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Lennox-Gastaut syndrome  

 

 

 

Lennox-Gastaut syndrome is the most distressing of childhood epilepsies. The children suffer frequent fits of many different types, and experience gradual mental deterioration. Infantile spasms often evolve into Lennox-Gastaut syndrome, and the age of onset is from 1 to 8 years, peaking at 3 to 5 years. The prognosis for children with Lennox-Gastaut syndrome is bleak.

Clinically, neurologists define Lennox-Gastaut syndrome by three characteristics:

In Lennox-Gastaut syndrome the primary focus for the epilepsy appears to be the brain stem, that is, the area at the top of the spinal cord and below the cerebral cortex. Because of its central position and the fact that the "higher" brain functions evolved from the brain stem, epilepsies in this area are particularly harmful. A simplistic view is that epilepsies of the brain stem disrupt autonomic functions like reflexes, and that Lennox-Gastaut syndrome represents the disruption of the proper functioning of the higher areas of the brain because the basic functions in the brain stem are inadequate to support its proper operation.

How do I know if my child has Lennox-Gastaut syndrome?

The basic answer is that you know when your neurologist tells you. The symptoms are variable; the first manifestation of Lennox-Gastaut syndrome is often a head drop where the head suddenly drops because the muscles in the neck lose their tone. The head drop is usually brief and may be accompanied by a loss of consciousness. These head drops usually develop into full drop attacks where the child falls down because many of the muscles in the body lose their tone at the same time. The drop attacks are physically dangerous, because the child falls and hurts itself.

Lennox-Gastaut syndrome is characterised by multiple types of fit. As well as drop attacks, these are likely to include atypical absence fits and massive myoclonic jerks. Atypical absence fits may begin and end gradually, with consciousness only partially affected; the child may continue to be active, although mentally distant and abstracted. Myoclonic jerks are the violent jerking of one or more limbs.

Specific cases of Lennox-Gastaut syndrome may suffer from a wide variety of other forms of fits, and in particular from severe status epilepticus. In status, the child will lose consciousness, and may suffer from a sequence of atonic and myoclonic fits alternating between floppiness and rigidity. Status can persist for hours, days or weeks, and can cause severe mental damage. If you think your child is in status, you should call an ambulance immediately. If your child is at risk of status, you should always:

Treatment

Lennox-Gastaut syndrome is refractive to treatment. Because of the variety of types of fit associated with Lennox-Gastaut syndrome, neurologists often resort to using multiple drugs in high quantities, which is almost certainly harmful in itself. Vigabatrin and felbamate are newer drug treatments which have shown some promise with Lennox-Gastaut syndrome. The ketogenic diet appears to be a useful treatment, although there are not any good statistics.

Prognosis

Sadly the prognosis is poor. Most (60%) children will be refractive to drug therapy. Mental retardation may be present at the onset of Lennox-Gastaut syndrome, and deterioration is likely to occur subsequently. Most (70% - 95%) children will be mentally retarded, and most of these severely. Some (5%) children will die of complications - physical damage or status epilepticus. Those that become adult will have continuing problems. Most (80%) will continue to have fits through their adult life. Some (25%) will have emotional disorders. Almost all (95%) will have continuing mental disabilities, ranging from language difficulties to learning arrest and impaired movement. About half (50%) will be completely dependent and only some (20%) will be able to lead independent lives. It is easy to quote the statistics, but remember that each represents a personal tragedy.

Medical information

Definition
Lennox-Gastaut is an epileptic syndrome characterised by the triad of multiple fit types, diffuse spike slow waves (1-3Hz) and mental retardation.

History
First described by Tissot in 1770. Characteristic EEG described by Gibbs in 1938. Lennox established clinical correlation between EEG and physical symptoms in 1950.

Incidence
About 1 in 10,000 – this represents 5% of childhood epilepsies.

Age of onset from 1 to 8 years, peaking at 3 to 5 years. More prevalent in boys than girls.

