Startle Epilepsy Triggered By Maternal Cough

An 18-month-old full-term boy with a history of mild neonatal hypoxic–ischemic injury, developmental delay, and prior generalized seizures (on levetiracetam) presented for characterization of paroxysmal jerking movements, typically in response to loud sounds. Differential diagnosis for these events included seizure, stereotypy, tic, hyperekplexia, and exaggerated startle in the setting of spasticity. cvEEG demonstrated ictal irregular generalized spike-wave and polyspike-wave complexes lasting for 1 to 2 seconds, clinically correlated with a sudden shock-like movement of the bilateral upper extremities ([Video 1]). In the video, these events are consistently triggered by his mother's habitual cough. Startle epilepsy is characterized by seizures that are triggered by sudden, unexpected auditory, tactile, and/or visual stimuli.[1] The patient develops a typical startle response, followed by complex but stereotyped epileptic motor and/or behavioral abnormalities. Treatment is often difficult, with variable responses to a broad spectrum of antiseizure medications.[2] In our patient's case, clobazam was initially added to the existing therapy with levetiracetam, with limited treatment response. Clobazam and levetiracetam were then replaced with topiramate monotherapy, leading to a significant reduction in seizures (2–3 per day from >20 per hour).

Video 1 Continuous video-EEG demonstrates ictal irregular generalized spike-wave and polyspike-wave complexes lasting for 1–2 seconds, clinically correlated with a sudden shock-like movement of the bilateral upper extremities. In the video, these events are triggered by the mother's habitual cough.

Publication History

Received: 14 February 2021

Accepted: 23 April 2021

Article published online:
30 June 2021

• References

• 1 Sáenz-Lope E, Herranz FJ, Masdeu JC. Startle epilepsy: a clinical study. Ann Neurol 1984; 16 (01) 78-81
• 2 Bhidayasiri R, Truong DD. Startle syndromes. Handb Clin Neurol 2011; 100: 421-430