Lennox-Gastaut Syndrome

Lennox-Gastaut Syndrome

What is Lennox-Gastaut Syndrome?

Lennox-Gastaut Syndrome is a severe form of epilepsy that affects between one and five of every 100 children with epilepsy. It is known as one of the most common forms of intractable (hard to treat) child epilepsy. It is not a form of adult epilepsy and will most often present itself between the ages of three and five years old.

It is also sometimes called epileptic and developmental encephalopathy, as the seizures are thought to be instrumental in the children’s developmental delay and can affect learning difficulties.

typical-symptoms

What are the symptoms of Lennox-Gastaut Syndrome?

The symptoms of Lennox-Gastaut Syndrome usually occur in the form of seizures, but these can vary from child to child. The most common types of seizures are atomic and tonic seizures. These are also known as drop attacks, as the child will always drop to the ground sharply. It may at first look as though the child has tripped or has poor balance.

In an atomic seizure, the child will fall like a rag doll, with floppy limbs. In a tonic seizure, the limbs will stiffen as they fall. The seizures will often happen multiple times in one day and injuries can occur as a result, often to the face, teeth, and head.

Another common type is an atypical absence seizure, often lasting anywhere from ten seconds to several minutes. During this, the child can appear vacant or blank and exhibit behaviour such as head nodding, rapid eye blinking, or turning the head from side to side. They may still have some awareness of what’s going on around them.

These seizures can be very frequent, even appearing to be constant, which is known as a state of non-convulsive status epilepticus. During these longer seizures, the child might drool, be slow or unable to speak, need help eating, or be floppy and off balance.

A focal seizure is another one of the most common seizures, often occurring while the child is asleep. They start in the temporal lobes and can present themselves in a variety of different ways, depending on the child.

There are several more types of seizures that a child with Lennox-Gaustat Syndrome may experience; you can see the symptoms of these other types and find out more about them on the Epilepsy Action website.

All children with Lennox-Gaustat Syndrome will also develop learning difficulties to a moderate or sometimes severe degree. Some may even exhibit these difficulties before the seizures present themselves.

risk-factors

What causes Lennox-Gastaut Syndrome?

For 70-80% of children with Lennox-Gastaut Syndrome, there is a definable cause for their condition, but these causes vary. They can include:

  • Abnormal development of the brain cortex, also known as cortical dysplasia
  • Congenital infections
  • Stroke
  • Trauma
  • Reduced oxygen supply that occurs before in utero, also known as perinatal hypoxia
  • Infections of the central nervous system, such as encephalitis or meningitis
  • A rare genetic disorder called tuberous sclerosis
  • A previous history of West syndrome (these cases of Lennox-Gaustat Syndrome tend to be more severe)

Some cases of the condition have no known cause, with the child also not having a history of seizures or prior neurological problems. These cases tend to present themselves at a later age, although still usually before the age of six.

treatments

What treatment options are available?

Treatment for Lennox-Gaustat Syndrome can be difficult, as not all forms of treatment are effective for all children affected. Prescription medicines that can be effective include:

  • Sodium valproate (Epilim)
  • Lamotrigine (Lamictal)
  • Levetiracetam (Keppra)
  • Topiramate (Topamax)
  • Clobazam (Frisium)
  • Phenytoin (Epanutin)
  • Corticosteroid drugs (short-term use to help with particularly bad seizures)
  • Cannabidiol (particularly useful for atomic and tonic seizures)

Sometimes ketogenic dietary therapy can also be useful in reducing the regularity of seizures. For children who suffer from repeated drop attacks, there is also a form of epileptic surgery that can reduce the number and severity of their seizures.

Overall, the long-term prognosis for children with Lennox-Gastaut Syndrome is poor. For some, the seizures will recede when they reach teenagehood, but for most, symptoms will continue, usually on a daily basis, well into adult life. It is more a matter of managing the symptoms, rather than finding a cure.

common-conditions

How can medical cannabis help with Lennox-Gastaut Syndrome?

When traditional medicine proves unhelpful, medical cannabis might offer some relief. In 2019, a study in the United States showed positive results for the use of medical cannabis with child epilepsy. 

Of course, when it comes to treating child epilepsy, or any child condition, parents want to know that the dosage and treatment are exactly right. Our experts at The Medical Cannabis Clinics can advise you on the right form of treatment through a private consultation and devise a personalised plan to help manage the child’s symptoms and offer them a better, more comfortable quality of life. Appointments start from just £70.

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