Fig. 2

Neuropathologic findings. a Macroscopic appearance of the optic nerves. Atrophy is evident. b Depletion of myelinated fibers and c loss and occasional swelling of axons in the optic nerve. Klüver-Barrera staining (KB) and phosphorylated neurofilament (pNF) immunohistochemistry. d Moderate neuronal loss in the lateral geniculate body (LGB) and e large neurons in layer IV of the striate cortex. KB staining. f Atrophy and myelin pallor of the lateral corticospinal and dorsal spinocerebellar tracts (arrows), and dorsal columns (arrowheads). KB staining. g Loss of ganglion cells with a Nageotte nodule (arrowheads) in the dorsal root ganglion of the lumbar spinal cord. HE staining. h Severe neuronal loss in Clarke’s nucleus and i the anterior horn. KB staining. j Neurogenic change with small grouped atrophy is evident in the iliopsoas muscle k–m Coronal sections of the k anterior and l posterior nerve roots of the lumbar cord and m sural nerve. Severe loss of large and small myelinated fibers and thinning myelin sheath of residual fibers. Toluidine blue staining. Ctrl, control; Pt, patient. Bar in a = 5 mm in a; 1.3 mm in f; 200 μm in d, i; 160 μm in h, j; 100 μm in e, g; 50 μm in b, c, j; 30 μm in k, l, m