1. Hereditary sensory and autonomic neuropathy type 1
Hereditary sensory and autonomic neuropathy type 1. A 1-μm stained resin section (A) and electron micrograph (B) of a sural nerve biopsy. The single myelinated fiber in (A) (arrow) is visible at the top of (B); many more unmyelinated axons (arrows) remain than myelinated axons. Scale bar = 10 μm (A) and 5 μm (B). A 1-μm stained resin section (C) and teased fiber preparation of the sural nerve biopsy from another patient with HSNA1 (D and E). There are around 50 myelinated fibers in the whole biopsy. At least two of them appear to have internodes that seem to be demyelinating in a segmental pattern (between arrows D and E). Scale bar = 10 μm (C) and 20 μm (D and E). (Reproduced with permission from Houlden H, King R, Blake J, et al. Clinical, pathological and genetic characterization of hereditary sensory and autonomic neuropathy type 1 (HSAN I). Brain. 2006;129(Pt 2):411–425.)
Source: Charcot–Marie–Tooth Disease and Related Disorders, Neuromuscular Disorders, 2e
Citation: Amato AA, Russell JA. Neuromuscular Disorders, 2e; 2015 Available at: Accessed: November 02, 2017
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