The Presence of Electrographic Status Epilepticus in Sleep (ESES) in Children with Developmental Regression Does Not Always Indicate Landau-Kleffner Syndrome (LKS).
Abstract number :
1.161
Submission category :
Year :
2001
Submission ID :
3134
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
Y. Shafrir, MD, Pediatrics, Sinai Hospital, Baltimore, MD; B. Say, MD, H. A. Chapman Institute of Medical Genetics, University of Oklahoma Medical Research Center, Tulsa, OK
RATIONALE: Based on detailed study of 5 patients, and review of the existing literature, Hirsch et al. suggested in 1990 the association of LKS) and the EEG finding of ESES. However, the misconception that a child with developmental regression, mainly in langqauge, and ESES has LKS has been accepted by many. The potential for cure in LKS has lead many to perform EEG on patients with unexplained developmental regression.
METHODS: Analysis of the current literature and a case report.
RESULTS: An 11 year old boy was evaluated for developmental regression. He enjoyed excellent early psychomotor development until the age of 5. His deterioration was gradual. His articulation was the first noticeable sign of deterioration. He never became mute. He had difficulties understanding spoken language. Hearing examination was normal. His understanding of language was lost completely for 3 months and than improved. He developed gait apraxia and lost his ability to run. He was failing to thrive. MRI showed only minimal brain atrophy. Multiple biochemical and chromosomal tests were negative. At the age of 7, an EEG showed frequent spikes and spike and wave complex discharges in the right centrotemporal area that increased significantly during drowsiness and light sleep. 2 year later the EEG showed continuous, mostly generalized spikes and wave discharges during stage 2 sleep. He has never suffered a seizure. Several antiepileptic drugs were tried without benefit. He was than put on prednisone, which he took for 4 months. The dose was escalated from 20 mg to 80 mg a day (3.5 mg/kg). He became extremely Cushingoid and finally the parents decided to stop the treatment. Unfortunately, no EEG was performed at the end of the treatment. Preparation were made for the administration of intravenous immunoglobulins, when a routine eye exam revealed corneal clouding. An extensive genetic and metabolic evaluation which finally revealed GM-1 gangliosidosis.
The association of ESES with LKS, although dramatic and common, is not the rule. For example, Landau and Kleffner[scquote]s original patients did not have ESES. Asymptomatic patients with ESES has been reported. EEG findings similar to ESES can be found in a number of inborn errors of metabolism and degenerative diseases in childhood.
CONCLUSIONS: ESES is only an EEG pattern, and not a diagnosis. A thorough search for its cause should be undertaken. A more specific clinical picture of LKS and its many variants has to be defined, based on the large available body of data of both LKS and ESES, is urgently needed. Otherwise, diagnostic error similar to our case acn be expected.