Erb-Charcot paralysis
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A rare syndrome consisting of spinal syphilis with paraesthesia, spastic weakness, and tiredness of the legs associated with pain, sphincter disorders, exaggerated deep reflexes, muscle atrophy, sensory disorders, and paraplegia. Occurs in several forms. According to the original description, the syndrome is seen in young syphilitic patients usually six years or more after the initial infection. Onset of the paraplegia of the lower extremities is gradual. Prevalent in males. Differentiation of this condition from other neurological diseases is often impossible and is dependent upon evidence of syphilis in the blood or cerebrospinal fluid.
Most cases either autosomal dominant or autosomal recessive, respectively x-linked recessive. Von Strümpell in 1886 pointed to the familial clustering of the condition.
Bibliography
- W. H. Erb:
«Spinaler Symptomenkomplex».
Berliner Zeitschrift Psych, 1875; 32. Über die spastische Spinalparalyse (tabès dorsal spasmodique Charcot).
[Virchows] Archiv für pathologische Anatomie und Physiologie und für klinische Medicin, Berlin, 1877, 70: 241-267, 293-328. Über syphilitische Spinalparalyse.
Neurologisches Centralblatt, Leipzig, 1892, 11: 161-168. - J. M. Charcot:
Du tabès dorsal spasmodique.
Progrés médical, Paris, 1876, 5: 737-737. - A. von Strümpell:
Über eine bestimmte Form der primären combinirten Systemerkrankungen des Rückenmarks.
Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1886, 17: 217-38.