Topical Review Article: The Age-Dependent Epileptic Encephalopathies

Donat, Jane F.

doi:10.1177/088307389200700102

Cited by 61 publications

(37 citation statements)

References 54 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A common feature is that these disorders are usually refractory to standard antiepileptic drugs (AEDs) [3]. As a result, more aggressive use of AEDs considered effective in suppressing interictal epileptiform discharges (e.g., benzodiazepines, valproic acid, and lamotrigine), immunomodulatory therapies (e.g., corticosteroids, intravenous immunoglobulin (IVIG), and plasmapheresis), ketogenic diet, and surgical options is often considered [3].…”

Section: Introductionmentioning

confidence: 99%

Epileptic Encephalopathies: An Overview

Khan

Baradie

2012

Epilepsy Research and Treatment

View full text Add to dashboard Cite

Epileptic encephalopathies are an epileptic condition characterized by epileptiform abnormalities associated with progressive cerebral dysfunction. In the classification of the International League Against Epilepsy eight age-related epileptic encephalopathy syndromes are recognized. These syndromes include early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, West syndrome and Dravet syndrome in infancy, myoclonic status in nonprogressive encephalopathies, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sleep in childhood and adolescences. Other epileptic syndromes such as migrating partial seizures in infancy and severe epilepsy with multiple independent spike foci may be reasonably added. In this paper, we provide an overview of epileptic encephalopathies including clinical neurophysiological features, cognitive deterioration, and management options especially that these conditions are generally refractory to standard antiepileptic drugs.

show abstract

Section: Introductionmentioning

confidence: 99%

Epileptic Encephalopathies: An Overview

Khan

Baradie

2012

Epilepsy Research and Treatment

View full text Add to dashboard Cite

show abstract

“…This syndrome was subsequently termed the Landau-Kleffner syndrome (LKS) or acquired epileptic aphasia (Commission on Classification and Terminology of the ILAE, 1989). Although other epileptic syndromes such as infantile spasms and Lennox-Gastaut, collectively known as epileptic encephalopathies, are associated with loss of cognitive skills [Donat, 1992], LKS was the first syndrome linking epilepsy, electroencephalographic (EEG) activity, and isolated language loss [Beaumanoir, 1992;Deonna, 1991;Landau and Kleffner, 1957]. Subsequently, Tassinari and his coworkers described a syndrome, electrical status epilepticus in slow wave sleep (ESES), where continuous epileptiform activity in slow wave sleep was associated with regression of specific skills, including language [Commission on Classification, 1989;Patry et al, 1971;Tassinari et al, 2000;.…”

mentioning

confidence: 99%

Landau–Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children

McVicar

Shinnar

2004

Ment. Retard. Dev. Disabil. Res. Rev.

View full text Add to dashboard Cite

The Landau-Kleffner syndrome (LKS) and electrical status epilepticus in slow wave sleep (ESES) are rare childhood-onset epileptic encephalopathies in which loss of language skills occurs in the context of an epileptiform EEG activated in sleep. Although in LKS the loss of function is limited to language, in ESES there is a wider spectrum of cognitive impairment. The two syndromes are distinct but have some overlap. The relationship between the epileptiform EEG abnormalities and the loss of cognitive function remains controversial, even in LKS which is the most widely accepted as an acquired epileptic aphasia. Language regression also occurs in younger children, frequently in the context of a more global autistic regression. Many of these children have epileptiform EEGs. The term autistic regression with epileptiform EEG has been proposed for these children. Whether these children are part of an extended LKS spectrum is very controversial, because there are differences in age of onset, clinical phenotype, and EEG findings. An understanding of the available data on clinical characteristics, EEG findings, pathology, prognosis, and treatment of these syndromes is essential for further progress in this area.

show abstract

“…Therefore, MIS continues to attract research concerning both pathogenesis and therapy. Several large series [1,3,[9][10][11] and recent reviews [6,8,[12][13][14] have focused on clinical and electroencephalographic phenomenology of MIS, and on the therapy and outcome aspects of this entity. Here I introduce an age-specific endogenous-convulsant hypothesis for the pathophysiology of MIS.…”

Section: Introductionmentioning

confidence: 99%

Pathophysiology of massive infantile spasms: Perpective on the putative role of the brain adrenal axis

Baram

1993

Annals of Neurology

135

View full text Add to dashboard Cite

Massive infantile spasms are an age-specific seizure syndrome of infancy. Uniquely, the spasms respond to hormonal manipulation using adrenocorticotropic hormone (ACTH) or glucocorticoids. A hypothesis explaining the efficacy of hormonal therapy, age-specificity, multiple causative factors, and spontaneous resolution of infantile spasms is presented. Corticotropin-releasing hormone (CRH), an excitant neuropeptide suppressed by ACTH/steroids, is implicated. Evidence for the age-specific convulsant properties of CRH is presented, and a putative scenario in which a stressinduced enhancement of endogenous CRH-mediated seizures is discussed. Clinical testing of the CRH-excess theory and its therapeutic implications are suggested.Massive infantile spasms (MIS) represent a seizure disorder with unique clinical and electrographic features [1][2][3][4]. It is relatively common (1:2,000-4,000 births [5,6]) and has been known to respond to adrenocorticotropic hormone (ACTH), since 1958 [7]. Long-term intellectual outcome of affected infants, however, remains poor, with 76 to 95% of survivors having moderate to severe mental retardation [1,3,6,8]. Therefore, MIS continues to attract research concerning both pathogenesis and therapy. Several large series [1,3,[9][10][11] and recent reviews [6,8,[12][13][14] have focused on clinical and electroencephalographic phenomenology of MIS, and on the therapy and outcome aspects of this entity. Here I introduce an age-specific endogenous-convulsant hypothesis for the pathophysiology of MIS. The hypothesis implicates an endogenous neuropeptide, which is known to cause seizures in infant rats, and is suppressed by ACTH and glucocorticoids (GCs). I shall present evidence for this hypothesis, discuss some of its predictions for treatment options, and place it in the context of current therapeutic controversies. Definition and DescriptionInfantile spasms were first described by West [15] The syndrome of MIS consists of a constellation of myoclonic seizures in an infant, whose EEG pattern is that of hypsarrhythmia or its variants [1-4, 7-11, 14, 17]. The electroencephalographic (EEG) pattern, response to therapy, and poor outcome distinguish MIS from a variety of other myoclonic epilepsies of infancy [1,18]. Furthermore, MIS is a time-locked entity; it arises in infancy after a delay from the time of insult and, in the majority of cases, disappears spontaneously. Even without treatment, 89% of patients are reported to be spasm free by 5 years of age [19]. Proposed PathophysiologyMIS develops in infants with a variety of central nervous system (CNS) pathologies [1][2][3][4]. Structural anomalies, tuberous sclerosis, and other phakomatoses are commonly associated with MIS. Prenatal as well as peri-and postnatal infections, stroke, trauma, and even chromosomal aberrations have all been implicated as causative factors. This multitude of associated factors has suggested that MIS may be a "final common pathway" or an agespecific yet cause-nonspecific response of the brain [1,20,21]. Cor...

show abstract

Topical Review Article: The Age-Dependent Epileptic Encephalopathies

Cited by 61 publications

References 54 publications

Epileptic Encephalopathies: An Overview

Epileptic Encephalopathies: An Overview

Landau–Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children

Pathophysiology of massive infantile spasms: Perpective on the putative role of the brain adrenal axis

Contact Info

Product

Resources

About