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A

Absence Seizure (“Petit Mal”)

Absence seizures account for 2-4 percent of epilepsy in children and begins between ages 3 and 10. They are characterized by brief episodes of staring, usually lasting only 2-10 seconds and may happen repeatedly during the day. There is no warning before a seizure and the child is completely alert afterwards, with no memory of it. Because they are so mild, the child might not even realize they had one and it’s easily not noticed by those around them. Forty percent of children with this epilepsy syndrome will outgrow it or go into remission by their teenage years.

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Aicardi Syndrome

A rare inherited (genetic) disorder in which the structure that connects the two sides of the brain (corpus callosum) is partly or completely missing.
As a type of seizure? The girls who have been diagnosed with Aicardi Syndrome have many different types of seizures. It is also more than just ACC or partial ACC. There is retinal lacunae, and Infantile Spasms. (I’m not sure why it would be listed as a type of seizure, perhaps as a seizure disorder). There is not just one type that is experienced with Aicardi Syndrome

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Alice in Wonderland Syndrome (Micropsia)

Children with Alice in Wonderland Syndrome (or AIWS) feel that their body is changing, because of migraines and headaches. They don’t just see themselves changing in size, though, but they also see other people, animals and objects look larger or smaller than they actually are. They can also feel that their hearing and sense of touch have changed. Like what happens to Alice in the beginning of the book, some people with epilepsy who also have AIWS can feel like they are falling down a hole…

Children are the most affected by Alice in Wonderland Syndrome. Most of the effects occur in the dark, where they feel the most scared. Oddly enough, some cough syrup ingredients might cause AIWS.. Other causes for AIWS in children are Epstein-Barr virus and Mononucleosis (or Mono for short). Most times, the symptom will last for only one month. And although it’s unlikely, adults are able to get Alice in Wonderland Syndrome as well.

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Atonic Seizures (Drop Attacks)

Without warning, a person will abruptly loses consciousness, collapse and fall to the floor. Your head may drop suddenly, your eyelids may droop, your head may nod, and you may drop things. This is caused by sudden loss of muscle tone. Although it’s not usually associated with loss of consciousness and There is no convulsion, it’s possible to bang your head as you fall. Recovery occurs after a few seconds. You regain consciousness, and can again stand and walk.

Atypical Absence Seizures

The person will stare (as they would in any absence seizure) but often is somewhat responsive. You may experience eye blinking or slight jerking movements of the lips.

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Autonomic Seizures

These seizures are accompanied by autonomic symptoms or signs, such as abdominal discomfort or nausea which may rise into the throat (epigastric rising), stomach pain, the rumbling sounds of gas moving in the intestines belching, flatulence and vomiting. This has sometimes been referred to as abdominal epilepsy. Other symptoms may include pallor, flushing, sweating, hair standing on end, dilation of the pupils, alterations in heart rate and respiration, and urination. A few people may experience sexual

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Atypical Absence Seizures

The person will stare (as they would in any absence seizure) but often is somewhat responsive. You may experience eye blinking or slight jerking movements of the lips.

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Angelman syndrome

Angelman syndrome (AS) is a genetic disorder rather than an epilepsy syndrome. AS is a rare condition, which affects about one in 15,000 children. It used to be called the ‘happy puppet’ syndrome because children with this syndrome often have a happy mood and movements that can look like a puppet. However, Angelman syndrome should be the only name used today. Children have learning difficulties and speech delay that are usually severe. They also have jerking movements, tongue-thrusting, a characteristic (typical) facial appearance, and a happy mood with sudden bursts of laughter and epilepsy. Many children also have a fascination for water, usually running water. The condition is due to an abnormality on chromosome 15. It is usually diagnosed between four and 10 years of age, but sometimes earlier.

B

Benign Rolandic Epilepsy (Sylvan Seizures or Benign Partial Epilepsy of Children)

Accounts for more than one third of epilepsy beginning in middle childhood between ages 3 and 13. Seizures usually occur infrequently in children, as generalized nocturnal seizures characterized by a variety of minor tonic-clonic movements and often affect only one side of the face. A typical attack involves twitching, numbness, or tingling of the child’s face or tongue (a partial seizure), which often interferes with speech and may cause drooling There’s a jerking of the corner of the mouth that may spread to the rest of that side of the face, causing a twisting motion. The child usually does not lose consciousness, except in cases of secondarily generalized seizures of this type. In rare cases, the seizure may progress to encompass the entire side of the body, becoming a generalized tonic-clonic condition. Seizures typically occur at night and these children are otherwise normal and healthy. The prognosis is favorable with 95% of children outgrowing their seizures by age 15

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Benign myoclonic epilepsy in infancy

This is a very rare epilepsy syndrome. It is more common in boys than girls. Seizures begin from 4 months to 3 years of age, but most commonly between 1 and 2 years of age. Approximately a third of children with this condition have a family member who has epilepsy, or has had febrile convulsions as a young child. In most cases no cause is found for this type of epilepsy

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Benign partial epilepsy in infancy

There is little information about this epilepsy syndrome. No one really knows how common it is. Of every 100 children whose epilepsy starts at less than 2 years of age, between 5 and 10 will be found to have this epilepsy syndrome. It probably happens equally in boys and girls. It is important to rule out other causes of partial (focal) seizures in infants before making a diagnosis of benign partial epilepsy in infancy. This is usually called a 'diagnosis of exclusion'

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Benign neonatal convulsions

Benign neonatal convulsions (BNC) is a relatively uncommon type of epilepsy that happens in babies and very young infants. The seizures, or convulsions, start in the neonatal period – between birth and 28 days after the baby has been born. Most seizures occur between 3 and 14 days after the baby has been born. Because many start on the fifth day of life, they used to be called ‘fifth day fits’. The seizures affect girls and boys equally.

