Table 2

Electrophysiological findings in patients with SCA6

Normal valuesFamily - patient
D-1E-1F-1E-2H-1A-2K-2L-2M-1N-1
Peroneal nerve DL (ms)< 4.74.1 5.2 4.33.74.0 5.0 6.2 3.83.53.4
CMAP (mV)> 5.07.6 4.0 6.07.16.1 1.5 2.9 6.4 4.7 5.8
MNCV (m/s)> 42.052.744.245.949.049.845.8 38.8 52.647.844.8
F wave (ms)< 56.040.3 60.0 50.344.247.853.9 56.6 43.148.953.9
Tibial nerve DL (ms)< 5.23.8 5.7 3.33.03.8 5.5 4.03.73.94.2
CMAP (mV)> 10.013.010.021.713.015.912.3 7.5 23.110.815.1
MNCV (m/s)> 42.048.5 39.6 48.242.749.349.4 40.2 49.150.248.7
F wave (ms)< 56.044.0 61.0 49.446.249.4 57.3 57.6 44.953.1 57.4
Sural nerve SNAP (μV)> 10.012.017.021.021.0 7.5 6.7 4.3 23.0 6.3 8.2
SNCV (m/s)> 47.056.0 38.0 47.355.151.8 44.0 50.059.6 46.4 53.4
EMG (TA) PSA00000+00000
Neurogenic00+00+++0+0
MEP PMCT (FDI) (ms)< 16.515.215.214.115.2 16.8
CMCT (FDI) (ms)< 8.56.37.48.28.25.5
PMCT (TA) (ms)< 16.214.114.114.814.117.1
CMCT (TA) (ms)< 18.518.015.613.3 20.0 14.8
  • Symbols for patients as in table 1. DL=distal latency; MNCV=motor nerve conduction velocity; CMAP=compound muscle action potential; SNAP=sensory nerve action potential; SNCV=sensory nerve action potential; 0=normal; +=mild neurogenic changes; Abnormal values in italics. MEP=Motor evoked potentials; FDI=first dorsal interosseus muscle; TA=tibialis anterior muscle; PMCT=peripheral motor conduction time; CMCT=central motor conduction time.