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Tracking Seizures and Keeping Records

www.epinet.org.au/content.asp?contentid=606

Lennox-Gastaut Syndrome

Lennox-Gastaut Syndrome [LGS] is one of the more severe forms of epilepsy. It usually develops during preschool years and is characterised by several seizure types and developmental delay. Seizures are generally difficult to control due to their resistance to antiepileptic drugs.

Often no specific cause is identifiable: however, some of the known causes include:
· developmental malformations of the brain
· genetic brain diseases, such as tuberous sclerosis, and inherited metabolic brain diseases
· brain injury due to problems associated with pregnancy and birth including prematurity, asphyxia [lack of oxygen] and/or low birth weight
· severe brain infections including encephalitis, meningitis, toxoplasmosis and rubella.

In many instances, LGS is a sequel to infantile spasms – sudden spasms or bending of the body, either at the trunk or neck. These usually commence at between three and eight months, and may develop into the mixed seizure pattern, which characterises LGS, at two to three years. There are also a number of rare childhood diseases that may lead to LGS.

 

It is estimated that LGS occurs in between three to eleven per cent of childhood epilepsies with slightly more males than females affected. The average age of onset is three years.

LGS is diagnosed by some or all of the following:
· the presence of a mixed seizure pattern
· some degree of developmental delay or intellectual disability.
· a typical electroencephalogram [EEG] pattern of slow spike wave discharges may be present.

An EEG is a graphic chart that displays electrical activity generated from the brain, recorded at the skin surface of the head, commonly referred to as a brain wave recording.

Seizures

Seizures are always of several types and a child may experience some or all of these. The most common seizure types associated with LGS are tonic, atonic and atypical absence seizures. Periods of frequent seizures may be interspersed with periods of relative freedom from seizures. The most common seizure types associated with LGS are:

Behavioural disturbances

These include poor social skills and attention seeking behaviour, and have at least four possible causes:
· the underlying condition causing the epilepsy
· the effects of medication
· the electrical disturbance in the brain in uncontrolled epilepsy
· difficulty in interpreting information and understanding the world as others do.

Medication

Antiepileptic drugs [AEDs] are usually the best treatment for seizures although complete seizure control is rarely achieved. LGS often requires frequent changes in dose and type of medication, with some medications losing their initial effectiveness. Unlike other childhood epilepsies, the simultaneous use of more than one type of medication is often necessary.

The AEDs most commonly prescribed for LGS are listed in the table below. There are also a number of new AEDs currently being trialed in Australia and overseas.

Surgery

Rarely, corpus callosotomy can be considered for very severe drop attacks.

Status epilepticus

Status epilepticus ['status'] occurs in about 50 per cent of people with LGS and is the term used to describe prolonged seizures of 30 minutes or more, or the occurrence of repeated seizures without regaining consciousness between attacks.

Status can occur with any type of seizure and is categorised as either convulsive or non-convulsive Status can last from hours to days or, in the case of non-convulsive status, even weeks or months. Factors that may lead to status include sudden withdrawal from medication, illness, fever and infections.

Status in children with LGS is most often non-convulsive It is characterised by severe confusion, apathy or a 'flat' mood, stupor or, at worst, apparent dementia. Inconspicuous muscle spasms and greatly impaired balance and coordination are usually evident.

Convulsive status may ultimately lead to brain damage and death unless stopped quickly-usually with the administration of rectal diazepam. Some parents and carers learn to administer rectal diazepam at home. This option would need to be discussed with your doctor.

To date, little research has been conducted into the prognosis of children with LGS. It is clear, however, that prognosis varies from child to child. Generally, this syndrome is permanent. A few years after diagnosis, the epilepsy, which was initially so difficult to control, may become less of an issue but impaired intellectual functioning and behavioural problems frequently persist. Complete recovery, with normal development and freedom from seizures, is very unusual for the child with LGS.