There are 2 types of BNC:
• Benign familial neonatal convulsions
• Benign non-familial (also called sporadic) neonatal convulsions

In the first type, benign familial neonatal convulsions, someone else in the family must have had similar convulsions and this is usually one of the baby’s parents. It may be hard for the parents to know whether they had convulsions as a baby, because they won’t remember themselves! Therefore, if possible, the baby’s grandparents should be asked if they can remember whether the baby’s parents had convulsions or ‘fits’ at the same age. A genetic fault that causes benign familial neonatal convulsions has been found in some genes, which are part of chromosome number 8 and also chromosome number 20.

In the second type, benign non-familial (also called ‘sporadic’) neonatal convulsions, there is no history of convulsions at a similar age in other members of the family

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Benign epilepsy of childhood with occipital paroxysms (BECOP)

Benign epilepsy of childhood with occipital paroxysms (BECOP) can start at any age from 15 months to 17 years. But it usually begins in middle childhood between seven and 11 years of age. This epilepsy syndrome is often called ‘late-onset occipital epilepsy’ to separate if from ‘early-onset occipital epilepsy’, also known as Panayiotopoulos syndrome.

About one third of children have a family history of epilepsy. Some children have had seizures with feverish illnesses (febrile convulsions) before starting to have BECOP. Most children with this condition have normal neurological and learning abilities. Brain scans are normal

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C

Catamenial Seizures

This is seizure activity associated with a woman’s menstrual cycle. Studies have shown that fluctuations in female hormones prior to and during menstruation may elevate seizure frequency in some women. In a small percentage of women, catamenial seizures may occur only during the days just before their periods; for others, during the period itself. Higher ratios of estrogen to progesterone may increase seizure frequency.... Water retention, electrolyte imbalance, and even poor sleep are also contributing causes.

The connection between menstruation and seizures has been demonstrated in women with simple partial, complex partial, and generalized tonic-clonic seizures. However, different types of seizures may be associated with different phases of a woman’s menstrual cycle. For example, a women with absence seizures may have more seizures during days 16 to 28 of her average cycle and may have the least during her period. A women with partial seizures, on the other hand, tends to have fewer seizures during days 16 to 28 and more just prior to ovulation and during menstruation.

 

Clonic seizure

During a clonic seizure, you may lose control of bodily functions and begin jerking rhythmically in various parts of your body. Consciousness may be temporarily lost and followed by confusion. Clonic seizures begin in early childhood. With time, clonic seizures may eventually progress to generalized tonic-clonic seizures.

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Focal Impaired Awareness Seizure

(Complex Partial Seizures)    (Psychomotor or Temporal Lobe Seizures)    Old Name

Begins with a blank look or empty stare. You may be unaware of your surroundings and seem dazed and confused. The seizure may progress to include chewing movements, mumbling, uncoordinated activity, or sometimes performing meaningless bits of behavior which appear random and clumsy. These may include picking at your clothes, trying to remove them, walking about aimlessly, picking up things, or mumbling. Someone experiencing a complex partial seizure may become frightened and try to run and struggle. Following the seizure, there will be no memory of it. A complex partial seizure usually lasts about 2 to 4 minutes. It may be followed by a longer lasting confusion. Once the pattern of seizures is established, it will usually be repeated with each subsequent seizure

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Childhood absence epilepsy (CAE)

This is a common epilepsy syndrome starting in early childhood. Absence seizures can happen in many different epilepsy syndromes occurring in childhood and adolescence. This information refers specifically to the syndrome of childhood absence epilepsy

D

Doose Syndrome

Also known as Myoclonic-Astatic Epilepsy (MAE) is an epilepsy syndrome of early childhood that is often resistant to medication. For this reason, it can be difficult to treat. MAE is idiopathic generalized epilepsy, meaning that there is no known cause for the seizures (idiopathic) and the seizures originate from all over the brain (generalized) as opposed to coming from one focal area. Onset of MAE occurs commonly in the first five years of life, with the mean age being three. Statistics show that it usually affects children who have previously developed normally, and boys are twice as likely as girls to develop MAE. In some cases, other family members (immediate or extended) may also have seizures.

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Dravet Syndrome — Severe Myoclonic Epilepsy of Infancy

(SMEI), is a severe form of epilepsy. It appears during the first year of life with frequent febrile seizures – fever-related seizures that, by definition, are rare beyond age 5.Later, other types of seizures typically arise, including myoclonus (involuntary muscle spasms). Status epilepticus – a state of continuous seizure requiring emergency medical care – also may occur.

Children with Dravet syndrome typically experience poor development of language and motor skills, hyperactivity, and difficulty relating to others.
In 30 to 80 percent of cases, Dravet syndrome is caused by defects in a gene required for the proper function of brain cells

E

Eclampsia

Convulsions are the other most common feature of this syndrome. Convulsions are usually generalized tonic-clonic in nature. Usually a brief single seizure occurs. Multiple seizures can also occur and status epilepticus is rare. Partial seizures or complex partial seizures can also occur. The seizures can occur prepartum, intrapartum, or postpartum. If the seizure occurs postpartum, it usually occurs within the first 24 hours aft...er delivery, however, late postpartum eclamptic convulsions are by no means rare and have been reported as late as 23 days postpartum.

