“…i, The large-diameter axons are degenerating in the absence of myelin alteration. Scale bars, 2.5 m. ab, Autophagic body; go, Golgi; L, lysosome; lf, lipofuscin; mb, membrane; m, mitochondria; nu, nucleus; r, polyribosome; rer, rough endoplasmic reticulum; v, vacuole. disease, with rare deposits seen in the dorsal horn, brain stem, cerebellar granular layer, and cortex (data not shown), confirming that this is a late event in the pathological process (H. Seznec, D. Simon, L. Monassier, P. Criqui-Filipe, A. Gansmuller, P. Rustin, M. Koenig, and H. Puccio, unpublished observations), and is reminiscent of the situation in FRDA patients (Lamarche et al, 1993;Waldvogel et al, 1999;Bradley et al, 2000).…”