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Table 1 Nerve conduction studies

From: Specific combination of compound heterozygous mutations in 17β-hydroxysteroid dehydrogenase type 4 (HSD17B4) defines a new subtype of D-bifunctional protein deficiency

  Normal Patient 1 Patient 2
   Age at study  
   12 years 13 years 16 years 13 ½ years
MOTOR:      
Median nerve      
 DML (wrist to APB) < 4.2   3.6 5.4 3.4
 CMAP (mV) ≥ 3.9   9.2 4.1 8.6
 CV (m/sec) > 47   27 27 33
Ulnar nerve      
 DML (msec; wrist to ADM) < 3.4 2.6 3.1 3.6 2.2
 CMAP (mV) ≥ 5.9 13.1 12.7 4.6 7.7
 CV (m/sec) > 47 25 27 23 24
Tibial nerve      
 DML (msec; ankle to AH) < 6.0 5.3 6.2 10.5 4.5
 CMAP (mV) ≥ 3.9 3.3 3.6 0.5 6.6
 CV (m/sec) > 39 21 22 15 26
Peroneal nerve      
 DML (msec; ankle to EDB) < 6.0 5.0   8.5 4.5
 CMAP (mV) ≥ 2.4 1.7   0.8 2.9
 CV (m/sec) > 39 22   17 43
SENSORY:      
Median nerve      
 PL (msec; wrist to digit-II) < 3.2 2.5 2.8 3.4 2.1
 SNAP (μV) ≥ 14 67 69 15 36
 CV (m/sec)   46 39 40 52
Ulnar nerve      
 PL (msec; wrist to digit-V) < 3.3 2.0 2.2 3.1 2.2
 SNAP (μV) ≥ 9 43 64 26 44
 CV (m/sec)   47 50 35 43
Sural nerve      
 PL (msec; calf to lat mall) < 4.2 3.8 3.0 NR 2.7
 SNAP (μV) ≥ 5 11 17 NR 9.5
 CV (m/sec)   42 40 NR 35
  1. Bold and underlined values are abnormal. All sensory responses are antidromic.
  2. Legend: DML=distal onset motor latency; CMAP=compound motor action potential; CV=conduction velocity; PL=peak onset latency; APB=abductor pollicus brevis; ADM=abductor digiti minimi; AH=abductor hallucis; EDB=extensor digitorum brevis; NR=no response.