Practical suggestions to help carers support the child with LGS

Consider using respite care schemes provided by local councils and other organisations. These schemes offer the services of a professional carer to visit your home to look after the child; or placement with another family or residential centre. Respite care can be on a regular or occasional basis.
· Keep a diary that details the child's seizures, seizure triggers and any side effects of current medications. This can be useful when consulting with doctors and in identifying seizure patterns and medication responses.
· Use an intercom in the child's bedroom to monitor any changes in their condition.
· On outings, consider using a child-stroller for school age children going through a phase of frequent seizures.
· Question your doctor about all aspects of the child's treatment, including medications and their side effects, to ensure that you are sufficiently informed to confidently coordinate the day-to-day management of the condition.
· If you have any unanswered questions about medications, contact your local pharmacist. However, do not alter your child's medication schedule without consulting your doctor.
· Consider a hand-held harness to help prevent falls and a helmet to protect the face and head.
· Lower the temperature on the household's hot water unit or install a thermostat to prevent scalds and burns.
· Buy a dosette box and tablet cutter from your local pharmacy to assist in preparing and administering medication.

Diet

The ketogenic diet has been clinically effective in reducing seizures. It is very high in fat, often unpalatable and must be followed exactly. The ketogenic diet elevates blood cholesterol levels markedly and is expensive to implement. It is not a long-term proposition. Some children benefit from this diet.

Vagus nerve stimulation

The vagus nerve stimulator is a device, similar in size to a heart pacemaker, which is implanted in the chest with a lead that passes up to the neck and is placed around the vagus nerve. This device stimulates the vagus nerve for about 30 seconds every 5 minutes and has been shown to improve seizure control in people with uncontrolled seizures. The device may take up to a year to show maximum benefit. This device requires an operation for insertion as well as for removal. The batteries require replacement approximately every 5 years entailing further surgery.

Attention to seizure triggers

It is recognised that the frequency of seizures may be associated with the child's level of alertness. The child who is overexcited for example, or lacks sufficient stimulation, may experience more seizures. A stimulating but stable environment can therefore be important in reducing the number of daily seizures. This may include a strict routine of regular meals, sleep and medication.

Illness may also trigger seizures. For example, seizure activity may increase just before the onset of high body temperature and fever. Vomiting and diarrhoea can also effect the body's ability to absorb medication.

Family support programs

Most state epilepsy associations run family support programs that often involve daytime and evening meetings, and usually include expert speakers. The Epilepsy Foundation of Victoria coordinates a group called the Uncontrolled Epilepsy Parents Support Group (UEPSG) for parents and carers of children with LGS and other forms of uncontrolled epilepsy. If you are unable to attend UEPSG meetings or you reside outside Victoria, you can be included on their mailing list which comprises many Australian families, and others worldwide.

It is recognised that the frequency of seizures may be associated with the child's level of alertness. The child who is overexcited for example, or lacks sufficient stimulation, may experience more seizures. A stimulating but stable environment can therefore be important in reducing the number of daily seizures. This may include a strict routine of regular meals, sleep and medication.

Illness may also trigger seizures. For example, seizure activity may increase just before the onset of high body temperature and fever. Vomiting and diarrhoea can also effect the body's ability to absorb medication.

Family support programs

Most state epilepsy associations run family support programs that often involve daytime and evening meetings, and usually include expert speakers. The Epilepsy Foundation of Victoria coordinates a group called the Uncontrolled Epilepsy Parents Support Group (UEPSG) for parents and carers of children with LGS and other forms of uncontrolled epilepsy. If you are unable to attend UEPSG meetings or you reside outside Victoria, you can be included on their mailing list which comprises many Australian families, and others worldwide.

Back to Epilepsy Foundation index

 

North East Valley Division General Practice, Victoria, Australia, Disclaimer 
Level 1, Pathology Building, Repatriation Campus, A&RMC, Heidelberg West VIC 3081. .. map
Phone: 03 9496 4333, Fax: 03 9496 4349,  Email: nevdgp@nevdgp.org.au
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Last modified: September 04, 2006