Clinical and electrophysiological data

SexAge at onsetAge at examAge at
abn walk
Abn swallowDeafnessARPMNCV m/sAmplitude mVNerve biopsy
DensityOB
I.1F436448+++Ulnar 52 0
I.2M346355+++
I.4M506060+++
I.7F407950?+?Ulnar 361
II.2F205249+++Median 340.30
II.5M3453++Median 443.5
III.1F1830+Median 57, 514
  • Exam=Examination; abn=abnormal; swallow=swallowing; ARP=Argyll Robertson-like pupils; MNCV=motor nerve conduction velocity; OB=onion bulb; 0=absence; ↘=lowered.

  • Normal values are: NCV >48 m/s; median nerve amplitude >5 mV; ulnar nerve amplitude >6 mV.