Friedreich Ataxia: Hypoplasia of Spinal Cord and Dorsal Root Ganglia

Arnulf H. Koeppen MD, Alyssa B. Becker BA, Jiang Qian MD, PhD, Paul J. Feustel PhD. Journal of Neuropathology & Experimental Neurology, DOI:10.1093/jnen/nlw111, First published online: 12 January 2017

The FA patients included a wide range of disease onset and duration suggesting that the SC undergoes growth arrest early and remains abnormally small throughout life. The results strongly support the conclusion that these neural tissues are small because of hypoplasia rather than atrophy and that their developmental delay occurs independently.
It is of interest that Friedreich had already proposed in 1877 that smallness of the clavae (gracile and cuneate nuclei) was developmental.


Friedreich Ataxia: Hypoplasia of Spinal Cord and Dorsal Root Ganglia