Acquired Epileptiform Opercular Syndrome: 3 New Cases with Central-Midtemporal Spikes and ESES, and Clinical Responses to Methylprednisolone and the Ketogenic Diet.
Abstract number :
1.158
Submission category :
Year :
2001
Submission ID :
3078
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
C.M. Roberts, MD, Neurology, Children[ssquote]s Hospital of Philadelphia, Philadelphia, PA; C.A.G. Bergqvist, MD, Neurology, Children[ssquote]s Hospital of Philadelphia, Philadelphia, PA; P.H. Berman, MD, Neurology, Children[ssquote]s Hospital of Philadel
RATIONALE: Acquired epileptiform opercular syndrome (AEOS) is a rare epileptic disorder characterized by severe oral motor apraxia with impairment of speech production, dysarthria, drooling, and dysphagia, associated with an abnormal EEG dominated by epileptiform discharges from one or both central-midtemporal regions. Clinical features follow a waxing and waning course, often worsening in association with the appearance of electrical status epilepticus during slow wave sleep (ESES). Mixed seizure types can occur, including complex absences, complex partial, and rare tonic-clonic seizures. Central nervous system imaging is normal, without evidence of perisylvian developmental or acquired defects. AEOS is distinct from the Landau-Kleffner syndrome which produces a verbal auditory agnosia, loss of receptive and non-motor expressive language skills.
Only 2 patients reported in the literature with symptoms consistent with AEOS had documented central-midtemporal ESES. We describe 3 patients age 2.5 to 4 years with EEG and clinical courses consistent with AEOS. All showed ESES with a central-midtemporal focus on 24-hour EEG monitoring. Treatment responses to methylprednisolone, anticonvulsants, and the ketogenic diet are outlined.
METHODS: 3 boys (2.5y-4y) with mixed seizure disorders developed oral apraxia, dysarthria, drooling, and dysphagia. All received MRI studies and 24 hour video EEG monitoring. Two received formal neuropsychological and speech pathology evaluations, one was evaluated at the bedside.
RESULTS: Brain MRIs were normal. All had prominent central-midtemporal spike discharges on waking EEG and in more than 85% of slow wave sleep, consistent with ESES. One patient responded promptly to intravenous methylprednisolone, with documented cessation of ESES over 72 hours of treatment. One patient responded initially to oral anticonvulsants, but presented with recurrent oral apraxia and ESES. One patient responded completely to the ketogenic diet after failing standard anticonvulsants.
CONCLUSIONS: Children with loss of language, with or without associated epilepsy, should be evaluated for symptoms of an acquired opercular syndrome, with oral motor apraxia, dysarthria, dysphagia, and drooling. AOES patients will not have receptive or expressive aphasias characteristic of the Landau-Kleffner Syndrome. 24-hour EEG monitoring should be obtained to identify central-midtemporal epileptiform discharges and ESES. Clinical symptoms may be refractory to standard anticonvulsant therapy but may respond well to steroids or the ketogenic diet. Relapses may be associated with the re-appearance of ESES.