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Abstract
Background: Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy characterised by various types of generalised seizures, a noticeable deceleration in intellectual development, and a distinct EEG pattern. Those afflicted with LGS may have previously experienced infantile spasms or an underlying brain condition, though in some cases, the exact cause may be unknown (idiopathic). LGS seizures frequently resist conventional treatment, and the long-term prognosis is poor. Case Presentation: We present two Lennox-Gastaut syndrome cases. The first is a 3-year-old female who was diagnosed with brachycephaly at 3-months-old. Her initial EEG at 4-months-old revealed moderate right frontopolar hypofunction then a month later she underwent physiotherapy, and was prescribed valproic acid. At 2-year- old, the MRI displayed bilateral parieto-occipital cerebral atrophy and the EEG showed general slow-wave abnormalities in the left frontal region then the levetiracetam was added for her treatment. At the age of 2.5- year-old, levetiracetam was substituted by clobazam because the seizure didn’t improve so that the patient got combination therapy of valproic acid and clobazam, and then the seizure decreased after the substitution therapy. The second case is a 3-year-old girl who was diagnosed with hypocalcaemia at the age of 2 weeks. Three months later, she was diagnosed with epilepsy and the brain CT scan showed encephalomalacia, microcephaly and cerebral atrophy. At the age of two she was eventually diagnosed with Lennox-Gastaut syndrome with a particular EEG showing general slow-wave abnormalities. Initially she was treated with valproic acid and phenobarbital. Since there was no improvement of the seizure frequency, levetiracetam was added and phenobarbital was substituted by clobazam, which fortunately cause better condition.
Discussion: The cases highlight the challenges of managing LGS, which tends to be drug-resistant and associated with developmental delays and cognitive impairment. They underscore the need for a multidisciplinary approach to optimize care and enhance the patient's quality of life. This report serves as a reminder of the complexity of LGS and the importance of customizing treatment strategies to meet the unique needs of each patient.
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References
- Shields WD. Diagnosis of Infantile Spasms, Lennox –Gastaut Syndrome and Progressive Myoclonic Epilepsy. Epilepsia 2004; 45:2-4
- Amrutkar CV, Riel-Romero RM. Lennox Gastaut Syndrome. In: StatPearls. Treasure Island (FL): StatPearls Publishing
- Berg AT, Nickels K, Wirrell EC, Geerts AT, Callenbach PM, Arts WF, Rios C, Camfield PR, Camfield CS. Mortality risks in new- onset childhood epilepsy. Pediatrics. 2013 Jul;132(1):124-31.
- Markland ON. Lennox Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol.2003;20:426-441
- Leary PM, Riordan G, Schlegel B, Morris S: Childhood secondary (symptomatic) epilepsy, seizure control, and intellectual handicap in a nontropical region of South Africa. Epilepsia. 1999; 40(8):1110-3.
- Asadi-Pooya AA, Sharifzade M. Lennox- Gastaut syndrome in south Iran: electro- clinical manifestations. Seizure. 2012 Dec;21(10):760-3.
- Trevathan E. Infantile Spasms and Lennox- Gastaut Syndrome. J Child Neurol. 2002; 17:9–22.
- Gastaut H, Roger J, Soulayrol R, Tassinari CA, Regis H, Dravet C. Childhood epileptic encephalopathy of children with diffuse slow spike-waves (otherwise known as "petit mal variant") or Lennox syndrome. Epilepsia. 1966; 7:139–179.
- Hirt HR. Nosology of Lennos Gastaut syndrome. Nevernazt;1996 Feb 67(2);109-22
- Hancock E, Cross H. Treatment of Lennox- Gastaut syndrome. Cochrane Database Syst Rev. 2003;(3).
- Karla V, Gulati S, Pandey RM et al. West syndrome and other Infantile epileptic encephaloparthy-Indian Hospital experience. Brain Dev 2002;24:130-139
- Leary PM, Riordan G, Schlegel B, Morris S. Childhood secondary (symptomatic) epilepsy, seizure control, and intellectual handicap in a nontropical region of South Africa. Epilepsia. 1999; 40:1110–1113.
- Schmidt D, Bourgeois B. A Risk-Benefi t Assessment of Therapies for Lennox-Gastaut Syndrome. Drug Saf. 2000; 22:467–477
- Hancock E, Cross H. Treatment of Lennox- Gastaut syndrome. Cochrane Database of Systematic Reviews 2003, Issue 3.
- Timmings PL, Richens A. Lamotrigine as add on drug in the management of Lennox- Gastaut syndrome. Eur Neurol 1992; 32:305-
- Ritter FJ, Glauser TA, Sachdeo RC, Shu- Chen W. Long-term experience with topiramate in Lennox-Gastaut syndrome. Epilepsia 1998; 39:2-3.
- McLachlan RS. Vagus nerve stimulation for intractable epilepsy: a review. J Clin Neurophysiol 1997; 14:358-368.
