ATYPICAL CHILDHOOD EPILEPSY WITH CENTROTEMPORAL SPIKES

Mandatory seizures

Seizures with fronto-parietal opercular features are the initial seizure type, seen from sleep, with hemifacial (lip, mouth and tongue) clonic movements (which may be unilateral), laryngeal symptoms, articulation difficulty (aphasia), swallowing or chewing movements and hyper-salivation.

NOTE The terms fronto-parietal opercular, centrotemporal, sylvian and rolandic seizures are synonymous, referring to seizures involving the region around the central sulcus. However, in typical seizures that occur in this epilepsy syndrome, cognitive (e.g. gustatory hallucinations), emotional (e.g. fear), and autonomic features are not seen. This distinction may, however, be difficult to establish from history given by a young child.

Focal motor seizures with negative myoclonus subsequently develop. Negative myoclonus results in a brief loss of posture or position of one or both arms, resulting in dropping of objects held, nodding of the head, instability during standing, and inclining of the body to one side or falls. Consciousness is entirely preserved during the negative myoclonus. The transient dropping of one or both arms is best identified on asking the child to stand with their arms outstretched.

CAUTION Some patients may have persistent functional motor impairment, including mild dysarthria and ipsilateral facial and arm paresis, during the period of time that the negative myoclonus is frequent. These impairments disappear when the negative myoclonus remits.

CAUTION Negative myoclonus is also seen in other etiologies.

May have

• Atypical absence seizures in wakefulness
• Focal to bilateral tonic-clonic seizures

Exclusionary

Generalized tonic seizures in sleep.