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
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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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