Prior risk factors are infantile spasms (30%) and family history of epilepsy. Known causes include hypoxic ischaemic encephalopathy (lack of oxygen at birth), intrauterine infections (CMV, rubella, toxoplasmosis), head trauma, meningitis, tuberous sclerosis, brain tumours and metabolic disorders (lipid storage disease, aminoacidopathies).

Clinical features
Fits very frequent - 25 a day or more - 50% during inactive wakefulness, 30% during drowsiness.

Wide variety of fit types:

Factors associated with worst prognosis include infantile spasms, onset before 3 years, high frequency of fits, frequent status epilepticus.

Investigations
EEG shows abnormally slow background with diffuse interictal slow spike waves at leas than 3Hz during waking and bursts of fast (10Hz) waves during sleep. The maximal voltage area is usually anterior.

CT/MRI likely to show abnormalities, particularly subcortical cerebral atrophy.

Treatment
There is disagreement as to whether polytherapy or monotherapy is better. The precise drugs used will depend on the types of fits for a particular child. Typical drugs used include valproic acid, clonazepam, vigabatrin , felbamate and topimirate. ACTH can be used successfully for short periods, but the child will usually regress afterwards. The ketogenic diet appears to work well with some children, although there are no useful clinical studies.

Information about Lennox-Gastaut syndrome

There is very little useful information about Lennox-Gastaut syndrome on the Web.

  Lennox-Gastaut Support Group  
Andrew Gibson
Lennox-Gastaut Support Group
9 South View
Burrough on the Hill
Melton Mowbray
LE14 2JJ
Tel 01664 454305

"The original Lennox-Gastaut Support Group went into abeyance in 1995 and was re-formed in 1996. It offers support and information by telephone and letter and linking families where possible. It publishes a newsletter and has information available, details on request. Over 100 families and health care professionals are in membership"


LGS Group
The LGS group is a parents support organisation with a newsletter and snail mail interchange. The contact is:

  Candace Lovrich  
6443 Riggs Place
Los Angeles, CA. 90045
phone (310) 670-8279

Rob Evers’   Epilepsy in Young Children   contains profiles of a number of children (40+ at November 2002) suffering from Lennox-Gastaut syndrome.

The Massachusetts General Hospital runs a bulletin board system   Harvard neurology web forum for epilepsy which sometimes refers to Lennox-Gastaut

Many parents whose children have Lennox-Gastaut use the ketogenic diet and subscribe to the   Ketogenic diet list   or to the other   ketogenic diet list  .

There is one book published on the subject:

Niedermayer E, Degen R (eds),   Lennox-Gastaut syndrome  . New York: AR Liss Inc 1988 - ISBN 0471500380/5807-0382616-726789

Med line lists about 50 papers published since 1996 which refer to Lennox-Gastaut syndrome :

Motte J, et al
Lamotrigine for Generalized Seizures Associated with the Lennox-Gastaut syndrome.
N Engl J Med. 1997 Dec 18; 337(25): 1807-1812.
 
Langtry HD, et al
Topiramate. A review of its pharmacodynamic and pharmacokinetic properties and clinical efficacy in the management of epilepsy.
Drugs. 1997 Nov; 54(5): 752-773. Review.
 
Wheless JW, et al
Lennox-Gastaut syndrome.
Pediatr Neurol. 1997 Oct 1; 17(3): 203-211.
 
Privitera M
Topiramate: a new antiepileptic drug.
Ann Pharmacother. 1997 Oct 1; 31(10): 1164-1173. Review.
 
Herranz JL
[Topiramate, a new antiepileptic drug].
Rev Neurol. 1997 Aug 1; 25(144): 1221-1225. Review. Spanish.
 
Oka E, et al
Mental deterioration in childhood epilepsy.
Acta Med Okayama. 1997 Aug 1; 51(4): 173-178. Review.
 
Shovic A, et al
'We think your son has Lennox-Gastaut syndrome'--a case study of monosodium glutamate's possible effect on a child.
J Am Diet Assoc. 1997 Jul 1; 97(7): 793-794. No abstract available.
 
