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Article
January 1984

Familial Startle Disease (Hyperexplexia): Electrophysiologic Studies

Author Affiliations

From the Departments of Neurology (Drs Markand and Garg) and Medical Genetics (Dr Weaver), Indiana University School of Medicine, Indianapolis.

Arch Neurol. 1984;41(1):71-74. doi:10.1001/archneur.1984.04050130077028
Abstract

• 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.

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