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Ecstatic Seizures

Also called Dostoyevsky’s Epilepsy, are a type of seizure activity that is characterized by feelings of ecstasy or transcendent joy. Mystical, spiritual, and hallucinatory experiences often occur as well. The temporal lobe is effected by the seizure activity and provides a neural basis for these experiences. These seizures can involve more than one seizure symptom, such as tonic-clonic, tonic & more

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Early myoclonic encephalopathy

Early myoclonic encephalopathy almost always starts in the newborn period or in very early infancy, before 2 or 3 months of age. It is a syndrome with many causes. One underlying cause is metabolic disorders, such as a condition called non-ketotic hyperglycinaemia. Other causes are brain malformations and neurogenetic disorders (problems with the brain or nerves that are inherited). Sometimes no cause can be found.

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Electrical status epilepticus during slow-wave sleep (ESESS)

This is a rare epilepsy syndrome. The most common age for it to develop is in mid-childhood. It is also called ‘continuous spike-wave of slow sleep’. The cause of this syndrome is not known. It usually happens in children who already have epilepsy

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Epilepsy with myoclonic absences

Epilepsy with myoclonic absences is a rare form of epilepsy. It happens slightly more commonly in boys than girls, and approximately 25 out of every 100 children (one quarter) will have another family member with epilepsy. The family member may have the same or a different form of epilepsy. The underlying cause of this epilepsy syndrome is unknown. It is likely there will be a genetic cause. Myoclonic absences may be seen in children with brain abnormalities or with some genetic disorders but it may also be seen in children with no obvious cause.

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Eyelid myoclonia with absences (EMA)

This epilepsy syndrome is rare and may be difficult to diagnose. It is sometimes mistaken for a much more common type of epilepsy called childhood absence epilepsy.

In EMA, most children start having seizures around 2 or 3 years of age. However, because the absences last only one to 3 seconds (rarely up to 4) and may not be noticed by the family, it may not be diagnosed until much later.

Eyelid myoclonia with absences (EMA) may also be none as Jeavons syndrome

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F

Febrile Seizures

Children aged 3 months to 5 years may have febrile seizures when they have a high fever. This occurs in only 2% to 5% of all children. A febrile seizure is usually mild and brief, often resulting in a slight slumping and loss of consciousness, or a rolling of the eyes back in the head. Sometimes there may be convulsive stiffening and jerking, but there is no need to panic. Protect the child from sharp, hot, or otherwise ...dangerous objects. Loosen tight clothing. Do not put anything in the child’s mouth. Do not restrict his/her movements. Roll the child on his/her side and try to keep everyone relaxed.

There are 2 types of febrile seizures: simple and complex. Simple febrile seizures are more common and relatively harmless. They usually last less than 15 minutes and have no after-effects. A neurological examination following a simple seizure will yield no abnormalities.

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(Complex) Febrile Sizures

May be more threatening. They can last longer than 15 minutes and although only one side of the body is affected during a complex febrile seizure, neurological reports may indicate abnormalities.

In both cases, febrile seizures pose no threat of mental retardation, cerebral palsy, learning disabilities, or death. Only 25-30% of children who experience one will ever have another. And the incidence of febrile seizures does not indicate a possibility of developing long-term epilepsy. Less than 2% of children who experience febrile seizures will develop epilepsy later in life. Febrile seizures are classed as incidents rather than as a condition.

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Focal Cortical Dysplasia

This is the most common cause of intractable epilepsy in children and is a frequent cause of epilepsy in adults. All forms of focal cortical dysplasia lead to disorganization of the normal structure of the cerebral cortex.

Frontal Lobe Epilepsy

May produce weakness or inability to use certain muscles, including those that govern speech. Frontal lobe seizures may involve thrashing movements during sleep, also stiffening with the head turned to one side and the arm rising into a brief frozen state. Some seizures may be dramatic and upsetting to others, with screaming, bicycling movements of the legs, running. Treatment is with medication, and, in some cases, surgery

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Frontal Lobe Epilepsy

May produce weakness or inability to use certain muscles, including those that govern speech. Frontal lobe seizures may involve thrashing movements during sleep, also stiffening with the head turned to one side and the arm rising into a brief frozen state. Some seizures may be dramatic and upsetting to others, with screaming, bicycling movements of the legs, running. Treatment is with medication, and, in some cases, surgery.

G

Gelastic Seizures

These seizures are both unpredictable and unprovoked by the person’s surroundings. They are abrupt in onset and quickly over. They may occur nocturnally, waking you from sleep and leaving you exhausted. Basically, they are characterized by brief outbursts of emotion, usually in the form of a laugh or a cry. They may be accompanied by forced eye movements, chewing or grinding the teeth, tonic posturing, and clonic jerking. You may appear confused and/or dazed during and after an episode. Gelastic seizures usually last 5 to 60 seconds and you may remember them clearly or may be completely unaware of what occurred.

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GLUT1 deficiency syndrome

This is a rare genetic disorder that impairs brain metabolism. GLUT1 (a protein) is responsible for the transport of glucose (a sugar) from the blood into the brain. Glucose is the main source of fuel for the brain. A shortage of glucose leads to impairment of brain function and growth.

