ESES Syndrome with Acquired Aphasia

Abstract

Case Report: A 7‐year‐old boy presented with a 2‐year history of convulsions. Birth history was unremarkable. His motor and mental development were normal until the onset of partial seizures of his face at the age of 5.3 years, usually upon awakening. CBZ failed to control seizures. At 6.3 years he began to display slow speech, drooling, and reading difficulties. Absence attacks began at age 7. Upon examination, muscle weakness and cerebellar ataxia and paresis were absent. He showed sudden loss of tone in the arms when eating or writing, considered to represent negative myoclonus. Laboratory examination including CBC, blood chemistry, CSF studies, and MRI all were normal. EEG showed continuous diffuse spike and wave bursts associated with focal spikes at mid‐temporal and central area in the sleep record. Overnight sleep EEG showed continuous diffuse spike and wave bursts during slow wave sleep. The spike wave index was about 30% among slow wave sleep stages. During REM sleep, rapid eye movements disturbed the discharges of the diffuse spike and wave complex on EEG. His intelligent quotient was about 73 in the Tanaka‐Binnet test. Attention was limited and he could not copy figures accurately due to pour eye‐hand coordination. Speech therapy evaluation found word finding difficulty, paraphasia, slow speech, misuse of different consonants, distortion of vowels, and motor articulatory disturbance or motor aphasia. He lacked oral dyspraxia. During a hospital admission, ACTH‐2 was administered intramuscularly 2 I times in addition to oral administration of valproate, phenytoin, and carbamazepine. Convulsions were controlled, and he gradually recovered from the motor aphasia. Diffuse abnormalities of EEG resolved, but focal spikes remained over centro‐temporal areas during sleep. Cognitive function and vocabulary improved. Negative myoclonus of his hand disappeared. Several months after ACTH therapy, however, he suffered from moderate difficulties with word finding and the EEG deteriorated. Conclusions: ESES syndrome consists of “electrical status epilepticus during sleep”, “nonconvulsive status epilepticus with epileptic negative myoclonus”, “atypical benign partial epilepsy”, and “LandauKleffner syndrome“. According to Ohtahara, Landau‐Kleffner syndrome is characteristic of auditory agnosia. There have been no reports of motor aphasia associated with this syndrome. The present case does not meet criteria for “electrical status epilepticus during sleep” because of the low spike‐wave index but may be considered a variant of ESES. The distinctive features of this case are motor aphasia and negative myoclonus, as well as diffuse spike‐waves during slow wave sleep. Long‐term prognosis of this patient is not presently known.