Yen DJ, et al
Lamotrigine as add-on therapy in adult patients with refractory epilepsy.
Chung Hua I Hsueh Tsa Chih (Taipei). 1997 May 1; 59(5): 303-307.
 
Li PH, et al
Clinical analysis of 22 infants with afebrile cluster seizures.
Chung Hua Min Kuo Hsiao Erh Ko I Hsueh Hui Tsa Chih. 1997 May 1; 38(3): 203-207.
 
Heiskala H
Community-based study of Lennox-Gastaut syndrome.
Epilepsia. 1997 May 1; 38(5): 526-531.
 
Hornig GW, et al
Left vagus nerve stimulation in children with refractory epilepsy: an update.
South Med J. 1997 May 1; 90(5): 484-488.
 
Farrell K, et al
Prospective, open-label, add-on study of lamotrigine in 56 children with intractable generalized epilepsy.
Pediatr Neurol. 1997 Apr 1; 16(3): 201-205.
 
Rougier A, et al
Callosotomy for intractable epilepsy: overall outcome.
J Neurosurg Sci. 1997 Mar 1; 41(1): 51-57. Review.
 
Casas-Fernandez C
[Experience with Health Quality of Life Questionnaire for the epileptic child].
Rev Neurol. 1997 Mar 1; 25(139): 415-421. Spanish.
 
Armijo JA
[Which drugs should be chosen for the different types of epilepsy]?
Rev Neurol. 1997 Mar 1; 25(139): 356-366. Review. Spanish.
 
Shields WD, et al
Vigabatrin.
Semin Pediatr Neurol. 1997 Mar 1; 4(1): 43-50. Review.
 
Glauser TA
Topiramate.
Semin Pediatr Neurol. 1997 Mar 1; 4(1): 34-42. Review.
 
Bourgeois BF
Felbamate.
Semin Pediatr Neurol. 1997 Mar 1; 4(1): 3-8. Review.
 
Baulac M, et al
[Therapeutic options provided by new antiepileptic drugs].
Rev Neurol (Paris). 1997 Feb 1; 155(1): 21-33. Review. French.
 
Velasco M, et al
Polygraphic characterization of the sleep-epilepsy patterns in a hydranencephalic child with severe generalized seizures of the Lennox-Gastaut syndrome.
Arch Med Res. 1997 Jan 1; 28(2): 297-302.
 
Glauser TA
Preliminary observations on topiramate in pediatric epilepsies.
Epilepsia. 1997 Jan 1; 38 Suppl 1: S37-S41.
 
Donaldson JA, et al
Lamotrigine adjunctive therapy in childhood epileptic encephalopathy (the Lennox Gastaut syndrome).
Epilepsia. 1997 Jan 1; 38(1): 68-73.
 
Arnold ST, et al
Epilepsy in children.
Baillieres Clin Neurol. 1996 Dec 1; 5(4): 783-802. Review.
 
Dulac OJ, et al
Malignant epileptic encephalopathies in children.
Baillieres Clin Neurol. 1996 Dec 1; 5(4): 765-781. Review.
 
Bauer J
Seizure-inducing effects of antiepileptic drugs: a review.
Acta Neurol Scand. 1996 Dec 1; 94(6): 367-377. Review.
 
Tartara A, et al
Efficacy and safety of topiramate in refractory epilepsy: a long-term prospective trial.
Ital J Neurol Sci. 1996 Dec 1; 17(6): 429-432.
 
Avanzini G, et al
Felbamate in therapy-resistant epilepsy: an Italian experience. Felbamate Italian Study Group.
Epilepsy Res. 1996 Nov 1; 25(3): 249-255.
 
Brodie MJ
Lamotrigine--an update.
Can J Neurol Sci. 1996 Nov 1; 23(4): S6-S9.
 
Czochanska J, et al
Intractable epilepsy in children who develop epilepsy in the first decade of life--a prospective study.
Mater Med Pol. 1996 Oct 1; 28(4): 133-137.
 
