EPILEPSY WITH READING-INDUCED SEIZURES (EwRIS)

OVERVIEW

Epilepsy with reading-induced seizures (EwRIS) is a rare combined generalized and focal epilepsy syndrome, characterized by reflex myoclonic seizures affecting orofacial muscles triggered by reading. If reading continues, these may worsen, and a generalized tonic–clonic seizure may occur. Good history-taking is therefore critical for diagnosis, as is awareness of this syndrome, as the task-specific eliciting of symptoms can result in misdiagnosis of seizures as non-epileptic seizures, as tics, or as stuttering. Seizures are elicited mainly by reading, but also by other tasks related to language. Prognosis is favorable, as spontaneous seizures are not expected, and seizures are responsive to treatment and can be avoided through reducing exposure to the triggering stimulus. In most patients, seizures require long-term treatment, although some patients may experience remission in time.

NOTE A reflex seizure is a seizure that is consistently or nearly consistently elicited by a specific stimulus, which may be sensory, sensory–motor, or cognitive. The stimulus may "elementary" (e.g., light, elimination of visual fixation, touch), "complex" (e.g., tooth-brushing, eating), or cognitive (e.g., reading, calculating, thinking, listening to music). Such a stimulus will have a high likelihood of eliciting a seizure, in contrast to a stimulus that may facilitate epileptiform abnormality (such as photoparoxysmal responses on EEG) or evoke a seizure, but not consistently.

Clinical context

Age at onset is typically in the late teens (median = 17.5 years, range = 10–46 years). A male sex predominance (~2:1) is recognized. Antecedent, birth, and neonatal history is typically normal. Development and cognition are typically normal. Neurological examination is normal. Prognosis is generally considered to be favorable, with a drug-responsive seizures and potential for remission in some patients with age. Reducing exposure to the triggering stimulus may be successful in reducing seizures; however, limiting reading can result in significant restrictions in capacity for education, employment, lifestyle, and even for religious practice.