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Erb-goldflam syndrome (erb ii syndrome; hoppe-goldflam disease; pseudoparalytic syndrome; myasthenia gravis)


Erb-Goldflam Syndrome (Erb II Syndrome; Hoppe-Goldflam Disease; Pseudoparalytic Syndrome; Myasthenia Gravis) 141f53b 141f53b 141f53b 141f53b 141f53b 141f53b

General: Occurs at any age; more frequent between ages 20 and 40 years; more females affected than males; progressive; spontaneous; symptoms improve or resolve with rest in early stages of disease (see Myasthenia Gravis, Neonatal or Infantile); caused by autoantibodies against the acetylcholine receptor at the neuromuscular junction, leading to abnormal fatigability and weakness of skeletal muscle.

Ocular: Transient diplopia; ptosis of upper eyelids.

Clinical: Excessive fatigability of musculature; symptoms appear and increase as day progresses; expressionless face; sagging jaw; difficulty in chewing and talking; nasal regurgitation.

Erb W. Zur Casuistick der Bulbaren La hmungen. Arch Psychiatr Vervenkr

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Goldflam S. Vebereinen Scheinbar Keilbaren Bulbarparalytischem Symptom Complex mit Betheiligung der Extremitaten. Dtsch Z Nerven 1983; 4:312-352.

Kim JH, Hwang JM, Hwang YS, et al. Chilhood ocular myasthenia gravis. Ophthalmology 2003; 110: 1458-l462.

Lepore FE. Pupillary dysfunction in myasthenia gravis. Ann Neurol 1979; 6:29-33.

Sommer N, et al. Ocular myasthenia gravis. A critical review of Clinical and pathophysiological aspects. Doc Ophthalmol 1993; 84:309-333.




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