- Sorenson JM, Wheless JW, Baumgartner JE, Thomas AB, Brookshire BL, Clifton GL, et al. Corpus callosotomy for medically intractable seizures. Pediatr Neurosurg 1997; 27:260-267.
- Velasco AL, Velasco F, Jiménez, F, Velasco M, Castro G, Carrillo-Ruiz JD, Fanghänel G, Boleaga B. Neuromodulation of the centromedian thalamic nuclei in the treatment of generalized seizures and the improvement of the quality of life in patients with Lennox-Gastaut syndrome. Epilepsia. 2006; 47:1203–1212.
- Shrestha, M., Shrestha, L., & Shrestha, P. S. (2010). Lennox Gastaut Syndrome: A Case Report2Journal of Nepal Paediatric Society, 30(1)1
- Abu Saleh, T., & Stephen, L. (2008). Lennox gastaut syndrome, review of the literature and a case report. Head & face medicine, 4, 9. https://doi.org/10.1186/1746-160X-4-9
References
Shields WD. Diagnosis of Infantile Spasms, Lennox –Gastaut Syndrome and Progressive Myoclonic Epilepsy. Epilepsia 2004; 45:2-4
Amrutkar CV, Riel-Romero RM. Lennox Gastaut Syndrome. In: StatPearls. Treasure Island (FL): StatPearls Publishing
Berg AT, Nickels K, Wirrell EC, Geerts AT, Callenbach PM, Arts WF, Rios C, Camfield PR, Camfield CS. Mortality risks in new- onset childhood epilepsy. Pediatrics. 2013 Jul;132(1):124-31.
Markland ON. Lennox Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol.2003;20:426-441
Leary PM, Riordan G, Schlegel B, Morris S: Childhood secondary (symptomatic) epilepsy, seizure control, and intellectual handicap in a nontropical region of South Africa. Epilepsia. 1999; 40(8):1110-3.
Asadi-Pooya AA, Sharifzade M. Lennox- Gastaut syndrome in south Iran: electro- clinical manifestations. Seizure. 2012 Dec;21(10):760-3.
Trevathan E. Infantile Spasms and Lennox- Gastaut Syndrome. J Child Neurol. 2002; 17:9–22.
Gastaut H, Roger J, Soulayrol R, Tassinari CA, Regis H, Dravet C. Childhood epileptic encephalopathy of children with diffuse slow spike-waves (otherwise known as "petit mal variant") or Lennox syndrome. Epilepsia. 1966; 7:139–179.
Hirt HR. Nosology of Lennos Gastaut syndrome. Nevernazt;1996 Feb 67(2);109-22
Hancock E, Cross H. Treatment of Lennox- Gastaut syndrome. Cochrane Database Syst Rev. 2003;(3).
Karla V, Gulati S, Pandey RM et al. West syndrome and other Infantile epileptic encephaloparthy-Indian Hospital experience. Brain Dev 2002;24:130-139
Leary PM, Riordan G, Schlegel B, Morris S. Childhood secondary (symptomatic) epilepsy, seizure control, and intellectual handicap in a nontropical region of South Africa. Epilepsia. 1999; 40:1110–1113.
Schmidt D, Bourgeois B. A Risk-Benefi t Assessment of Therapies for Lennox-Gastaut Syndrome. Drug Saf. 2000; 22:467–477
Hancock E, Cross H. Treatment of Lennox- Gastaut syndrome. Cochrane Database of Systematic Reviews 2003, Issue 3.
Timmings PL, Richens A. Lamotrigine as add on drug in the management of Lennox- Gastaut syndrome. Eur Neurol 1992; 32:305-
Ritter FJ, Glauser TA, Sachdeo RC, Shu- Chen W. Long-term experience with topiramate in Lennox-Gastaut syndrome. Epilepsia 1998; 39:2-3.
McLachlan RS. Vagus nerve stimulation for intractable epilepsy: a review. J Clin Neurophysiol 1997; 14:358-368.
Sorenson JM, Wheless JW, Baumgartner JE, Thomas AB, Brookshire BL, Clifton GL, et al. Corpus callosotomy for medically intractable seizures. Pediatr Neurosurg 1997; 27:260-267.
Velasco AL, Velasco F, Jiménez, F, Velasco M, Castro G, Carrillo-Ruiz JD, Fanghänel G, Boleaga B. Neuromodulation of the centromedian thalamic nuclei in the treatment of generalized seizures and the improvement of the quality of life in patients with Lennox-Gastaut syndrome. Epilepsia. 2006; 47:1203–1212.
Shrestha, M., Shrestha, L., & Shrestha, P. S. (2010). Lennox Gastaut Syndrome: A Case Report2Journal of Nepal Paediatric Society, 30(1)1
Abu Saleh, T., & Stephen, L. (2008). Lennox gastaut syndrome, review of the literature and a case report. Head & face medicine, 4, 9. https://doi.org/10.1186/1746-160X-4-9