Camillo Golgi and the pathology of Huntington’s disease: An unresolved controversy

In 1874, Camillo Golgi published the neuropathological study of a patient with chronic chorea and dementia, demonstrating impairments in  the frontal-parietal and temporal cortices, striatum, and cerebellum. This study anticipated by fifty years those traditionally recognized as the first to link choreic movements with cortico-striatal involvement, but regardless of the author’s intention, has also come to be considered the first on the neuropathology of Huntington’s disease. This has given rise to a dispute between one position in favor of this diagnosis based on the clinical-anatomical correlation and the other opposed to the diagnosis, based on the lack of a family history of chorea. Given the absence of remains and slides to be used for DNA and histological analyses, we attempted to fill this gap by carrying out genealogical studies, but have so far been unable to identify any other choreic patients in the patient’s family or any association with homonymous families with Huntington’s disease currently living in the same region as Golgi’s patient. This does not end the controversy and indeed raises the issue of diagnosis, but it does not deprive Golgi of the merit of having first identified the neuropathology of chronic chorea.