Elsevier

Pediatric Neurology

Volume 27, Issue 5, November 2002, Pages 388-391
Pediatric Neurology

Original article
Successful treatment of Ohtahara syndrome with chloral hydrate

https://doi.org/10.1016/S0887-8994(02)00464-2Get rights and content

Abstract

We present a patient with early infantile epileptic encephalopathy with suppression bursts (Ohtahara syndrome) with an excellent response to chloral hydrate to draw attention to a possible role of the “old” drug in the treatment of intractable epilepsy. Chloral hydrate (58 mg/kg/day) was used for a short-term administration in a 5-week-old female with typical features of cryptogenic Ohtahara syndrome after the failure of conventional antiepileptic drugs. Seizures disappeared in the course of 24 hours after the launch of chloral hydrate therapy and have not recurred. Results of electroencephalogram studies of the child demonstrate marked improvement. Psychomotor development is significantly delayed. Detailed diagnostic tests have not revealed any metabolic or structural abnormalities of the brain. We conclude that chloral hydrate could be useful in the treatment of severe epileptic encephalopathies. Possible indications of the drug for intractable epilepsy treatment are discussed.

Introduction

Early infantile epileptic encephalopathy (Ohtahara syndrome) was first described in 1976 [1] as the earliest form of the age-dependent epileptic encephalopathies. In 1992, Ohtahara et al. [2] summarized the following characteristics of the syndrome: (1) onset usually during the first week of life, (2) main seizure pattern is brief tonic seizures, (3) suppression bursts in electroencephalogram, (4) severe psychomotor retardation, (5) intractability of the seizures, (6) poor prognosis, (7) polyetiology, and (8) frequent progression to West syndrome. Obvious brain lesions, most frequently malformations of cortical development, are usually evident [3]; however, a few cryptogenic cases have also been reported [2], [4], [5].

No effective antiepileptic treatment of the syndrome has been proposed to date. In general, antiepileptic drugs may help to reduce the seizure frequency, although they are of little aid in halting the deterioration of psychomotor development. Adrenocorticotropic hormone therapy, the treatment of choice in West syndrome, proved to be ineffective [4]. One isolated case of early infantile epileptic encephalopathy profited from zonisamide administration [5]. Selected patients could benefit from epilepsy surgery [6], [7]. Despite different and often vigorous treatment strategies, all patients are severely handicapped in the final follow-up. Many of them die, especially in the early stage of the disease, and all survivors have severe mental retardation [8].

We report a uniquely successful chloral hydrate treatment of a patient with cryptogenic early infantile epileptic encephalopathy.

Section snippets

Case report

A 3-week-old female infant was admitted to our department for treatment of intractable neonatal seizures. There was no family history of neurologic disorders, and her older half-brother and half-sister were healthy. The pregnancy was normal, delivery was at the fortieth week, birth weight was 3,380 gm, birth length was 51 cm, and Apgar score was 9-10 of 10. The infant was considered healthy. On the fourth day of life, hypoglycemia of 1.4 mmol/L was detected. Several hours later, after normal

Discussion

Introduced clinically in 1869, chloral hydrate is one of the oldest hypnotic drugs. It is widely used for inducing light sedation during some diagnostic procedures, such as electroencephalogram, neuroimaging, or lumbar puncture [9]. The literature on its use for epilepsy is sparse, although the efficacy of rectal chloral hydrate in the treatment of either febrile or epileptic childhood seizures has been reported [10], [11]. A few older studies reported good results with chloral hydrate in the

Acknowledgements

This research was supported by the Research Project of the Czech Ministry of Health No. 00000064203 and Research Projects of the Czech Ministry of Education No. 111300003 and 111300004.

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