Glossary
Below is a glossary of many of the terms used on the IDEA League website. Please feel free to browse the terms below or you will see them highlighted the first time they appear on any page on the site.
Ataxia - Inability (total or partial) to coordinate voluntary body movements. This is often first noticeable as a unstable walking gait.
Absence Seizure - Absence seizures usually occur in children and are characterized by brief episodes of staring with impairment of awareness and responsiveness. They begin and end suddenly. Often they can be accompanied by motor movement such as eye blinks, brief automatic mouth or hand movements, and or changes in muscle tone.
Adverse effect - Side effect; negative effect from medication or therapy
Anticonvulsant Medications - Medicines used to control seizures. Also referred to as antiepileptic drugs or AEDs.
Aphasia - A total or partial loss of ability to use or understand language; usually caused by stroke, brain disease, or injury.
Atonic seizure - An epileptic seizure characterized by sudden loss of muscle tone; may cause the head to drop suddenly, objects to fall from the hands, or the legs to lose strength, with falling and potential injury; usually not associated with loss of consciousness.
Atypical Absence Seizure - Also occurs mostly in children, often beginning before the age of 6. They may begin and end gradually (over seconds), usually last 5-30 seconds. The child stares but can be semi-responsive. Blinks, twitches and deviations of the eye may be seen as well as slight twitches of the lips. They are often hard to distinguish from a child’s usual behavior in children with global cognitive impairment.
Aura - A warning before a seizure; a simple partial seizure occuring within seconds before a complex partial or secondarily generalized tonic-clonic seizure, or it may occur alone; also a warning before a migraine headache.
Automatism - Automatic, involuntary movement during a seizure; may involve mouth, hand, leg, or body movements; consciousness is usually impaired; occurs during complex partial and absence seizures and after tonic-clonic seizures.
Autonomic - Pertaining to the autonomic nervous system, which controls bodily functions that are not under conscious control (e.g., heartbeat, breathing, sweating); some partial seizures may cause only autonomic symptoms; changes in autonomic functions are common during many seizures.
Autosomal dominant - A mode of inheritance in which a gene is passed on by either parent; in most cases, the child has a 50% chance of inheriting the gene; the expression of the gene (that is, the development of the physical trait or the disorder) can vary considerably among different individuals with the same gene.
Blood drug level - The concentration, or amount, of circulating drug in the bloodstream, measured in micrograms (µg) or nanograms (ng) per milliliter (mL). The concentration may be measured as the free or total level because some of the drug is bound to the protein in the blood and some is not; the free level is the amount of drug that is "free" (unbound); the total level is the amount of drug that is both bound and unbound to the blood protein; the drug that is free (unbound) is the portion that reaches the brain and exerts an effect on the disorder.
Clonic seizure - An epileptic seizure characterized by jerking movements and involving muscles on both sides of the body.
Cognitive - Pertaining to the mental processes of perceiving, thinking, and remembering; used loosely to refer to intellectual functions as opposed to physical functions.
Comprehensive epilepsy centers - Comprehensive epilepsy centers are clinics staffed by epileptologists and other experts in epilepsy treatment. They are valuable resources for anyone who has unresolved problems related to definite or suspected epilepsy. Patients may be referred to a comprehensive epilepsy center for a single outpatient visit to assess their diagnosis and therapy, or they may receive long-term follow-up and treatment, including epilepsy surgery or the use of new medications that are still being investigated.
Consciousness - State of awareness; if consciousness is preserved during a seizure, the person can respond (either in words or actions, such as raising a hand on command) and recall what occured during the spell.
Dietary Supplement - Products that contain one or more ingredients (such as vitamins, minerals and herbs) that are intended to supplement the diet, are intended for human use and are in the form of a tablet, capsule, powder or another preparation that is not a conventional food.
Electrode - A conductor through which electrical current enters or leaves. When used to record the electroencephalogram, a small metal disc attached to a wire is usually used.
