Lennox- Gastaut Syndrome

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What is Lennox-Gastaut Syndrome?

Lennox-Gastaut Syndrome is a type of epilepsy where a person may experience multiple types of seizures including tonic (stiffening of the body), atonic (brief loss of muscle tone), and atypical absence  (starring). This type of seizure often begins during infancy and early childhood.

Statistics and Facts

4% of children with epilepsy are diagnosed with Lennox-Gastaut Syndrome with more of a prevalence in males than females. Most mortality rates are due to accidents.

Other Known Names

LGS

Symptoms

Symptoms often include frequent seizures and multiple types of seizures including:

  • Atonic seizures (drop attacks)- a sudden loss of muscle tone and limpness, a person may suddenly fall which can lead to head injuries.
  • Tonic seizures- the body stiffens and can last for up to a minute. Will generally occur when the person is asleep.
  • Abnormal seizures- a period pf unconsciousness where the person has no memory of the episode.

Causes

  • Abnormal development of the brain cortex
  • Congenital infections
  • Stroke
  • Trauma
  • Lack of oxygen during birth
  • Encephalitis
  • Meningitis
  • Tuberous Sclerosis
  • Seizures that start in infancy referred to as West syndrome

Diagnostic and Test

  • Clinical evaluation
  • Detailed patient history
  • Complete physical and neurological evaluation
  • Electroencephalography (EEG)
  • Magnetic Resonance Imaging (MRI)
Treatment
  • Anti-epileptic drugs (AED’s)
  • Dietary therapy
  • Surgery (VNS therapy)

 

Disclaimer: this article is for information only and should not be used for the diagnosis or treatment or medical condition.

West’s Syndrome

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What is West’s Syndrome?

The average onset generally begins at an average age of 6 months. Symptoms may include a pattern of an infant bending forward with a stiffening of the body, including the arms and the legs. Each episode generally last for a few seconds. These episodes typically occurs I clusters that can last up to 20 minutes.

Causes
  • Trauma
  • Brain malfunctions
  • Infections
  • Down syndrome
  • Tuberous sclerosis complex
Statistics and Facts

West syndrome occurs 1 in every 2,000 to 6,000 live births, which peak between the ages of 4 and 7 months old. 90 percent of incidences occur before the age of 1 year. West syndrome accounts for approximately 30 percent of incidences involving infants. Boys are more likely to be affected than girls.

Other Know Names
  • Generalized Flexion Epilepsy
  • Infantile Epileptic Encephalopathy
  • Infantile Myoclonic Encephalopathy
  • Jackknife Convulsions
  • Massive Myoclonia
  • Infantile Spasms
  • Salaam Spasms
History

Initially discovered in the 1840’s by Dr. William James West when he noticed his own son, James E. West showed the characteristics of “bobbings” that caused a complete heaving of the head towards his knees and then immediately relaxing to the upright position. Dr. West originally coined the phenomena as “Salaam Tics”.

Diagnosis and Testing
  • Electroencephalography (EEG)
  • A physical and neurological exam
  • Computer Tomography (CT)
  • Magnetic resonance Imaging (MRI)
  • Blood Test
  • Urine Test
  • Wood Lamp (for the purpose determining if tuberous sclerosis is a possible diagnosis)
Treatment
  • Steroid therapy by injection into a muscle or prednisone by mouth
  • A seizure medication called Sabril
  • Ketogenic diet

 

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions.

Benign Roladric Epilepsy

Benign Rolandic Epilepsy

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What is Benign Rolandic Epilepsy?

Benign Rolandic Epilepsy is a type of seizure that affects primary children. It is referred to as “Benign” since most children grow out of it during puberty and “Rolandic” describes the part of the brain where seizures begin.

Other know names:

  • benign focal epilepsy
  • benign childhood epilepsy with centrotemporal spikes (BCECETS)
  • benign rolandic epilepsy of childhood (BREC)
  • benign epilepsy with centro-temporal spikes (BECTS)

How Common is Benign Rolandic Epilepsy?

Benign Rolandic Epilepsy (BRE) is considered one of the most common types of epilepsy children will experience. It generally begins between the ages of 3 and 13 and peaks around the age of 7-8 and will stop around the ages of 14-18 (puberty). Children usually do well with this type of seizure although some may experience learning disability including reading, language and drawing.

Symptoms

  • A feeling of tingling on one side of the mouth which involves the tongue, lips, gums and inner side of cheek.
  • Generally begins when the child is sleeping or wakes up in the morning characterized by occurring in clusters and long intervals with no seizure activity.
  • twitching movements on one side of the face.
  • May make gurgling sounds.
  • Drooling and the inability to speak.
  • Day time seizures may be infrequent and typically last less than 2 minutes.
  • May spread from the rolandric area to the rest of the brain which becomes a tonic-clonic  seizures.

Diagnosis

Diagnosis are done through an accurate history of the seizures and an electroencephalogram (EEG) test, which picks up seizure activity.

Treatment

In most cases, children may not take any medication since the seizures end around puberty. May be treated with anti-epileptic drugs including:

  • Carbamazepine
  • Levetiracetam
  • Oxcarbazepine

How professionals can help

  • Recognize the signs of seizures
  • Teachers should be aware that a child may often feel tired and sleepy afterwards for hours
  • There may be learning difficulties and behavior problems

Resources