SCL2A1 is the name of the gene that provides instructions to produce the protein, GLUT1. It is found on chromosome 1. Mutations (changes in the way the gene is made up or ‘sequenced’) in the SCL2A1 gene cause this condition. It is inherited in an autosomal dominant pattern. This means one copy of the altered gene in a cell is enough to cause the condition. However, most cases happen because of a new mutation in the gene. This means there will be no family history, although rarely one parent may be mildly affected.

Most children start having seizures in the first few months of life, although some children only develop seizures later, at around 2 or 4 years of age. They may also show other neurological symptoms including difficulties with balance and co-ordination and they may show abnormal movements of their limbs and eyes.

GLUT1 deficiency syndrome may also be none as Glucose transporter type 1 deficiency syndrome

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Generalised epilepsy with febrile seizure plus (GEFS+)

Generalised epilepsy with febrile seizures plus (GEFS+) is an unusual epilepsy syndrome. It describes families who have several members from different generations with different types of epileptic seizures and even different epilepsy syndromes. The epileptic seizures nearly always start in a family member who has had febrile convulsions.

Febrile convulsions are seizures associated with a high temperature. Usually febrile convulsions stop after the age of 6 years. In GEFS+ families, children may go on to have febrile seizures well beyond this age, even into adult life. They may also develop other seizure types not associated with a high temperature. Very rarely, there may be a family member with a very severe type of epilepsy called Dravet syndrome or a less severe type, myoclonic-astatic epilepsy (Doose syndrome). Dravet syndrome used to be called severe myoclonic epilepsy in infancy (SMEI).

GEFS+ has only recently been described as an epilepsy syndrome in its own right. Some people with GEFS+ are found to have a specific fault, called a ‘mutation’, in their genes. The gene that is commonly at fault is the SCN1A gene but other genes may also be at fault. This is providing a lot of interest in and information on the genetic basis of many different types of epilepsy.

Generalised epilepsy with febrile seizures plus (GEFS+) may also be none as Genetic epilepsy with febrile seizures plus

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H

Hot water epilepsy

Is one of the reflex epilepsies induced by hot water being poured over the head, face, neck, or trunk during bathing. Two types of hot water epilepsies exist:

the classical one seen in older children and adults, which is usually triggered by head baths,

And a variant seen in infants precipitated by immersion of the lower trunk in hot water. hot water epilepsy is increasingly being reported all around the world. Hot water epilepsy in infancy may have an association with febrile seizures and Dravet syndrome,

 This type of reflex epilepsy was first reported from New Zealand in 1945 (Allen 1945). Even though cases have been subsequently reported from different countries around the world and across all races, there was a clustering of cases from South India

Apart from classical hot water epilepsy, another type of reflex epilepsy triggered by hot water baths in infants has also been reported in literature (Plouin and Vigevano 2004). This condition is also called bathing epilepsy or water immersion epilepsy, as the temperature of the water has not always been hot and the trigger includes immersion of only the lower part of the body in hot water, as in the Japanese style bath

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I

Infantile Spasms (West Syndrome)
These consist of a cluster of sudden jerks followed by stiffening. Often the arms are flung out as the knees are pulled up and the body bends forward.
Infantile spasms consist of clusters of sudden, quick movements. Typically, if the child is sitting up, the head may fall forward, the arms will flex forward, and the body may flex at the waist. If lying down, the knees will be drawn up, with arms and h...ead flexed forward as if the body is reaching for support. Individual spasms last only 1 or 2 seconds. They often repeat in a series of 5 to 50 or more. A child may have many series per day. Spasms are most likely to occur when the child is drowsy, just waking from a nap or falling asleep. They’re sometimes called “jackknife seizures” and are very rare. They occur only during the first year of life, usually starting around 3-7 months of age. Many children with infantile spasms have associated developmental delay and may go on to develop other forms of epilepsy, such as Lennox-Gastaut syndrome

J

Jacksonian Seizure (Also called Jacksonian March)
A kind of a simple partial seizure. “Simple” in this context means patients do not lose awareness. Partial means that abnormal neuron firing only occurs in part of the brain, and, accordingly, abnormal movement or sensation is limited to only part of the body. The characteristic features of Jacksonian march are that it only occurs on one side of the body and it progresses in a predictable... pattern from twitching or a tingling sensation or weakness in a finger, a big toe or the corner of the mouth, then marches over a few seconds to the entire hand, foot or facial muscles.
Jacksonian march seizures are generally brief and relatively mild. They are episodic, come and go. There is no confusion afterwards. Sometimes patients may not even notice them. Some patients may also have a phenomenon called “automatism,” such as compulsively licking the lips, fumbling with clothing, or other rhythmic finger movements. Others may experience head turning, eye movement, muscle cramping, numbness, tingling, and a crawling sensation over the skin. Hallucinations can occur too, which can be visual or auditory, meaning patients may see or hear things that are not there.

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Juvenile Myoclonic Epilepsy
(Also called Janz’s syndrome, impulsive petit mal, myoclonic epilepsy of adolescence, and jerk epilepsy.) Typically begins at puberty in otherwise healthy children. The first symptom is usually a generalized convulsion. These children may also have myoclonic seizures (jerking of the muscles) on awakening. A hand may suddenly fling out, a shoulder may shrug, a foot may kick, or the entire body may jerk. A child may spill or drop whatever he or she is holding or fall from their chair. Absence seizures may also occur. Juvenile myoclonic seizures can occur as a single event or in a series. Consciousness and memory are not impaired

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Juvenile absence epilepsy

Juvenile absence epilepsy (JAE) is a relatively common epilepsy syndrome. The main seizure type in JAE is absence seizures. These can happen in other childhood and adolescent epilepsy syndromes, including childhood absence epilepsy (CAE) and juvenile myoclonic epilepsy (JME).

