Pierre Marie’s état vermoulu to Spatz’ cortical granular atrophy and cortical microinfarcts: a scarcely recognised cerebral vascular pathology

J. J. Zarranz Imirizaldu
Neurosciences and History 2020;8(3):87-98

Type of article: ORIGINAL

AUTHOR

J. J. Zarranz Imirizaldu
Emeritus chair. Department of Neuroscience. Universidad del País Vasco, Instituto de Investigación BIOCRUCES, Barakaldo, Spain.

ABSTRACT

Introduction. Pierre Marie’s description of état lacunaire, the predominantly motor clinical syndrome associated with multiple lacunar infarcts, is well known. However, he also described état vermoulu, another vascular pathology characterised by even smaller infarcts limited to the cerebral cortex.

Material and methods. I reviewed original articles and communications by Pierre Marie and his school, and consulted classic neuropathology texts and today’ s literature to analyse the evolution of the concept of état vermoulu.

Results. Pierre Marie associated état vermoulu with age and cognitive impairment, and considered it a rare condition. To the contrary, Augusta Dejerine-Klumpke believed that these lesions were frequently identified in studies of serial sections of the brain. Thanks to Hugo Spatz, the concept of état vermoulu spread to the scientific literature of other countries under the name cortical granular atrophy. In the following decades, there was little interest in cortical microinfarcts, the underlying pathology in état vermoulu, mainly because they cannot be detected in basic neuroimaging studies (CT and MRI up to 3T). As a result, the clinical correlation with the two main syndromes associated with small-vessel disease, cognitive impairment and parkinsonism, led to a (probably biased) focus on subcortical lesions: these were the only lesions visible on basic neuroimaging studies, and the only ones taken into consideration in conventional neuropathology.

Discussion. Seven-Tesla MRI scanners enable us to view the cortical microinfarcts that cause état vermoulu, making it possible to estimate the true extent of their contribution to vascular dementia and parkinsonism.

KEYWORDS

Cerebral infarct, arteriolosclerosis, small-vessel disease, lacunar state, granular atrophy, état vermoulu, cerebral microinfarcts, Pierre Marie, Dejerine

Neurosciences and History 2020;8(3):87-98