Carl Westphal's 'pseudosclerosis' of 1883: A misleading term and a source of errors
After C. Westphal’s 1883 differentiation of ‘pseudosclerosis’ from multiple sclerosis, and after Strümpell’s description in 1898 of children dying from ‘pseudosclerosis’ (one of them with cirrhosis of the liver), the concept of Westphal-Strümpell pseudosclerosis survived for a long time, even after the discovery of the disease entity by K. Wilson in 1912 which was identical for most cases of ‘Westphal-Strümpell pseudosclerosis.’ Its clinical and histological characteristics were established in the first two decades of the 20th century in Germany (Kayser, Fleischer, Alzheimer, Spielmeyer, Opalski), parallel to Wilson’s putative delineation. After the contribution of Hall from Copenhagen in 1921, the term hepato-lenticular degeneration (Wilson) was adopted, but in Germany the application of Westphal-Strümpell pseudosclerosis to the adult type of Wilson’s disease continued until recently. On the other hand, the first of Westphal’s two cases got another interpretation: it was related to the akinetic-rigid, mostly juvenile type of Huntington’s disease (Kehrer 1928, Bittenbender and Quadfasel 1962), and the eponym “Westphal’s variant of HD” was created by Dutch authors (Mackenzie-va der Noorda 1960, Bruyn 1968). Finally the term ‘pseudosclerosis’ seems to have expired. Its long tenacious life especially in the German literature would have been unnecessary if Wilson’s advice from 1912 to abandon it had been followed.
Tenth Annual Meeting of the International Society for the
History of the Neurosciences (ISHN) and
St. Andrews, Scotland, 2005