Occipital spikes or spikes and waves generally present as an age-dependent, EEG-defined benign focal epilepsy64 manifesting in the younger child as autonomic symptoms followed by brief or prolonged partial motor seizures. Older children have visual symptoms and headache.

Occipital spikes are not always benign, however. Nass et al.65 reported 7 young children (5 from a consecutive series of 42) presenting clinically with autism or autistic regression and possible or definite seizures who had solely or predominantly occipital spikes or spikes and waves on the EEG. These children all exhibited severe language and behavior deficits.

Tenembaum et al.66 reported two teenagers who had carried the diagnosis of benign occipital epilepsy but who deteriorated cognitively and behaviorally when they developed continuous spikes and waves during slow sleep (CSWS) and persistent occipital paroxysms that extended anteriorly.

Beaumanoir67 suggests that the semiology of seizures with occipital spikes or spikes and waves depends on:

• the child’s age
• the maturation of the occipital cortex
• the cortex’s connection with other structures

Occipital maximal spikes can have fields extending to posterior temporal and parietal regions, both of which are involved in language acquisition and cognitive development. This effect could be most prominent in the young child, who is in the process of acquiring language.

Adapted from: Nass R and Gross A. Landau-Kleffner syndrome and its variants. In: Devinsky O and Westbrook LE, eds. Epilepsy and Developmental Disabilities. Boston: Butterworth-Heinemann; 2001;79–92.
With permission from Elsevier (www.elsevier.com).
Reviewed and revised May 2004 by Steven C. Schachter, MD, epilepsy.com Editorial Board.