Three Patients of the Early Onset Epileptic Spasms without Hypsarrhythmia

Abstract

Epileptic spasms without hypsarrhythmia occur when patients do not display hypsarrhythmia on electroencephalogram (EEG) at the onset and throughout the clinical course. We report three patients of epileptic spasms in patients with early onset, all of whom experienced other types of seizures.

We detail three patients (two boys and one girl) of epileptic spasms without hypsarrhythmia, occurring between 1 and 3 months of age, with no abnormalities detected on neurometabolic analysis and brain magnetic resonance imaging. Long-term video-EEG monitoring revealed epileptic spasms with focal onset seizures in two patients, and epileptic spasms followed by generalized tonic-clonic seizures in one patient. Hypsarrhythmia was never observed in repeated EEG examinations. Two patients achieved seizure freedom and improved development through treatment with topiramate alone or in combination with valproate, without requiring hormonal therapies or vigabatrin. The remaining patient achieved seizure freedom following administration of antiseizure medications, including topiramate, after a trial of adrenocorticotropic hormone therapy.

We report the cases of three patients with early onset epileptic spasms without hypsarrhythmia. All patients achieved seizure freedom after topiramate treatment. Topiramate may be considered as a relatively effective antiseizure medication for early onset epileptic spasms without hypsarrhythmia.

Keywords

epileptic spasms without hypsarrhythmia - early onset - topiramate - focal onset seizure

Publication History

Received: 23 January 2024

Accepted: 01 April 2024

Accepted Manuscript online:
02 April 2024

Article published online:
24 April 2024

• References

• 1 Pavone P, Striano P, Falsaperla R, Pavone L, Ruggieri M. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013. Brain Dev 2014; 36 (09) 739-751
• 2 Riikonen R. Epidemiological data of West syndrome in Finland. Brain Dev 2001; 23 (07) 539-541
• 3 El Sabbagh S, Lebre AS, Bahi-Buisson N. et al. Epileptic phenotypes in children with respiratory chain disorders. Epilepsia 2010; 51 (07) 1225-1235
• 4 Mei D, Marini C, Novara F. et al. Xp22.3 genomic deletions involving the CDKL5 gene in girls with early onset epileptic encephalopathy. Epilepsia 2010; 51 (04) 647-654
• 5 Demarest ST, Shellhaas RA, Gaillard WD. et al; Pediatric Epilepsy Research Consortium. The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium. Epilepsia 2017; 58 (12) 2098-2103
• 6 Caraballo RH, Fejerman N, Bernardina BD. et al. Epileptic spasms in clusters without hypsarrhythmia in infancy. Epileptic Disord 2003; 5 (02) 109-113
• 7 Oguni H, Funatsuka M, Sasaki K. et al. Effect of ACTH therapy for epileptic spasms without hypsarrhythmia. Epilepsia 2005; 46 (05) 709-715
• 8 Wirrell EC, Shellhaas RA, Joshi C, Keator C, Kumar S, Mitchell WG. Pediatric Epilepsy Research Consortium. How should children with West syndrome be efficiently and accurately investigated? Results from the National Infantile Spasms Consortium. Epilepsia 2015; 56 (04) 617-625
• 9 Koichihara R, Hamano SI, Daida A. et al. Clinical features and electroclinical evolution in 22 cases with epileptic spasms without hypsarrhythmia. Epileptic Disord 2020; 22 (01) 73-82
• 10 Caraballo RH, Ruggieri V, Gonzalez G. et al. Infantile spams without hypsarrhythmia: a study of 16 cases. Seizure 2011; 20 (03) 197-202
• 11 Takahashi Y, Ota A, Tohyama J. et al. Different pharmacoresistance of focal epileptic spasms, generalized epileptic spasms, and generalized epileptic spasms combined with focal seizures. Epilepsia Open 2022; 7 (01) 85-97
• 12 Glauser TA, Clark PO, Strawsburg R. A pilot study of topiramate in the treatment of infantile spasms. Epilepsia 1998; 39 (12) 1324-1328
• 13 Nadig PL, Sahu JK, Suthar R, Saini A, Sankhyan N. Topiramate as an adjunct in the management of West syndrome. Indian J Pediatr 2020; 87 (01) 6-11
• 14 Shank RP, Maryanoff BE. Molecular pharmacodynamics, clinical therapeutics, and pharmacokinetics of topiramate. CNS Neurosci Ther 2008; 14 (02) 120-142
• 15 Ben-Menachem E. Mechanism of action of vigabatrin: correcting misperceptions. Acta Neurol Scand Suppl 2011; 192 (192) 5-15
• 16 Vawter-Lee M, Natarajan N, Rang K, Horn PS, Pardo AC, Thomas CW. Topiramate is safe for refractory neonatal seizures: a multicenter retrospective cohort study of necrotizing enterocolitis risk. Pediatr Neurol 2022; 129: 7-13