What is Pierre Marie's Hereditary Cerebellar Ataxia

Pierre Marie cerebellar ataxia is a hereditary degenerative condition with a predominant lesion of the cerebellum (during histopathological examination, atrophy of cells of the cortex, nuclei of the cerebellum and pathways: spinocerebellar and pyramidal, is determined). The condition occurs in several generations in a row. It has an autosomal dominant type of inheritance. Disease incidence is 0.5 for 100,000 people. The disease manifests at a young or middle age (20–40 years), the course is slowly progressive.

Clinical differentiation of spinocerebellar ataxias is practically impossible, nosological affiliation is established, mainly, during the pathomorphological examination of all structures of the brain and spinal cord. Therefore, most authors consider Pierre Marie cerebellar atrophy as a separate nosological form. This syndrome with a similar clinical picture includes: olivopontocerebellar inherited atrophy (Menzel type), sporadic (Dejerine Thomas) oliocerebellar atrophy (Holmes type), late cortical cerebellar atrophy of Marie-Foix-Alajuanin, olivo-rubro-cerebellar atrophy of Lejonne-Lhermitte. There are also about 42 genetic types of autosomal dominant spinocerebellar ataxias.