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Table 4 Autonomic findings

From: Neuromuscular abnormality and autonomic dysfunction in patients with cerebrotendinous xanthomatosis

   I-1 I-2 II-1 II-2
RRIV (%) (N > 15)   3.53 15.7 24.2 25.4
Head-up tilt test   NR NR NR NR
   BR sensitivity (msec/mmHg)   -1.9 -1.4 2.3 0.7
30:15 ratio (N > 1.03)   1.03 0.95 1.11 1.37
SSR      
   onset latency (ms) R palm 1320 1700 1440 1720
  L palm 1400 1700 1400 1770
  R sole 2300 2280 2060 2280
  L sole 2290 2280 2250 2280
   amplitude (μV) R palm 2652 1569 978 1314
  L palm 2198 921 869 1099
  R sole 315 159 61 688
  L sole 399 239 74 562
Q-Sweat test      
   onset latency (seconds) Forearm 137 219 185 275
  Proximal leg 217 194 165 128
  Distal leg 165 148 168 136
  Foot 165 203 189 122
   total volume (μl) Forearm 0.150 0.298 0.015 0.450
  Proximal leg 0.134 0.063 0.199 1.283
  Distal leg 0.183 0.108 0.294 1.409
  Foot 0.029 0.095 0.030 0.700
   skin temperature (°C) Forearm 36.5 36.4 36.5 35.4
  Proximal leg 35 35.8 36.4 35.7
  Distal leg 35.6 36.3 34.9 35.3
  Foot 35.7 35.2 34.7 35.4
   room temperature (°C)   23 21 21 21
   room humidity (%)   56 57 58 58
  1. RRIV: R-R interval variation; NR: normal blood pressure (BP) and heart rate (HR) response in head-up tilt test: no abnormal change in BP and HR; N: normal; SSR: sympathetic skin response; Q-Sweat: quantitative sweat measurement.
  2. In Q-Sweat test, the total sweat volume (median (min, max) for female normal control were 0.495 (0.02, 3.814) (forearm), 0.505 (0.06, 4.49) (proximal leg), 0.389 (0.015, 4.914) (distal leg) and 0.203 (0.014, 0.802) (foot), and for male normal control were 1.079 (0.02, 3.814) (forearm), 1.091 (0.029, 2.734) (proximal leg), 1.107 (0.043, 2.902) (distal leg) and 0.585 (0.041, 1.718) (foot).