Functional assessment of Aδ and C fibers in patients with Fabry's disease

✍ Scribed by Massimiliano Valeriani; Paolo Mariotti; Domenica Le Pera; Domenico Restuccia; Liala De Armas; Toni Maiese; Federico Vigevano; Daniela Antuzzi; Giuseppe Zampino; Roberta Ricci; Pietro Tonali

• Publisher: John Wiley and Sons
• Year: 2004
• Tongue: English
• Weight: 187 KB
• Volume: 30
• Category: Article
• ISSN: 0148-639X
• DOI: 10.1002/mus.20174

✦ Synopsis

Abstract

The pathophysiology of neuropathic pain in Fabry's disease (FD) is still largely unknown. Seven FD patients were studied by laser evoked potentials (LEPs) to assess the function of the Aδ and C fibers. Laser pulses were delivered on the skin of the hand and perioral region at painful intensity to record LEPs related to Aδ‐fiber inputs and at nonpainful intensity to obtain LEPs related to C‐fiber inputs. When the perioral region was stimulated, a vertex positive component was recorded with a mean latency of 260.3 ms and 376 ms after Aδ‐ and C‐fiber stimulation, respectively. The mean Aδ‐LEP amplitude was significantly lower in FD patients (N1/P1 mean values were 2.8 μV and 4.5 μV after hand and face stimulation, respectively, compared to 4 μV and 8.9 μV for controls; N2/P2 mean values were 8.2 μV and 11.1 μV after hand and face stimulation, respectively, and 16.7 μV and 22.3 μV in controls). Unlike the healthy subjects, 6 FD patients, suffering from neuropathic pain, showed a late positive potential related to C‐fiber function (mean latency, 377.1 ms) also after facial stimulation at painful intensity, suggesting a relative overflow of C‐fiber input, which may be relevant in the pathophysiology of pain in this disease. Muscle Nerve, 2004