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Jeavons Syndrome

Also known as Eyelid myoclonia with absences (EMA) is a generalized epileptic condition clinically characterized by eyelid myoclonia (EM) with or without absences, eye closure-induced electroencephalography (EEG) paroxysms, and photosensitivity; in addition, rare tonic-clonic seizures may also occur. Although first described in 1977 and widely reported by several authors within the last few years, EMA has not been yet recognized as a definite epileptic syndrome. However, when strict criteria are applied to the diagnosis, EMA appears to be a distinctive condition that could be considered a myoclonic epileptic syndrome, with myoclonia limited to the eyelids, rather than an epileptic syndrome with absences.

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L

Lafora Disease
Is a severe form of epilepsy, characterized by seizures and progressive neurological degeneration. It occurs during late childhood or early adolescence. Death usually occurs within 10 years of the first symptoms....

Landau-Kleffner Syndrome
Also a rare disorder beginning between ages of 3 and 7. Produces seizures and affects speech. Children develop normal speech, and then slowly lose it. Simple partial and tonic clonic seizures. Treated with antiepileptic drugs to control seizures, and, possibly, steroids.

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Lennox-Gastaut Syndrome
Affects between 3-10% of children with epilepsy, more commonly males. The peak age for onset is between 1 and 8 years of age with extreme incidence occurring in the first and tenth years of life The most characteristic manifestation of the Lennox-Gastaut syndrome is in a large variety of seizures.


Limbic Epilepsy
Limbic areas are regions in the temporal and frontal lobes, which are involved with memory and emotion and this is a seizure happening in this area

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Landau Kleffner Syndrome

Landau-Kleffner syndrome (LKS) is an age-related epilepsy syndrome of childhood. Its main features are a loss of speech and language skills, with seizures. The speech and language skills may improve over time. LKS usually starts before the age of 6 years and affects twice as many boys as girls. The underlying cause of LKS is not yet known

M

Musicogenic Seizures,

Singing Seizures, and TEA (Transient Epilepsy Amnesia) which is interesting because both Inter-Ictal and Post-Ictal the patient is fully aware of total memory loss....
For those that don’t know: Musicogenic Epilepsy is a form of reflexive epilepsy in which a seizure is triggered by music or specific frequencies.

Sensitivity to music varies from person to person.

Some people are sensitive to a particular tone from a voice or instrument. Others are sensitive to a particular musical style or rhythm. Still others are sensitive to a range of noises.

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Doosy Syndrome (Myclonic Astatic Epiliepsy or MAE)

“Myclonic Astatic Epiliepsy or MAE is actually a atonic or drop attack.

It’s an epilepsy syndrome of early childhood that is often resistant to medication.
For this reason, it can be difficult to treat.
MAE is an idiopathic generalized epilepsy, meaning that there is no known cause for the seizures (idiopathic) and the seizures originate from all over the brain (generalized) as opposed to coming from one focal area.
Onset of MAE occurs commonly in the first five years of life, with the mean age being three.
Statistics show that it usually affects children who have previously developed normally, and boys are twice as likely as girls to develop MAE.
In some cases, other family members (immediate or extended) may also have seizures.”

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Motor Seizures
They are a form of simple partial seizures which include clonic, jerking, convulsive movements. Jerking typically begins in one area of the body – your face, arm, leg, or trunk — and may spread to other parts of the body. These seizures are sometimes called Jacksonian motor seizures, their spread is called a Jacksonian march and they cannot be stopped.

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Multifocal Seizures
While most seizures can be neatly split into partial and generalized, there exists some that don’t fit. For example: the seizure may be generalized only within one hemisphere. Alternatively there may be many focal points (multifocal seizures) that are distributed in a symmetrical or asymmetrical pattern

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Mitochondrial disorders

The mitochondrial disorders are an uncommon group of conditions that are known as ‘metabolic disorders’.

There are many different types of mitochondrial disorders. The mitochondria are known as the ‘powerhouse of the cell’. All our organs (brain, nerves, muscles, heart, liver and so on) are made up of millions of cells. These cells can only work properly if the mitochondria in the cells work properly. The mitochondria are as important to the cells as the engine is to a car.

The organs with the most mitochondria are the brain, nerves, muscles and liver. Because of this, epilepsy occurs quite commonly in mitochondrial disorders. Most of the epilepsy starts in childhood and usually in the first 2 years of life. However, it can start within a few days of birth, or as late as the teenage years or even adult life

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Migrating partial epilepsy in infancy

This epilepsy syndrome was described for the first time in 1995. This makes it one of the newer epilepsy syndromes. It is very rare.

Most children start having seizures in the first few weeks of life and all will start having seizures by 6 months of age.

migrating partial epilepsy in infancy may also be none as migrating partial seizures of infancy and malignant migrating partial seizures in infancy

N

Neonatal Seizures
Neonatal seizures occur in babies soon after birth. As many as 1.5 to 2.5% of newborns have seizures in the first month of life. A further 20% of all seizures in children under 3 years of age have neonatal seizures. They’re generally classified as subtle, clonic, tonic, and myoclonic.
Subtle attacks are characterized by apneas with episodes of pallor, fixed staring, deviation of one or both eyes, eye blink, motor chang...es, episodic chewing movements, or stereotypic limb movements such as swimming or bicycling motions. Drooling and unusual alertness may accompany neonatal seizures. Usually, most neonatal seizures occur over only a few days and fewer than half of affected infants develop seizures later in life.

