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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).
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BMC Neurology

ISSN: 1471-2377

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