Skip to Main Content

At a glance

It is a neuromuscular disorder characterized by significant muscle weakness and rapid muscle fatigue during mild exercise, such as a slow walk. Congenital myasthenias are present in the first years of childhood. Spontaneous forms of Myastheria Gravis (MS) are usually recognized during adulthood. Usually apparent during adulthood, the onset of the symptoms occurs at any age and spontaneously. 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).

Synonyms

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.

History

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 ophthalmoplegia, 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.

Classification

There are nine types of myasthenia gravis described. The clinical presentation and the age of onset of the symptoms define the type:

  • Transient neonatal myasthenia: Occurs in 10 to 15% of newborns whose mothers are affected by the disorder. It usually disappears after a few weeks of symptomatology.

  • Congenital myasthenia: Rarest form of the disease. It occurs when genes are present from both parents.

  • Juvenile myasthenia gravis: Most common form of the disease observed in females.

  • Classic Generalized Myasthenia Gravis: Characterized by a fluctuating degree of ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.