Wilson-Krankheit und Westphal-Strümpell-Pseudosklerose

Abstract

The article investigates the hitherto little elaborated conceptual history of Wilson's hepatolenticular degeneration and Westphal's pseudosclerosis from the mid-nineteenth century up to present day, mainly in German-speaking countries. This disease exemplifies how neurological diseases have come into existence and also demonstrates how clinical descriptions of a few isolated cases merge into a nosological diagnosis, how recognized therapeutic measures are developed and how the underlying genetic causes are identified. For some time German-speaking neurologists were in disagreement about whether or not a disease described by Carl Westphal in 1883 was identical with one described by and later named after Kinnier Wilson. This article presents the publications by Adolf Strümpell, Alois Alzheimer, August Bostroem and Walther Spielmeyer as the major contributions of German-speaking specialists to a deeper understanding of an illness and investigates whether or not the two disease concepts represent identical illnesses or not. From 1970 onwards the "Morbus Wilson Center" in Leipzig became important for the development of recognized therapeutic measures and their application in medical practice in Eastern Germany (the former GDR) and its findings have been incorporated in the present Guidelines of the German Society for Neurology published in 2008 for the treatment of Wilson's disease.

The history of Wilson disease

“…[2] Yet the term Pseudosclerosis persisted for some time after SAKW published his paper in Brain describing the neurological aspects and other associations of what became known eponymously as his disease, namely WD. [2,25] However, Spielmeyer in 1920 [26] and Hall in 1921, [27] in their analyses, concluded that pseudosclerosis and Wilson disease were the same entity. Thus, Spielmeyer from Munich reported the neuropathology of pseudosclerosis and WD.…”

“…SAKW, in an addendum to his paper in Brain, drew attention to a paper by Völsch 24 published in 1911 describing another patient with possible pseudosclerosis and cirrhosis, but noted (on page 508) that “this unsatisfactory and makeshift expression … coined for certain cases which were said to resemble disseminated sclerosis clinically, but not pathologically, should be abandoned, and that re-examination of the subject should be undertaken” 2 . Yet the term Pseudosclerosis persisted for some time after SAKW published his paper in Brain describing the neurological aspects and other associations of what became known eponymously as his disease, namely WD 2,25 . However, Spielmeyer in 1920 26 and Hall in 1921, 27 in their analyses, concluded that pseudosclerosis and Wilson disease were the same entity.…”

Neurologie in der DDR: eine systematische Literaturübersicht zur historischen Aufarbeitung

“…[ 33 ] 2010 Personen Zeitschriftenartikel Entwicklung der Kinderneuropsychiatrie in Rostock Institutionen Fächerdifferenzierung Kumbier [ 25 ] 2010 Personen Habilitationsschrift Entwicklung der Nervenheilkunde in der DDR zwischen 1946 und 1961 Politische und ideologische Einflussnahme Boide et al. [ 9 ] 2011 Diagnostik und Therapie Zeitschriftenartikel „Zentralstelle für Morbus Wilson“ der DDR in Leipzig Eisenberg [ 16 ] 2011 Fachgesellschaften und Fachzeitschriften Buchbeitrag Übersicht über den persönlichen und inhaltlichen Austausch der west- und ostdeutschen Fachgesellschaften für Neurologie zwischen 1945 und 1970 Politische und ideologische Einflussnahme Eisenberg [ 17 ] 2012 Fachgesellschaften und Fachzeitschriften Buchbeitrag Kontroverse um die Aufnahme der DDR in die World Federation of Neurology (1960) Politische und ideologische Einflussnahme Matthies [ 36 ] 2012 Personen Buch Autobiografie inklusive der Entstehungsgeschichte des Zentrums für Neurowissenschaften in Magdeburg Fächerdifferenzierung Teitge [ 46 ] 2013 Fachgesellschaften und Fachzeitschriften Dissertationsschrift Entstehung der einzigen DDR-Fachzeitschrift für Neurologie und Psychiatrie und Analyse der Fachbeiträge Politische und ideologische Einflussnahme Kumbier et al. [ 30 ] 2015 Personen Zeitschriftenartikel Hochschullehrer an den Universitäts-Nervenkliniken in der SBZ und DDR bis 1961 Institutionen Politische und ideologische Einflussnahme Teitge et al.…”

“…Auch bei den 11 Aufsätzen der Kategorie Diagnostik und Therapie beschäftigen sich allein fünf Arbeiten mit der Liquordiagnostik [ 7 , 8 , 11 , 35 , 39 ], bei den Beiträgen zur Epileptologie [ 38 ] und der Neurotraumatologie [ 5 , 6 ] liegt der Fokus auf der Subspezialisierung. Dazu fand sich für Leipzig mit der Zentralstelle für Morbus Wilson ein Alleinstellungsmerkmal [ 9 , 53 ].…”