Table 1

 Survey of abnormal reflex excitation and inhibition in patients with familial or acquired hyperekplexia or with stiff-man syndrome

StimulusFH/AH (n = 7)SMS (n = 10)
MAS, masseter; OOC, orbicularis oculi; SCM, sternocleidomastoideus muscles; TRA, trapezius; V1/V3, first/third trigeminal nerve branch.
Abnormal reflex excitation
Tap to chinMonosynaptic MAS reflex exaggerated0/72/10
Short-latency spread into TRA/SCM7/72/10
Pulse to V3Short-latency spread into TRA/SCM7/70/10
Tap to glabellaShort-latency spread into TRA/SCM7/74/10
Widespread reflex spasms0/75/10
Pulse to V1Short-latency spread into TRA/SCM7/70/10
AnyWidespread reflex spasms0/75/10
Abnormal reflex inhibition
Tap to chinLoss of reflex inhibition in TRA/SCM1/73/10
MAS post reflex silent period incomplete/delayed2/73/10
Pulse to V3Loss or delay of S2 component of MAS inhibition2/71/10
Elevated reflex thresholds for MAS inhibition0/77/10
Tap to glabellaLoss of OOC inhibition0/72/10