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J Neurol Neurosurg Psychiatry 63:534-536 doi:10.1136/jnnp.63.4.534
  • Short report

Machado-Joseph disease presenting as severe asymmetric proximal neuropathy

Neurophysiological findings in the index patient

Motor Sensory F response
Dlt (ms) Amplitude (mV) NCV (m/s) Dlt (ms) Amplitude (μV) NCV (m/s)
Distal Proximal Distal Proximal
R median nerve 5.7 2.7 6.8 52 3.9 1.0 46 38.4-40.5
R ulnar nerve 3.2 18.3 13.6 39 5.3 16.2 9.8 49 29.3-29.8
R peroneal nerve 4.4 A A A
L peroneal nerve 3.5 A A A 9.9 A
R posterior tibial nerve 12.1 36.5
L posterior tibial nerve 3.8 0.5 0.8 38
  • Dlt = Distal latency time; NCV = nerve conduction velocity; A = absent.

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