Gravis
  • Congenital Myasthenia Gravis
  • Familial Infantile Myasthenia Gravis
  • Congenital Endplate Acetylcholinesterase Deficiency
  • Slow Channel Syndrome
  • Fast Channel Syndrome

The first written description of myasthenia gravis is granted to Thomas Willis (1621-1675), an English physician, who published a book in 1672 entitled: De anima brutorum in which he wrote about “a woman who temporarily lost her power of speech and became `mute as a fish.'" This was subsequently interpreted as myasthenia gravis. Thomas Willis was born in Great Bedwin, a Wiltshire village in England. He graduated from Oxford University Legion with a Bachelor of Medicine in 1646. In 1660 he was appointed Sedleian Professor of Natural Philosophy at Oxford and given the diploma of MD. The earliest description of the disease was done by Samuel Wilks, an English physician, in 1877 which was reported in Guy's Hospital Reports 22:7. However, it seems that it was a case of “bulbar paralysis." Aleksei Kozhevnikov, a Russian physician, described a “progressive familial spastic diplegia” and wrote on the neuropathology of nuclear ophtalmoplegia, myasthenia, and bulbar paralysis. His textbook on nervous diseases was a popular manual because of its brevity and lucidity. He was born in Ryazan and entered the University of Moscow in 1853 and received his MD in 1860. He later studied abroad in Germany, England, Switzerland, and France. Wilhem Heinrich Erb (1840-1921), a German neurologist, was responsible (in part) for the delineation of myasthenia gravis (called Erb-Goldflam-Oppenheim disease at the time). Finally, the use of physostigmine in the treatment of myasthenia gravis is granted to Mary Broadfoot Walker (1896-1974), a salaried Assistant Medical Officer in “Poor Law Service" at St. Alfege's Hospital, Greenwich, England, who introduced it in 1934.

Prevalence in general population is 50 to 125:1 million.

Sporadic for acquired cases.

In acquired cases, there is a decrease in the number of acetylcholine receptors (AChR) at the neuromuscular junction. This decrease is secondary to circulating antibodies against AChR, which increase the rate of degradation of AChR and decrease its rate of synthesis. These antibodies are present in 80 to 90% of the classic and juvenile forms of myasthenia; their production occurs in the thymus. The thymus is abnormal in 80 to 90% of those patients: thymic follicular hyperplasia (65-75%), thymoma (10%, only in adults), and ...

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