The Life Behind the Syndromes: Joseph Gerstmann’s Lasting Influence in Neurology
Divya Nayar1, Sama Almasri1
1University of Arkansas for Medical Sciences
Objective:
To explore the life and contributions of Joseph Gerstmann, the neurologist behind the discovery of the well-known Gerstmann tetrad and the rare prion disorder Gerstmann-Sträussler-Scheinker (GSS) syndrome.
Background:

In 1924, while working in Vienna, Joseph Gerstmann described a 52-year-old woman with a left-sided stroke. She exhibited symptoms including finger agnosia, left-right disorientation, agraphia, and acalculia—now recognized as Gerstmann syndrome. Gerstmann initially attributed these deficits to the dominant parietal lobe. Gerstmann later collaborated with Mark Scheinker and Ernst Sträussler to identify a rare hereditary prion disease, now known as Gerstmann-Sträussler-Scheinker (GSS) syndrome. Discovered in an Austrian family and initially misdiagnosed as neurosyphilis, GSS presents with cerebellar ataxia progressing to dementia. It is now classified as part of the transmissible spongiform encephalopathies (TSEs).

Design/Methods:

Literature review.

Results:

Despite his achievements, Gerstmann struggled to reestablish his career in the U.S., spending most of his later years in private practice. He battled professional setbacks and legal challenges to recover confiscated property from Vienna. Gerstmann passed away in 1969, but his work continues to influence modern neurology. Recent studies suggest the symptoms may result from disconnection of co-localized fiber tracts in the subcortical white matter, rather than damage to a single cortical region. Modern imaging supports this hypothesis through combined functional and structural mapping of the healthy brain.

Conclusions:

Joseph Gerstmann’s contributions reflect the importance of curiosity, persistence, and research. His syndromes inspire ongoing studies in neurology. His life also illustrates how Nazi persecution disrupted the careers of renowned neuroscientists, delaying scientific progress.

10.1212/WNL.0000000000212576
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.