Lopez-Valdes E, et al
[Treatment of refractory infantile epilepsy with vigabatrin in a series of 55 patients].
Rev Neurol. 1996 Oct 1; 24(134): 1255-1257. Spanish.
PMID: 8983724; UI: 97079884.
 
Wong SH, et al
Totally automated analysis by robotized PrepStation and liquid chromatography: direct-sample analysis of felbamate.
Ther Drug Monit. 1996 Oct 1; 18(5): 573-580.
 
Verrotti A, et al
Early infantile epileptic encephalopathy: a long-term follow-up study.
Childs Nerv Syst. 1996 Sep 1; 12(9): 530-533.
 
Buchanan N
Lamotrigine: clinical experience in 200 patients with epilepsy with follow-up to four years.
Seizure. 1996 Sep 1; 5(3): 209-214.
PMID: 8902923; UI: 97058604.
 
Ferrie CD, et al
Focal abnormalities detected by 18FDG PET in epileptic encephalopathies.
Arch Dis Child. 1996 Aug 1; 75(2): 102-107.
 
Mitsufuji N, et al
Lennox-Gastaut syndrome associated with leukoencephalopathy.
Pediatr Neurol. 1996 Jul 1; 15(1): 63-65.
 
Sidenvall R, et al
Prevalence and characteristics of epilepsy in children in northern Sweden.
Seizure. 1996 Jun 1; 5(2): 139-146.
 
de Romanis F, et al
[New antiepileptic drugs: a review and personal contribution].
Clin Ter. 1996 May 1; 147(5): 217-237. Review. Italian.
 
Minguez-Castellanos A, et al
[Callosotomy in the treatment of drug-resistant epilepsy].
Rev Neurol. 1996 May 1; 24(129): 539-548. Spanish.
 
Duse M, et al
Intravenous immune globulin in the treatment of intractable childhood epilepsy.
Clin Exp Immunol. 1996 May 1; 104 Suppl 1: 71-76. Review.
 
Kwong KL, et al
Neurodevelopmental profile of Down syndrome in Chinese children.
J Paediatr Child Health. 1996 Apr 1; 32(2): 153-157.
 
Kongelbeck SR
Case study. A child with Lennox-Gastaut syndrome.
BZQ. 1996 Apr 1; 3(2): 8-10. Review. No abstract available.
 
Boon P, et al
Epilepsy surgery in Belgium, the Flemish experience.
Acta Neurol Belg. 1996 Mar 1; 96(1): 6-18.
 
Vossler DG
Exacerbation of seizures in Lennox-Gastaut syndrome by gabapentin.
Neurology. 1996 Mar 1; 46(3): 852-853. No abstract available.
 
Hirt HR
[Nosology of Lennox-Gastaut syndrome].
Nervenarzt. 1996 Feb 1; 67(2): 109-122. Review. German.
 
Chakova L
On a rare form of epilepsy in infants--Ohtahara syndrome.
Folia Med (Plovdiv). 1996 Jan 1; 38(2): 69-73.
 
Badalian LO, et al
[Hemimegalocephaly-agyria as the possible cause of the development of the Lennox-Gastaut syndrome].
Zh Nevropatol Psikhiatr Im S S Korsakova. 1996 Jan 1; 96(2): 90-92. Review. Russian. No abstract available.
 
Chamberlain MC
Nitrazepam for refractory infantile spasms and the Lennox-Gastaut syndrome.
J Child Neurol. 1996 Jan 1; 11(1): 31-34.
 
Yagi K
Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study.
Epilepsia. 1996 Jan 1; 37 Suppl 3: 48-51.
 
Oguni H, et al
Long-term prognosis of Lennox-Gastaut syndrome.
Epilepsia. 1996 Jan 1; 37 Suppl 3: 44-47.
 
Tagawa T, et al
[Clinical and electroencephalographic studies in children with hemimegalencephaly].
No To Hattatsu. 1996 Jan 1; 28(1): 53-59. Japanese.

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return to   Symptomatic generalised epilepsies

 

 

(checked: 15 November 2002)
(update 1.3: 22 January 2003)
(issue 1: 12 January 1998)