Electroencephalogram (EEG) - A diagnostic test of brain electrical activity; helpful in diagnosing epilepsy.
Endocrinology - Endocrinology is a branch of medicine dealing with disorders ofthe endocrine system and its specific secretions called hormones. An endocrinologist is a doctor who specializes in treating such disorders.
Epilepsy - A disorder characterized by recurrent seizures. It is specifically defined as having two or more unprovoked seizures. Unprovoked means that it does not occur as a result of illness or injury.
Epilepsy syndrome - A disorder defined by seizure type, age of onset, clinical and EEG findings, family history, response to therapy, and prognosis.
Epileptologist - A neurologist with specialty training in epilepsy.
Expressive language skills - Ability to use spoken and/or written language.
Febrile - Associated with a fever.
Focus - The center r region of the brain from which seizures begin; used in reference to partial seizures.
Generalized seizure (grand mal seizure; convulsive seizure) - These seizures cause a loss of consciousness following an initial tonic phase of stiffening, a fall and often and audible cry forced out through contracted vocal cords. The subsequent, clonic phase, consist of jerking of the extremities which gradually slows before stopping. There may be drooling, biting of the tongue and loss of bowel control. These seizures can last from a few seconds to several minutes in duration. In the case of Dravet children, these are the seizures that can become problematic if not controlled, often developing into status epilepticus.
Hyperthermia - An increase in body temperature above normal.
Hypothermia - A condition of characterized by low body temperature.
Ictal - Referring to the period during a sudden attack, such as a seizure or stroke.
Interictal - Referring to the period between seizures.
Intractable -Difficult to alleviate, remedy, or cure; for example, intractable seizures are difficult to control with the usual antiepileptic drug therapy.
Intravenous immunoglobulin (IVIg) - A blood product mainly used as treatment in two major categories, inflammatory and autoimmune diseases , and immune deficiencies diseases featuring low antibody levels.
Ketogenic Diet - A form of antiseizure therapy that consists of a rigid high-fat diet that forces the body to produce higher than normal levels of ketones and use fat instead of glucose for energy.
Magnetic resonance imaging (MRI) - A scanning technique that creates pictures of the inside of the body and the brain; uses a strong magnet (does not use x-rays); more sensitive than CT.
Metabolism - The physical and chemical processses by which substances are produced or transformed (broken down) into energy or products for the uses of the body.
Mitochondrial disease - Mitochondrial myopathies are a group of neuromuscular diseases caused by damage to the mitochondria - small, energy-producing structures found in every cell in the body that serve as the cells' "power plants." Nerve cells in the brain and muscles require a great deal of energy, and thus appear to be particularly damaged when mitochondrial dysfunction occurs.
Movement Disorders - Psychomotor delay can be apparent as early as one year and increasingly obvious as the child approaches age two. May include, but is not limited to ataxic gait, tremors in large and fine motor movement as well as in resting state, poor coordination and ease of injury due to accidents.
Mutations - A sudden variation in an inheritable characteristic. In this case, a change in the gene identified and associated with Dravet syndrome.
Myoclonic seizures (myoclonus) - One of the primary seizure types associated with this syndrome, particularly when the children are under the age of four. These seizures involve a brief, shock-like jerk of a muscle or group of muscles. Myoclonus can occur in healthy people (the jerks we feel as we fall asleep). The epileptic myoclonus usually cause bilateral (two-sided; involving both arms, for example), simultaneous jerks of the body. These quick jerks often affect the neck, head and eyes only, shoulders, upper arms, entire body and upper legs. It is hard to verify, in research, if consciousness is impaired due to the short time span of the seizure (less than 1 second). If several occur in a row it can precipitate into a clonic seizure and may be associated with altered awareness.
Neurodevelopmental - The subject pertaining to the neurological (brain and nervous system) growth and related mental and physical developmental process. In this case, SMEI is a neurodevelopment disorder characterized by both cognitive and physical delays.
Neuron Cells - The neuron cell is the structural and functional unit of the nervous system. It consists of the nerve cell body and all of its processes which include an axon and one or more dendrites.
