JLE

Epileptic Disorders

MENU

Diaper changing-induced reflex seizures in CDKL5-related epilepsy Volume 20, numéro 5, October 2018

Figure 1

(A, B) Interictal EEG during sleep shows sporadic isolated spikes in the vertex region and both right and left centro-parietal regions, often related to single myoclonic jerks of the lower limbs (A) or upper limbs (B), asynchronous on the two sides. (C, D) Back-averaging of 30 different myoclonic events (trigger channel R-flex and L-flex, respectively): note that in both cases, there is evidence of agonist muscle co-contraction, followed by a contralateral myoclonic event (probably due to a propagation of the discharge); the myoclonic event is preceded by a positive transient discharge maximal on the central vertex and central region in the hemisphere contralateral to the earliest myoclonic potential. EMG abbreviations: R-delt: right deltoideus; R-ext: right extensor carpi; R-flex: right flexor carpi; R-quad: right quadriceps femoris; L-delt: left deltoideus; L-ext: left extensor carpi; L-flex: left flexor carpi; L-quad: left quadriceps femoris.

Figure 2

Ictal polygraphic recording during diaper changing (upper panel and lower panel are consecutive): the seizure starts with paroxysmal low-voltage rhythmic theta activity in the left centro-parietal and vertex regions (arrows), followed by generalized delta activity and a series of spasms, characterized by high-voltage slow complexes related to EMG periodic contractions of the four limbs (arrowheads). Note that the muscular contractions are characterized by an abrupt onset, which corresponds clinically to a “myoclonic” component (see video). The first spasms coincide with a tonic contraction lasting for 7-8 seconds (from the first arrowhead in the upper panel to the first three seconds of the lower panel). In the lower panel, between the second and third spasm, there is a sequence of focal spikes involving the vertex and left centro-parietal regions, each related to a myoclonic jerk of the right limbs (black asterisks). R-delt: right deltoideus; R-quad: right quadriceps femoris; L-delt: left deltoideus; L-quad: left quadriceps femoris.