• Six affected members from a family of 15 patients with familial startle disease (hyperexplexia) underwent extensive electrophysiologic evaluation. The most marked abnormality consisted of prominent C response 60 to 75 ms after median and peroneal nerve stimulation. The somatosensory evoked responses were also relatively high in amplitude. These findings suggest that hyperactive long-loop reflexes may constitute the physiologic basis of startle disease.
Markand ON, Garg BP, Weaver DD. Familial Startle Disease (Hyperexplexia): Electrophysiologic Studies. Arch Neurol. 1984;41(1):71–74. doi:10.1001/archneur.1984.04050130077028
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