Pathology and functional diagnosis of small-fiber painful neuropathy
Journal
Acta Neurologica Taiwanica
Journal Volume
19
Journal Issue
2
Pages
82-89
Date Issued
2010
Author(s)
Abstract
Small-fiber sensory neuropathy with neuropathic pain had been a diagnostic challenge for neurologists. We and several groups have developed skin biopsy with quantitation of intraepidermal nerve fiber (IENF) density as a diagnostic approach. In the skin with small-fiber sensory neuropathy, there are pathological hallmarks: reduced IENF density with degeneration of subepidermal nerve plexuses and dermal nerves. Skin denervation is a major presentation of diabetic neuropathy and inflammatory neuropathies including Guillain-Barr? syndrome and chronic inflammatory demyelinating polyneuropathy. The skin biopsy approach also provides an opportunity to examine dermal vasculature and inflammatory vasculopathy is demonstrated in vasculitic neuropathy, systemic lupus erythematosus, and eosinophilia-associated neuropathy. In addition to neuropahtologic evidence, the functional consequences of cutaneous nerve degeneration can be assessed with quantitative sensory testing (QST), contact heat evoked potential (CHEP), and functional magnetic resonance imaging (fMRI). One major etiology of small-fiber sensory neuropathy is familial amyloid polyneuropathy caused by mutations of transthyretin (TTR). We recently conducted studies on a large cohort of unique TTR mutation on Ala97Ser in Taiwan. These patients had significant skin denervation in addition to motor and autonomic neuropathy. Taken together, the skin biopsy with quantitation of IENF density provides diagnostic utility for small-fiber sensory neuropathy and the combination of psychophysical, physiological, and neuroimaging examinations offer comprehensive assessments for patients with neuropathic pain due o cutaneous nerve degeneration.
Subjects
Amyloid neuropathy; Contact heat evoked potential; Functional magnetic resonance imaging; Skin biopsy; Transthyretin
SDGs
Other Subjects
prealbumin; autonomic neuropathy; chronic inflammatory demyelinating polyneuropathy; denervation; dysautonomia; evoked response; familial amyloid polyneuropathy; functional assessment; Guillain Barre syndrome; human; motor neuropathy; neuroimaging; neuropathic pain; neuropathy; non insulin dependent diabetes mellitus; pathology; physiological process; psychophysiology; review; sensory system examination; skin biopsy; skin denervation; small fiber painful neuropathy; Taiwan; Amyloid Neuropathies, Familial; Humans; Mutation; Pain; Peripheral Nervous System Diseases; Prealbumin; Sensory Receptor Cells
Type
review