Brain Advance Access published online on November 10, 2009
Brain, doi:10.1093/brain/awp281
Review Article |
The enigma of Gerstmann's syndrome revisited: a telling tale of the vicissitudes of neuropsychology
1 INSERM, Unité 562, F-91191 Gif-sur-Yvette, France 2 Institute of Cognitive Neuroscience, University College London, London, UK 3 Laboratories of Functional Neuroimaging, Center for Mind/Brain Sciences (CIMeC), University of Trento, Via delle Regole, 101, 38060 Mattarello (TN), Italy 4 CEA, DSV, I2BM, NeuroSpin, F-91191 Gif-sur-Yvette, France 5 Université Paris-Sud, F-91405 Orsay, France
Correspondence to:
Elena Rusconi, Laboratories of Functional Neuroimaging, Center for Mind/Brain Sciences (CIMeC), University of Trento, Via delle Regole, 101 38060 Mattarello (TN), Italy E-mail: elena.rusconi{at}gmail.com
Eighty years ago, the Austrian neurologist Josef Gerstmann observed in a few patients a concomitant impairment in discriminating their own fingers, writing by hand, distinguishing left from right and performing calculations. He claimed that this tetrad of symptoms constituted a syndromal entity, assigned it to a lesion of the dominant parietal lobe and suggested that it was due to damage of a common functional denominator. Ever since, these claims have been debated and an astute synopsis and sceptical discussion was presented 40 years ago by MacDonald Critchley in this journal. Nonetheless, Gerstmann's syndrome has continued to intrigue both clinical neurologists and researchers in neuropsychology, and more frequently than not is described in textbooks as an example of parietal lobe damage. In this review, we revisit the chequered history of this syndrome, which can be seen as a case study of the dialectic evolution of concepts in neuropsychology. In light of several modern era findings of pure cases we conclude that it is legitimate to label the conjunction of symptoms first described by Gerstmann as a ‘syndrome’, but that it is very unlikely that damage to the same population of cortical neurons should account for all of the four symptoms. Instead, we propose that a pure form of Gerstmann's syndrome might arise from disconnection, via a lesion, to separate but co-localized fibre tracts in the subcortical parietal white matter, a hypothesis for which we have recently provided evidence using combined imaging of functional and structural organization in the healthy brain.
Key Words: Gerstmann syndrome; acalculia; agraphia; left–right confusion; finger agnosia
Received July 6, 2009. Revised August 29, 2009. Accepted September 18, 2009.