Saturday, May 5, 2012

Friedreich's Ataxia

The Friedreich's ataxia is a hereditary, familial, and recessive syndrome which is anatomically characterized by degeneration of the posterior and spinocerebellar tracts of the spinal cord, causing lack of coordination of muscular movement, specially of limbs, when walking, which is called ataxia; other symptons are abolition of reflexes, dysarthria (speech problems), scoliosis, nistagmus, etc. Affecting more frecuently males than females, Friedreich's ataxia starts in early childhood and is chronic and progressive.

Pathological anatomy

Macroscopically, the spinal cord appears to have shrunk in size as the cerebelum shows slight signs of atrophy. Microscopically, there is proliferation of glial cells and degeneration of the spinal cord posterior and lateral tracts (spinocerebellar tracts), especially the direct and Gowers' tracts. The degenerative foci of these tracts impinges upon the pyramidal tract. The Clarke column ganglionar cells get also destroyed secondarily as well as the posterior roots.


There is no effective treament.