What’s in a name? It turns out……. everything. In medicine understanding the language that doctors, healthcare professionals, and researchers utilize for describing individual seizures or epilepsy is essential. Just like learning a foreign tongue, understanding the vocabulary for that language is the only way that useful communication can occur. Oftentimes patients will use terms such as “grand mal” or “petit mal” to describe their seizures. Yet, I am not sure what they are telling me as these names do not have meaning for the doctor, nurse, or other caretaker that is helping to control and cure these episodes. Therefore, familiarity with the venacular of seizures is vital so that helpful and useful information disseminates between patient and physician.

Seizures and epilepsy were first catalogued by Henri Gastaut in 1969. The accepted classifications were published in 1970 and updated for seizures in 1981 and then epilepsy syndromes in 1989. Although the changes were minimal, the purpose of this classification system was to provide a common dictionary and concepts to facilitate dialogue about research and treatment. These first efforts in classification were considered major breakthroughs at their time. However, clinicians recognized that our understanding of seizures in epilepsy would evolve as technological advances radically changed our ability to study and understand the causes, mechanisms, and ultimately the treatment of epilepsy. However, with the advent of a classification system and its widespread dissemination, we have now become accustomed to the current expressions that are utilized for the description of seizures and epilepsy. Because these names are imprinted in our minds, it becomes quite difficult to make changes even when medical science recommends us to do so. Therefore, when changes are proposed with regards to the terminology used for seizures, controversy and confusion ensues. Nevertheless, it is important that we are all on the same page so that we can define the terms appropriately and use these terms in light of current knowledge to help propel epilepsy.

On the epilepsy.com website there are multiple links that help to explain the different phrases used to describe epilepsy. For a more detailed discussion regarding these terms, I refer you to the following links: http://www.epilepsy.com/EPILEPSY/Types_seizures.

Recently, in 2009, the International League against Epilepsy (ILAE) proceeded to make changes to the current classification of seizures and epilepsy. Given that November is Epilepsy Month, I share with you a basic review and provide a glimpse of some of the proposed changes. One of the criticisms raised against the current classification system is that there is a heavy reliance on the use of EEG to make a diagnosis. There is also a significant amount of attention paid as to whether a patient loses consciousness as a distinction between two of the most common partial seizure types. Moreover, the various features of an individual seizure that can be helpful in giving doctors useful information for finding the area of brain responsible for those seizures is not taken into account. As such, let me briefly review how seizures are currently classified and how we propose to change the terms utilized to describe the seizures.

Seizures are the individual symptom of epilepsy. Epilepsy is a condition of having more than one seizure. Both epilepsies and seizures are divided by answering one important question: Are the seizures starting from one area of the brain, which would term the seizure as being partial; or does the seizures start from both sides of the brain at one, which is termed generalize? The distinction between partial and generalized has important clinical ramifications, particularly in deciding what treatments are offered. For instance, some medications can worsen generalized seizures and surgery is most offered to patients who have partial epilepsy. The new system for classification changes the names so that there is no confusion based on these issues.

The new system, as proposed by the International League against Epilepsy in 2009, helps to establish seizures in epilepsy along three organizing patterns:

• Electroclinical syndromes based on age

• Constellations

• Epilepsies associated with structural or metabolic conditions.

The first system arranges syndromes involving epilepsy by age of onset. Distinctive constellations, which are a group of symptoms that helps to form a recognizable pattern associated with epilepsy are another form of cataloging. Then epilepsies attributed to by structural and metabolic causes are organized; these are seizures that happen as a result of some other condition. Lastly, angiomas is used to describe epilepsy caused by stroke and birth injuries whereas epilepsies that we have yet to classify round out the system.

The biggest change in the classification of seizures comes in the area of partial seizures. Two of the most commonly recognized seizure terms are complex partial and simple partial seizures. Complex partials are seizures that start from a discrete area of the brain and results in loss of consciousness; whereas simple partial seizures are seizures which also arise from the discrete part of the brain, but there is no loss of consciousness. In the new classification system, partial seizures, both complex and simple partial, will no longer be used as terms to describe seizures. Instead, the term focal seizures will be utilized to make this distinction. In the instance of simple partial seizures, this will be known as focal seizures without impairment of consciousness and then a description of the symptoms that occur with the seizures. For complex partial seizures, they will be called focal seizures with impairment of consciousness, and then a description of symptoms associated with the events.

As for epilepsy syndromes classified by age of onset, the following list helps to summarize the various epilepsy types occurring at different ages according to this new taxonomy. In infants, one can see the following syndromes:

• Epilepsy of Infancy with Migrating Focal Seizures

• West Syndrome

• Myoclonic Epilepsy in Infancy

• Benign Infantile Epilepsy

• Benign Familial Infantile Epilepsy

• Dravet Syndrome

• Myoclonic Encephalopathy in Non-Progressive Disorders

In childhood, the following syndromes can be seen:

• Febrile Seizures Plus (FS+)

• Panayiotopoulos Syndrome

• Epilepsy with Myoclonic Atonic Seizures

• Benign Epilepsy with Central Temporal Spikes

• Autosomal Dominant Nocturnal Frontal Lobe Epilepsy

• Late Onset of Childhood Occipital Epilepsy

• Epilepsy with Myoclonic Absences

• Lennox-Gastaut Syndrome

• Epileptic Encephalopathy with Continuous Spike and Wave during Sleep

• Landau-Kleffner Syndrome

• Childhood Absence Epilepsy

In adolescence to adults, the following syndromes can occur:

• Juvenile Absence Epilepsy

• Juvenile Myoclonic Epilepsy

• Epilepsy with Generalized Tonic-Clonic Seizures Alone

• Progressive Myoclonic Epilepsy

• Autosomal Dominant Epilepsy with Auditory Features

• Other Familial Temporal Lobe Epilepsy

• Familial Focal Epilepsy with Variable Foci

• Reflex Epilepsies

Under the new system there are also constellations. Constellations are a grouping of symptoms that are somehow associated with epilepsy but may not have the characteristics to describe it as a syndrome. Although this may sound like gobbledegook to the reader, these are basically groupings of symptoms that many of you will recognize and the following are the areas that are considered distinctive constellations:

• Medial Temporal Lobe Epilepsy with Hippocampal Sclerosis

• Rasmussen Syndrome

• Gelastic Seizures with Hypothalamic Hemartoma

• Hemiconvulsion-Hemiplegia-Epilepsy

Epilepsies attributed to structural or metabolic problems elsewhere in the brain or body will fall under the following categories:

• Malformations of Cortical Development (Hemimegalencephaly, Heterotopias)

• Neurocutaneous Syndromes (Tuber Sclerosis Complex and Sturge-Weber)

• Tumor

• Infection

• Trauma

• Angiomas

  • Perinatal Insults
  • Stroke

Seizures that do not require diagnosis of epilepsy will include:

• Benign Neonatal Seizures
• Febrile Seizures

How Can Epilepsy Therapy Project Help?

Given that the mission of the Epilepsy Therapy Project is to advance and speed the transition of novel therapeutic innovations from the laboratory to the marketplace where it can best help patients, it is essential that we are aware of the changes that are proposed in the classification of seizures and epilepsy. Because epilepsy.com is an important educational resource for the epilepsy community, it is essential that you are mindful of the vocabulary and idiom when discussing the issue of seizures and epilepsy. Epilepsy Therapy Project partners with research organizations to help get invaluable treatments to the patient. Speaking the jargon of epilepsy specialists, will foster a better knowledge of this condition and help destigmatize this disease so that we may be able to eradicate it in the future.