Acquired Epileptiform Opercular Syndrome: A Second Case Report, Review of the Literature, and Comparison to the Landau‐Kleffner Syndrome

Abstract

A 5-year-old girl developed recurrent prolonged episodes of severe oral apraxia, dysarthria, and drooling, similar to the opercular syndrome in children. Each episode lasted several weeks to > 6 months and was associated with exacerbation of epileptiform activity in her EEG. Electrographic status epilepticus during slow wave sleep (ESES) was recorded during three of the exacerbations. The EEG improved markedly when clinical symptoms subsided. Antiepileptic drugs (AEDs) were not effective, although there was some improvement when they were combined with a ketogenic diet. A similar case was described by Roulet et al. We believe that this is a distinct epileptic syndrome, equivalent to the Landau-Kleffner syndrome (LKS).

Complex partial seizures and aphasia as initial manifestations of non-ketotic hyperglycemia: case report

“…It is very well established that aphasia can be the initial, only and sometimes progressive manifestation of epilepsy and focal SE 2,[11][12][13][14] . Other focal signs, like amaurosis, disartria, speech arrest may manifest localized seizures [15][16][17] .…”

Acquired childhood dysarthria: Review of its clinical presentation

“…Shafrir and Prensky [40] observed opercular syndrome of epileptic origin in a child with recurrent prolonged episodes of severe oral apraxia, dysarthria, and drooling. During these episodes, there was an increase in right temporal spikes and appearance of generalized spikes.…”

“…Remissions were associated with improvement evident in the electroencephalogram. which either showed right temporal focus or was 1'rec of cpilcptiform activity [40].…”

Clinical Reasoning: An 11-year-old boy with language disorder and epilepsy

“…Acquired epileptiform opercular syndrome: it was first proposed by Shafrir and Prensky. 6 When strong, persistent epileptic discharge spreads to bilateral opercular cortices, interfering with the cortical function in perisylvian regions, seizure patients can develop FCMS symptoms, which will disappear with EEG improvement. 6 However, the operculum syndrome in the present case is supposed to be irrelevant to epileptic discharge or seizure episode.…”

“…6 When strong, persistent epileptic discharge spreads to bilateral opercular cortices, interfering with the cortical function in perisylvian regions, seizure patients can develop FCMS symptoms, which will disappear with EEG improvement. 6 However, the operculum syndrome in the present case is supposed to be irrelevant to epileptic discharge or seizure episode. The explanations are as follows: the paralysis of orofacial muscles precedes seizure; the symptoms are persistent, do not fluctuate, and are not relieved with seizure remission; and no epileptic discharge has been detected in the symptoms.…”