Pain medicine (Malden, Mass.) · 2008
Detection of a characteristic painful neuropathy in Fabry disease: a pilot study.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Maag Rainer, Binder Andreas, Maier Christoph, Scherens Andrea, Toelle Thomas, Treede Rolf-Detlef, Baron Ralf
Аннотация
OBJECTIVE: Fabry disease (FD) is an X-linked lipid storage disorder showing a high prevalence and early occurrence of painful neuropathy. Early detection of this likely underdiagnosed disease is an important approach because a causal therapy is available.
DESIGN: We used a quantitative sensory testing to determine the detailed somatosensory profile of male Fabry patients and compare this profile with somatosensory profiles of other painful sensory neuropathies (SN).
RESULTS: Within this pilot-study, the profile revealed a small-fiber sensory neuropathy selectively affecting C- and A-delta fibers. The comparison with different somatosensory profiles of painful SN, including painful small-fiber sensory neuropathies of other etiologies, showed that the FD profile differs significantly and is characterized by a severe impairment of thermal and preserved vibratory and mechanical discrimination.
CONCLUSION: Thus, somatosensory profiling in male patients with painful extremities may be useful in the detection of FD.
DESIGN: We used a quantitative sensory testing to determine the detailed somatosensory profile of male Fabry patients and compare this profile with somatosensory profiles of other painful sensory neuropathies (SN).
RESULTS: Within this pilot-study, the profile revealed a small-fiber sensory neuropathy selectively affecting C- and A-delta fibers. The comparison with different somatosensory profiles of painful SN, including painful small-fiber sensory neuropathies of other etiologies, showed that the FD profile differs significantly and is characterized by a severe impairment of thermal and preserved vibratory and mechanical discrimination.
CONCLUSION: Thus, somatosensory profiling in male patients with painful extremities may be useful in the detection of FD.