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Nocturnal Seizures
These are usually tonic-clonic. They might occur just after a person has fallen asleep, just before waking, during daytime sleep, or while in a state of drowsiness. People who experience nocturnal seizures may find it difficult to wake up or to stay awake. Although unaware of having had a seizure while asleep, they may arise with a headache, have temper tantrums, or other destructive behavior throughout the day.
Nocturnal seizures are very uncommon and their mechanisms poorly understood. There is evidence that sleep enhances epileptic discharges in the EEG, though their daytime recordings may appear to be normal. However, if a pattern of limiting seizures to the hours of slumber is maintained, the chance of them occurring during the daytime is greatly reduced

 

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O

Ohtahara Syndrome

This is a very rare epilepsy syndrome. Seizures start before 3 months of age. Most babies have an underlying structural brain abnormality. This may be genetic in origin (passed on through the genes), or happen because of brain damage before or around the time of birth.

Ohtahara syndrome may also be none as Early infantile epileptic and encephalopathy with suppression bursts

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P

Post Traumatic Seizures
Seizures may develop immediately after an injury to the brain or may develop in delayed fashion, showing up months or years after the initial trauma. Generally speaking, the risk of post traumatic seizures is related to the severity of the injury. The greater the injury, the higher the risk of developing seizures. Even mild to moderate injuries can result in seizures.
It is thought that a head injury disrupts the pathways of the brain and that an epileptic seizure can be viewed as a sort of short circuit of the brain’s electrical functioning. During the seizure the electrical fields in the brain are overloaded, resulting in seizures. The most commonly seen seizures related to traumatic brain injury are “generalized” seizures, which are also called tonic-clonic or “grand mal” seizures.

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Primary Reading Epilepsy
This is a reflex epilepsy where seizures are triggered by reading. Seizures usually begin in adolescence, and onset is unusual in younger children or adults over 30 years old. Patients report jaw jerking or clicking while reading, often with jerks of the arms, and if reading continues, a generalized convulsion may occur. Transient cognitive impairment has also been noted with the jerks.

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Progressive Myoclonic Epilepsy
A rare, form of epilepsy with myoclonic (jerking) and tonic-clonic (grand mal) seizures. Children with this condition may have trouble with maintaining balance and experience rigid muscles. There is also a loss of mental ability. A gene for this disorder has recently been discovered

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Psychogenic Seizures (PNES or “Pseudo Seizures”)
Psychogenic seizures are not classified as a form of epilepsy. They may be brought on by stress, trauma, anxiety or a history of sexual abuse.
It’s difficult to differentiate between psychogenic and epileptic seizures. However, one reliable indicator of a psychogenic seizure is eye closure during the seizure. If you have a seizure, your eyes tend to stay open. Still, statistics indicate that, in 20-30% of cases, epileptologists are incorrect in attempting to distinguish one from the other. Although psychogenic seizures are not caused by electrical discharges in the brain and thus do not register any EEG abnormalities, they are often mistaken for epileptic disorders. It’s also possible to have both psychogenic seizures and epilepsy. Most patients with psychogenic seizures are misdiagnosed and consequently treated with epilepsy drugs or other epilepsy therapies, sometimes with severe and fatal side effects

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Pattern — Sensitive Epilepsies
In this reflex condition, seizures are produced by particular visual patterns. These triggers may consist of circles, stripes, or other patterns, usually of high contrast. Moving patterns are most likely to incite a seizure.

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Photosensitive Epilepsy
If you have photosensitive epilepsy, certain types of flickering or flashing light may incite a seizure. The trigger could be exposure to television screens due to the flicker or rolling images, computer monitors, certain video games or TV broadcasts containing rapid flashes, even alternating patterns of different colors, in addition to intense strobe lights. And surprisingly, seizures may be triggered by natural light, such as sunlight, especially when shimmering off water, even sun flickering through trees or through the slats of Venetian blinds,

Also Sunflower syndrome: 

The stereotyped, episodic abnormal behaviour manifested by a child and her mother on exposure to a particular sort of sunlight is described in detail. The child is the first fully documented patient with this so-called “self-induced” form of photosensitive epilepsy in whom for several years no electroencephalographic sensitivity to flickering light could be demonstrated.

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Panayiotopoulos syndrome

It is not known how common this syndrome is. It may affect between 1 in 10 or 1 in 20 of all children with epilepsy. The most common age it happens is 3 to 5 years, but it may affect children as young as 1, and as old as 10 years. Boys and girls appear to be equally affected. It does not usually run in families. More research needs to be undertaken in this epilepsy syndrome.

Panayiotopoulos syndrome is different to the epilepsy syndrome called ‘benign epilepsy of childhood with occipital paroxysms (BECOP)’. BECOP is sometimes called Gastaut syndrome. Gastaut syndrome is less common than Panayiotopoulos syndrome. In BECOP, or Gastaut syndrome, the children are usually older, between 6 and 13 years. The seizures include problems with vision and flashing lights and are usually accompanied by headaches. The seizures are briefer in duration than in Panayiotopoulos syndrome. 

