What Is Developmental And Epileptic Encephalopathy (DEE) and How Does It Affect You?

Jing-Jane Tsa^*
*Correspondence: Jing-Jane Tsa, Department of Neurology, National Cheng Kung University, Taiwan, Tel: +5637992781, Email:

Letter

Developmental and Epileptic Encephalopathy (DEE) is a set of severe epilepsies characterised by drug-resistant seizures as well as encephalopathy, which is a word used to indicate considerable developmental delay or even loss of developmental skills. There are two variables that lead to developmental delay in DEEs.

The term "developmental encephalopathy" refers to developmental delays caused by the underlying cause of their epilepsy.

The International League Against Epilepsy recognises eight age-related epileptic encephalopathy disorders in its classification. Early myoclonic encephalopathy and Ohtahara syndrome in infants, West syndrome and Dravet syndrome in childhood, myoclonic status in non-progressive encephalopathy’s, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sleep in childhood and adolescence are among these syndromes.

Other epileptic disorders, such as migrating partial seizures in children and severe epilepsy with several independent spike foci, could be included to the list. We present an overview of epileptic encephalopathy in this work, including clinical neurophysiological aspects, cognitive impairment, and treatment choices, keeping in mind that these disorders are typically resistant to normal antiepileptic medicines.

In addition, some children with DEEs develop an epileptic encephalopathy as a result of frequent seizures and abnormal EEGs, which can exacerbate developmental issues.

Importantly, if seizure management can be improved, the Epileptic Encephalopathy component of the delay should improve, but the Developmental Encephalopathy component of the delay would remain unchanged.

Infants with Epileptic Encephalopathy Syndromes

The majority of DEEs start early in life, frequently in infants. Seizures in children are common and severe, and they can be of various sorts. It's common to notice epileptic spasms, tonic or atonic seizures, and myoclonic seizures. Seizures are usually permanent, though they can subside over time with some syndromes or specific reasons.

Electroencephalograms (EEGs) in children with DEEs are frequently aberrant, demonstrating diffuse background slowing and frequent seizure discharges. However, in rare circumstances, EEGs performed early on (before to or immediately after the commencement of the seizure) may not reveal any abnormalities.

“Early Infantile Epileptic Encephalopathy (EIEE) with burst-suppression” or “early myoclonic encephalopathy (EME)” Symptoms normally occur within the first three months of birth, and in most cases within the first ten days. Symptoms usually develop during the first few hours after birth, although in some cases, moms have reported feeling suspected seizures activity while still in the womb. In previously healthy youngsters, the onset is sudden. Tonic/clonic, clonic, myoclonic, atonic, absences, partial, complicated partial (with or without subsequent generalisation), gelastics, and Jacksonians are the most common seizure patterns.

During both waking and sleeping stages, the EEG pattern is characterised by burst suppression. This implies the EEG (electroencephalogram) shows periods of very low electrical brain activity followed by a burst of intense spiky activity before returning to very low activity. One side of the brain appears to be affected more than the other at times. Seizures are uncontrollable, though they can be improved in some situations with treatment. With severe psychomotor impairment and major learning difficulties, the prognosis is often poor. West syndrome or partial epilepsy are common complications (usually during infants).