In this study sample, at an assigned specificity of 90%, the sensitivity of the vibrotactile threshold for identifying distal
axonopathy was 86% (Gerr et al.
Amyotrophic lateral sclerosis is a distal
axonopathy: Evidence in mice and man.
ENMG results were recorded as acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor-sensory axonal neuropathy (AMSAN), or "unclassified" using specific criteria for demyelination and
axonopathy (8).
Tyl and Friedman (28) elucidated this abnormality and stated that AA binds motor proteins kinesin and dynein, thereby halting transaxonal transport and leading to distal
axonopathy.
(2004) Amyotrophic lateral sclerosis is a distal
axonopathy: evidence in mice and man.
When there was a significant reduction in motor amplitude,
axonopathy was deemed present.
The Cell and Molecular Biology of Glaucoma:
Axonopathy and the Brain.
Nerve biopsy in HSAN shows
axonopathy with loss of myelinated and unmyelinated nerve fibers of all diameters but with normal appearance of brain and spinal cord [2].
On EMG, there was no sensory function abnormality, but motor
axonopathy concerning for underlying neuromuscular disorder was detected.
Fernyhough, "Development of selective
axonopathy in adult sensory neurons isolated from diabetic rats: role of glucose-induced oxidative stress," Diabetes, vol.
Hensley, "Semaphorin 3A signaling through neuropilin-1 is an early trigger for distal
axonopathy in the SOD1G93A mouse model of amyotrophic lateral sclerosis," Journal of Neuropathology & Experimental Neurology, vol.
Pleasure, "Initiation and progression of
axonopathy in experimental autoimmune encephalomyelitis," The Journal of Neuroscience, vol.