Panayiotopoulos syndrome may also be none as Early-onset benign partial epilepsy with occipital paroxysms and Idiopathic susceptibility to early-onset benign childhood seizures with EEG occipital spikes

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Partial (Focal) Seizure

They may occur at any age, as a single episode or as a repeated, chronic seizure disorder. They are seen less frequently in children than in adults, but still account for about 45% of pediatric seizure disorders. Patients with focal seizures can have any of the symptoms below. Those with simple focal seizures do not lose consciousness and will be aware and remember the events that occur at the time. If you have complex partial seizures, you will have abnormal consciousness and may or may not remember any or all of the symptoms or events surrounding the seizure.

Symptoms include: abnormal muscle contractions affecting one side of the body, abnormal head movements, staring spells, abnormal mouth movements like lip smacking, chewing or swallowing without cause, numbness, tingling, abdominal pain, nausea, or discomfort, sweating, a flushed face, rapid heart rate or pulse, sensation of déjà vu and changes in mood or emotion. 

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R

Rasmussen’s Encephalitis — also Chronic Focal Encephalitis (CFE)
This is a rare, progressive neurological disorder which affects one half of the brain, producing severe seizures, loss of motor control and speech along with paralysis on one side of the body. Various treatments have been tried, including surgery to remove the affected half of the brain....

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Reflex Epilepsy
A seizure is considered a reflex seizure when it occurs as a direct response to a particular stimulus or event in the environment. These seizures are experienced by approximately 6% of people with epilepsy. They can be triggered by visual stimulation, a sudden “startle”, or a variety of other factors alone or in combination. If a patient only has seizures as a result of a certain stimulus then it is possible to control them by avoidance. Begins in childhood, is associated with absence epilepsy, and may disappear in adulthood

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Rett syndrome

This syndrome happens in about one in every 10,000-12,000 girls. It very rarely affects boys, but when it does, they are always affected far more severely than girls. Rett syndrome is caused by a genetic abnormality, called a ‘mutation’. This abnormality or mutation is usually found on one of the sex chromosomes – the X chromosome. The most common mutation is called the MECP2 mutation. A different and much less commonly found genetic mutation is nearly always seen in those children with Rett syndrome who have a very severe type of epilepsy. This less common mutation is called the CDKL5 mutation.

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Ring Chromosome 20

Ring Chromosome 20 (R20) is a rare condition, which causes epilepsy in children. It is caused by an abnormality in chromosome number 20. Chromosomes are structures within each cell in the body, which hold our genetic material. Each human being has 23 pairs of chromosomes - 46 in total.

When viewed down a microscope, chromosomes usually look fairly straight. In R20 a little piece of genetic material is missing from each end of one of the number 20 chromosomes, and the ends fuse together to form a ring. This happens very early in pregnancy. It happens sporadically (by chance) and therefore does not usually affect more than one child in the family

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S

Secondarily Generalized Seizures
Generally, they’re partial seizures evolving into generalized seizures, most often with tonic-clonic convulsions. The partial seizures which were once limited to one hemisphere of the brain progress to encompass the entire brain bilaterally, causing a generalized seizure. The clinical nature of a secondarily generalized seizure usually does not differ from that of the initial, originating seizure.
Secondarily generalized seizures are predominant in 16% of all children and 9% of all adults with seizure disorders. Most people with complex partial seizures and many with simple partial seizures will experience such a seizure at some point. When they occur frequently, the chances for future partial seizures may be increased.

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Focal aware (Simple Partial Seizures) (Focal Seizures) Old Name
Although your ability to respond may be preserved, motor manifestations or anxiety relating to the seizure symptoms may prevent you from responding appropriately You may see or hear things that are not there. Feel emotions, often fear, but sometimes sadness, anger, or joy. There may be a bad smell or a bad taste, a funny feeling in the pit of your stomach or a choking sensation. Sudden or restless movement or vision distortion are also characteristics. These seizures are sometimes called simple partial seizures of temporal lobe origin or temporal lobe auras.

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Startle Epilepsy
This is a type of reflex epilepsy in which seizures are provoked by loud noises or sudden surprises. Most patients with startle epilepsy are only sensitive to one sensory modality (i.e. temperature, taste, sound, pressure); however, it is the unexpected nature of the stimulus, rather than the sensory modality, that characterizes startle epilepsy. These seizures usually last less than 30 seconds. The seizure begins with a startle response, followed by a brief tonic phase. Patients sometimes fall to the ground and experience clonic jerks. Responsiveness to the stimulus decreases as a result of repeated exposure to the stimulus. Spontaneous seizures also occur in patients with startle epilepsy, but are infrequent in most cases.
People with startle epilepsy usually have static cerebral lesions and developmental delay. For many people, half of the body is partially paralyzed and it is the weak side of the body that is primarily involved in the startle seizures. Startle epilepsy is often associated with disorders such as Down syndrome and cortical dysplastic lesions.

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Status Epilepticus
Status epilepticus (SE) is a common, life-threatening neurologic disorder. It is essentially an acute, prolonged epileptic crisis. Aggressive treatment is necessary. Maintenance of vital signs, including respiratory function, is of major importance. It may occur as a first seizure or if you are known to have seizures. Duration may last for a long time, or repeat without recovery. This prolonged or repeated seizure activity can result in death if it’s not treated immediately. Status epilepticus can be convulsive (tonic-clonic or myoclonic seizures) or non-convulsive (absence or complex partial seizures). A person in non-convulsive status epilepticus may appear confused or dazed

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Sturge-Weber syndrome

Sturge-Weber syndrome (SWS) is a rare condition, in which the presence of a birthmark (called a ‘port wine’ stain), usually on one side of the face but sometimes both sides, is associated with an abnormality of the brain. This is caused by abnormal blood vessels on the surface of the brain. This abnormality usually results in epileptic seizures and other problems.