Orthotics - The science and art of custom designing and constructing appliances for the feet, ie, arch supports. Orthotics help in the prevention and correction of deformities that may hinder a person’s ease of movement. These also aid in support and alignment helping to improve function of the movable part of the body.
Orthopedic Disorders - Imparements of the skeletal system and associated structures such as muscles and ligaments, especially in the spine and extremeties.
Partial Seizures - Seizures that involve only part of the brain and the corresponding part(s) of the body. Simple Partial Seizures are partial sezures that do not impair consciuosness. Complex Partial Seizures are partial seizures during which consciuosness is altered but observers may think the affected person is fully aware of what they are doing. These seizures can take the form of automatisms or repetitions (chewing, playing with hair, even wandering away and having no recollection of where they have been) and can also be predisposed to developing into a secondary generalized tonic clonic seizure.
Perseveration - Persistent repetition of an activity, word, phrase, or movement, such as tapping, wiping, and picking.
Pes planus - The condition in which the arch of the foot collapses, with the entire sole of the foot coming into complete or near-complete contact with the ground.
Photosensitivity - Epileptic seizures triggered by certain frequencies of flashing or flickering lights, or by certain geometric shapes or patterns.
Regression - A process of moving backward (in this case in reference to development). For example, children with Dravet syndrome can often experience a loss of psychomotor development after the age of one year.
Receptive language skills - Ability to understand spoken and/or written language.
Seizure Threshold - The place at which neurons are able to function properly without going the next step into seizure state (either by exposure to environment or due to therapeutic deficiencies). For kids with Dravet syndrome, this is a fine balance!
Seizures - Seizures are caused by abnormal firing of brain cells that can lead to a change in behavior, such as slight temporary confusion, complete loss of consciousness or uncontrollable movements of arms and/or legs or other parts of the body.
Seperation anxiety - When an infant or toddler is anxious about being away from her primary caregiver. Infants can have this as early as 7 months, but separation anxiety usually peaks between a year and 18 months.
Sodium Ion Channel - A voltage-gated (electrically charged) ion channel that allows sodium ions to move in and out of cells and is necessary for the making and maintaining of action potentials, mainly in nerves and muscles.
Status Epilepticus - A prolonged seizure (usually defined as thirty minutes or more) or a series of repeated seizures; a continuous state of seizure activity; may occur in almost any seizure type. Often is resistant to medical interventions and results in hospitalization. For many children Dravet syndrome it is also associated with respiratory distress.
Stranger anxiety - A dislike or mistrust of unfamiliar people, often beginning around a baby's eighth or ninth month.
Therapeutic blood level - The amount of drug circulating in the bloodstream that brings about seizure control without troublesome adverse effects in most patients. "Subtherapeutic" (lower) levels are effective in some patients, and "supratherapeutic" or "toxic" (higher) levels are tolerated by others.
Tonic Clonic Seizures - (grand mal seizures; convulsive seizures) These seizures cause a loss of consciousness following an initial tonic phase of stiffening, a fall and often and audible cry forced out through contracted vocal cords. The subsequent, clonic phase, consist of jerking of the extremities which gradually slows before stopping. There may be drooling, biting of the tongue and loss of bowel control. These seizures can last from a few seconds to several minutes in duration. In the case of Dravet children, these are the seizures that can become problematic if not controlled, often developing into status epilepticus. Generalized tonic clonic seizures involve all or most of the brain and all or most of the body. Unilateral tonic clonic seizures involve only one side of the brain (left or right) and the corresponding side of the body.
Video-EEG monitoring - A technique for recording the behavior and the EEG of a patient simultaneously; changes in behavior can be correlated with changes in the EEG; useful for making the diagnosis of epilepsy and localizing the seizure focus.
VNS or Vagus Nerve Stimulator - A pacemaker-like therapeutic device implanted in the upper chest, which sends stimulation to the brain via the left vagus nerve and can reduce seizure activity.