XXXX variants in @entity6 : measuring treatment response with a small nerve fiber test.

@entity1 @entity2175 @entity1183 @entity6 @entity500 @entity73 @entity1109 @entity2646 @entity158 @entity258
INTRODUCTION: @entity2646 ( @entity2646 ) is eleven times more common among @entity1 with @entity6 than the general population and is treatable with appropriate immunotherapy. Treatment response is usually measured clinically (symptomatic and functional improvement). We present a case of a @entity1 with @entity2175 ( @entity2175 ) and @entity2646 whose treatment response was measurable with the Sudoscan sudomotor function test. This test may represent a new objective evaluation of the treatment of @entity2646 . CASE DESCRIPTION: The @entity1 is a 60year old male initially referred to our center in August 2012, at which time he was diagnosed with @entity2646 based on AAN electrodiagnostic criteria (NCS). Autonomic functions were significant for @entity258 variability response to expiration/inspiration (E/I), Valsalva maneuver and the ratio of the RR interval for the 30th to the 15th beat upon standing (1.08, 1.12, 1.05 respectively), and frequency analysis of the total spectral power, the standard deviation of the normal RR intervals (sdNN) and their root mean squared (rmsSD). Sudoscan electrochemical skin conductances (ESC), measuring small nerve fiber function on the palms and soles, were very low: 23  S in the feet and 32  S in the hands. After one cycle of intravenous immunoglobulin (IVIG: 6 doses total, 75g each) the @entity1 had no change in symptoms of burning, @entity1183 , @entity158 , and @entity73 . However, E/I, Valsalva, and 30:15 ratios (1.19, 1.36, 1.39 respectively) were improved, as were NCS. Sudoscan scores for feet and hands were unchanged (23  S and 32  S). In March 2013, the @entity1 's autonomic functions worsened (E/I, Valsalva, and 30:15 ratios 1.1, 1.07, 1.12 respectively), but feet and hand ESC started to show improvement (35  S and 52  S respectively). @entity500 was started. Eight days after a second cycle of IVIG in January 2014, the @entity1 reported for the first time less burning, @entity158 and tingling. E/I, Valsalva, and 30:15 ratios remained low (1.03, 1.07, and not analyzable, respectively), while foot and hand ESC scores continued to improve (43  S and 55  S respectively). DISCUSSION: @entity2646 diagnosis and treatment response are difficult in the @entity6 @entity1 . We found that NCS and autonomic function tests did not correlate well with clinical status while numerical Sudoscan scores matched closely symptomatic changes. ESC have been found to correlate well with peripheral small fiber function and @entity1109 in DPN. The findings in this @entity1 warrant further investigation of the use of Sudoscan to monitor @entity2646 response to therapy.