The abnormal blood vessels are usually over the back part of the brain (the occipital lobe). They may also be in other regions of the brain and, rarely, on both sides of the brain. The abnormality develops during pregnancy, probably in the first 3 to 4 months.

SWS is usually a spasmodic occurrence – meaning it does not run in families. It is very, very rare for two children in the same family to have SWS

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Sunflower syndrome:    Please see photosensitive epilepsy

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Sensory Seizures

Some simple partial seizures consist of a sensory experience. People with sensor seizures may smell or taste things that aren’t there, hear clicking, ringing, or a person’s voice when there is no actual sound. You may also see lights, hear a buzzing sound, or feel tingling or numbness in a part of the body. Simple partial seizures usually last just a few seconds, although they may be longer. If there are no convulsions, they may not be obvious to those around you.

T

Tonic Seizures
Tonic seizures are characterized by facial and muscle spasms of your trunk, flexing or reaching of your upper and lower extremities, and impaired consciousness. Several types of tonic seizures exist. Those grouped with absence, myoclonic, and atonic seizures are non-convulsive and tend to be brief. The more prolonged seizures usually are convulsive and may manifest dilation of your pupils, tachycardia, apnea, a bluish ting...e to your skin, salivation, and the loss of bladder or bowel control. Tonic seizures are often followed by postictal confusion.

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Tonic-Clonic Seizures (“Grand Mal”)
In a generalized tonic-clonic (grand mal) seizure, you will probably giver out a short cry and fall to the floor. Your muscles will stiffen during the tonic phase and then, during the clonic phase your extremities will jerk and twitch. Often you will lose consciousness, stop breathing or have difficulty breathing, turn blue and lose bladder control which in not uncommon, but extremely embarrassing.
Afterwards, you may feel tired, confused and disorientated. This may last from 5 minutes to several hours or even days. Rarely, this disorientation may last up to 2 weeks. You may asleep, or gradually become less confused until full consciousness is regained

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Temporal Lobe Epilepsy

The seizures associated with temporal lobe epilepsy consist of simple partial seizures without loss of awareness and complex partial seizures, with loss of awareness. You lose awareness during a complex partial seizure because the seizure spreads to involve both temporal lobes, which causes impairment of memory. The result is complex partial seizures coupled with simple partial seizures. Generalized tonic clonic seizures may be part of this syndrome as well

U

Unverricht-Lundborg syndrome

This condition is very rare. Most children with this syndrome begin to have epilepsy between the age of 6 and 16 years. It is one of the epilepsies in the group called ‘progressive myoclonic epilepsy’.

One of the main problems in this syndrome seems to be a fault in the part of the brain called the cerebellum. The cerebellum controls motor activity, for example it sends messages to the muscles telling them to move smoothly. Following a period of malfunction, these nerve cells will eventually die. The motor problems are referred to as ataxia.

The condition is inherited genetically (in the genes). The pattern of genetic inheritance is called autosomal recessive. People with the condition receive one faulty gene from each of their parents. There are 2 copies of our genes, therefore the parents themselves are quite healthy because they still have a good gene copy working. But every time they have a child they have a 1 in 4 chance of having another child with the same condition.

The genetic cause of Unverricht-Lundborg syndrome is due to a mutation in a gene called the CSTB gene. The CSTB gene gives instructions for making a protein called cystatin B. This protein is important in brain function and particularly part of the brain called the cerebellum. The abnormal cystatin B protein causes the different symptoms and signs in Unverricht-Lundborg syndrome.

Other names for Unverricht-Lundborg syndrome

• Baltic myoclonus
• A progressive myoclonic epilepsy
• Progressive myoclonic epilepsy Type 1

V

Vertigo Epilepsy
While epilepsy is commonly accompanied by dizziness or vertigo, vertigo is only rarely caused by epilepsy. This arises primarily because vertigo is much more commonly caused by ear conditions. Epileptic vertigo is due to brain injury, typically the part of the temporal lobe that processes vestibular signals. Loss of consciousness usually occurs at the time of injury. The typical symptom is “quick spins

W

Withdrawal Seizures
This type of seizure is seen when certain medications, such as barbiturates and benzodiazepines, are stopped abruptly. In this case, continued treatment with antiepileptic medications is usually not advisable. Withdrawal seizures are common when a person with alcoholism is trying to quit drinking. If a person with epilepsy drinks alcohol heavily and experiences withdrawal seizures, it is difficult for the physician or specialist to determine the exact cause of the seizure and to determine the next appropriate step. Therefore, it is important to avoid alcoholic beverages once you begin taking antiepileptic medications

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West syndrome (infantile spasms)

Infantile spasms are a type of epilepsy with a characteristic age of onset (typical age when seizures start), pattern of seizures and electroencephalogram (EEG). This means that it is an ‘electroclinical epileptic syndrome’. The syndrome is called ‘West syndrome’ after Dr West, who first described the condition in his 4-month-old son in 1841. This type of epilepsy occurs in about one in 2,500-3,000 children. Every year in the UK about 350-400 children will develop West syndrome

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UPDATED April 2024

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