The causes of progressive supranuclear paralysis (PSP) are still unknown. This pathology is said to be multifactorial with probably a genetic predisposition coupled with an environmental factor. It is not a hereditary disease and the genetic component is very possibly based on several genetic variants not identified to date. A toxic environmental origin is a hypothesis that has been investigated, such as exposure to arsenic or chromium in the textile industries, but has never been formally proven.
The causes of PSP
PSP is described as a taupathy because abnormal toxic accumulation of the abnormally phosphorylated TAU protein in neurons leads to their degeneration. Other brain cells such as astrocytes and oligodendrocytes, which protect and nourish neurons, are also affected.
The brain regions severely affected in all patients are the pallidum, the sub-thalamic nuclei, the black substance. Other regions may also be the site of neuronal death in some patients, the thalamus, the hyppocampus, and the frontal cortex.
At Paris Brain Institute
Dr. Isabelle LEBER, Neurologist, Coordinator of the Reference Centre for Progressive Supranuclear Paralysis of the Pitié-Salpêtrière Hospital and researcher in the team of Alexandra DURR and Giovanni STEVANIN is seeking to identify causes and better understand the mechanisms of the disease.