PURPOSE OF THE REVIEW: Secondary axonal atrophy is common in most if not all demyelinating neuropathies and is likely responsible for the majority of clinical symptoms. We review clinical, electrophysiological and morphological evidence for secondary axonal atrophy in demyelinating neuropathies and summarize recent hypotheses on possible pathomechanisms. RECENT FINDINGS: Elucidation of genetic defects responsible for hereditary demyelinating neuropathies and insights into axon-Schwann cell interactions have allowed longitudinal studies of genetically defined demyelinating neuropathies and research into the pathomechanism of secondary axonal atrophy. SUMMARY: There is ample clinical electrophysiological and electropathological evidence that secondary axonal atrophy is found in hereditary and demyelinating neuropathies. Recognizing secondary axonal atrophy as a main cause for clinical disability in demyelinating neuropathies is important for the clinician and may reveal a therapeutic target common to all different forms of demyelinating neuropathies.
PURPOSE OF THE REVIEW: Secondary axonal atrophy is common in most if not all demyelinating neuropathies and is likely responsible for the majority of clinical symptoms. We review clinical, electrophysiological and morphological evidence for secondary axonal atrophy in demyelinating neuropathies and summarize recent hypotheses on possible pathomechanisms. RECENT FINDINGS: Elucidation of genetic defects responsible for hereditary demyelinating neuropathies and insights into axon-Schwann cell interactions have allowed longitudinal studies of genetically defined demyelinating neuropathies and research into the pathomechanism of secondary axonal atrophy. SUMMARY: There is ample clinical electrophysiological and electropathological evidence that secondary axonal atrophy is found in hereditary and demyelinating neuropathies. Recognizing secondary axonal atrophy as a main cause for clinical disability in demyelinating neuropathies is important for the clinician and may reveal a therapeutic target common to all different forms of demyelinating neuropathies.