Infantile Epileptic Spasms Syndrome (IESS)
Conditions
Interventions
Study setting
The study will be conducted across eight tertiary care hospitals with paediatric neurology units in Sri Lanka. These include the Lady Ridgeway Children’s Hospital (3 units), Colombo No
Sponsors
University of Colombo
Eligibility
Inclusion criteria
Inclusion criteria: 1) male and female infants between ages of 3 to 24 months. 2) Newly confirmed diagnosis of infantile spasms made by the referring paediatrician/ paediatric neurologist based on the diagnostic criteria outlined in the recent ILAE classification and definition of epilepsy syndrome with onset in neonates and infants in 2022. 3) Hypsarrhythmia recorded on pre-treatment EEG. This will be those who show a BASED score of 4 or 5 in a standard EEG performed.
Exclusion criteria
Exclusion criteria: 1) Infants with tuberous sclerosis complex^1 2) Ever treated previously for infantile spasms with steroids or other anticonvulsants^2 3) Infants already on steroids or ACTH for any other illness 4) Contraindication for the use of high dose steroids such as underlying infection, immune deficiency, hypertension etc. 5) Children in critical conditions^3 such as severe infections, congenital heart disease or requiring ventilation or care in an ICU 6) Not accompanied by parent/s or parent’s inability to complete follow up^4 ^1 A diagnosis or high risk of tuberous sclerosis. (known affected parent, previously diagnosed cardiac rhabdomyoma, hypomelanic macules, forehead fibrous plaque, shagreen patch, retinal phakoma or known polycystic kidneys). ^2 Previous treatments for infantile spasms including a therapeutic trial of pyridoxine to exclude pyridoxine dependent seizures. Note - previous treatment for other seizure types is not a reason for exclusion. ^3 A lethal or potentially lethal condition, other than infantile spasms, with a risk of death before 18 months of age. ^4 Doubt about the ability of the parents or legal guardians to know when the spasms stop. Important: infants on anticonvulsants therapy (other than steroids) given for treatment of other forms of seizures i.e. neonatal seizures or focal seizures will not be excluded
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Compare two regimes of oral prednisolone (7 days vs. 14 days) in the control of epileptic spasms and electroclinical remission. Control of spasms: Absence of any recognizable spasms for at least 24 hours, as reported by parents using a spasm diary (the spasm frequency will be recorded by the parent in the spasm diary for the first 28 days after the commencement of the treatment). Electroclinical remission: Disappearance of hypsarrhythmia on EEG (the BASED score improving to 3 or less). This will be assessed on day 14 and day 28 by recording a 30-minute sleep EEG followed by 5 minutes of wakefulness. The 2021 BASED (Burden of AmplitudeS and Epileptiform Discharges) score is an EEG grading scale used to assess the severity of epileptic activity [At Day 14 and Day 28 from the commencement of therapy.] | — |
Secondary
| Measure | Time frame |
|---|---|
| Compare the efficacy between 7-day oral prednisolone and 14-day oral prednisolone in the control of IESS, over a longer period of follow up at 42 days, 3 months, 6 months and 12 months. The absence of any visible spasms for at least 72 hours, as reported by parents using a spasm diary, on the day of assessment. [At 42 days, 3 months, 6 months, and 12 months. ] Compare the efficacy between intervention arm with protracted taper and the standard arm with regular taper on relapse rate. Metrics/Method of Measurement: The reappearance of clinical spasms and EEG findings consistent with hypsarrhythmia (BASED score = 4) is considered as a relapse. The number of children who relapse after initial spasm control, the duration of spasm control up to first relapse and the relapse rate of spasms over the first 12 months will be measured. [ During the first 12 months of therapy.] Compare the developmental outcomes at 24 months following steroid therapy and examine the influence of spasm control on developmental outcomes. Metrics/Method of Measurement: The development will be assessed using the IIIrd Bayley Infant and Toddler Developmental Scale. [ 24 months post- treatment.] Compare the evolution of epilepsy syndromes including non-spasm types. Metrics/Method of Measurement: The identification of the occurrence of two or more unprovoked seizures other than the spasms which the child has already experienced will be made through a direct interview with the parent. [ 12 months post-treatment ] | — |
Countries
Sri Lanka
Contacts
Public ContactProfessor Jithangi Wanigasinghe
Professor in Paediatric Neurology
Outcome results
None listed