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“Dystrophia Myotonica” and the legacy of hans gustav wilhelm steinert
Shri Kant Mishra1, Sandeep Singh2, Brian Lee3, Shaweta Khosa4, Negar Moheb5, Vishal A Tandon6
1 Department of Neurology, David Geffen School of Medicine, University of California, Los Angeles; Department of Neurology, Veterans Affairs Greater Los Angeles Healthcare System; Department of Neurology, Olive View UCLA Medical Center, Sylmar; Department of Neurology, Keck School of Medicine, University of Southern California, Los Angeles, USA
2 Western University of Health Sciences, Pomona, USA
3 Department of Neurology, Veterans Affairs Greater Los Angeles Healthcare System, Sylmar, USA
4 Department of Neurology, Veterans Affairs Greater Los Angeles Healthcare System; Department of Neurology, Olive View UCLA Medical Center, Sylmar, USA
5 Department of Neurology, David Geffen School of Medicine, University of California, Los Angeles; Department of Neurology, Olive View UCLA Medical Center, Sylmar, USA
6 Department of Neurology, Veterans Affairs Greater Los Angeles Healthcare System, Sylmar; University of California, Santa Barbara, USA
Correspondence Address:
Prof. Shri Kant Mishra
Department of Neurology, Olive View UCLA Medical Center, 14445 Olive View Drive, Sylmar, California 91342
USA
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/aian.AIAN_182_17
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The objective of this analysis is to study the life of Hans Gustav Wilhelm Steinert and his role in identifying several neurologic disorders including myotonic dystrophy (DM). DM type 1 (DM1) is a commonly inherited adult muscle disorder. In 1909, its characteristics were first described by Hans Steinert (1875–1911), a German neurologist. Born in Dresden, Germany, Steinert studied philosophy and medicine at the Universities of Leipzig, Berlin, Freiberg, and Kiel. There, under the supervision of Heinrich Curschmann, he accomplished his own works on aphthongia, cerebral muscular atrophy, and cerebral hemiplegia. In 1909, he published his study on six patients exhibiting characteristic myotonia. With autopsy findings of muscular fibrosis, Steinert identified this independent symptom complex as “Dystrophien Myotoniker” (DM). Overall, Steinert's accurate clinical characterization, coupled with the first ever autopsy finding of this condition, laid the foundations of our current understanding about DM1. This review serves as a tribute to the achievements of Hans Steinert and provides an opportunity to understand the historical perspective of DM1. Information for this analysis was gathered from PubMed and libraries at University of Southern California and University of California, Los Angeles. In addition, personal communications with Professor Benedikt Schoser at The University of Munich, Professor Tiemo Grimm at The University of Wuerzburg, and Professor Peter Harper at The University of Wales are acknowledged.
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