Survey of abnormal reflex excitation and inhibition in patients with familial or acquired hyperekplexia or with stiff-man syndrome
Stimulus | FH/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 chin | Monosynaptic MAS reflex exaggerated | 0/7 | 2/10 |
Short-latency spread into TRA/SCM | 7/7 | 2/10 | |
Pulse to V3 | Short-latency spread into TRA/SCM | 7/7 | 0/10 |
Tap to glabella | Short-latency spread into TRA/SCM | 7/7 | 4/10 |
Widespread reflex spasms | 0/7 | 5/10 | |
Pulse to V1 | Short-latency spread into TRA/SCM | 7/7 | 0/10 |
Any | Widespread reflex spasms | 0/7 | 5/10 |
Abnormal reflex inhibition | |||
Tap to chin | Loss of reflex inhibition in TRA/SCM | 1/7 | 3/10 |
MAS post reflex silent period incomplete/delayed | 2/7 | 3/10 | |
Pulse to V3 | Loss or delay of S2 component of MAS inhibition | 2/7 | 1/10 |
Elevated reflex thresholds for MAS inhibition | 0/7 | 7/10 | |
Tap to glabella | Loss of OOC inhibition | 0/7 | 2/10 |