Epilepsy Syndromes
Description Medicine treatment Referral G40.3-5 DESCRIPTION An epilepsy syndrome is a specific seizure type associated with characteristic EEG and imaging features, natural history, age-dependent features, triggers, response to ASM, and prognosis. For classification of seizure types, see Figure 1 in Epileptic seizures . Pointers for the identification of an epilepsy syndrome: Early-onset se
G40.3-5
DESCRIPTION
An epilepsy syndrome is a specific seizure type associated with characteristic EEG and imaging features, natural history, age-dependent features, triggers, response to ASM, and prognosis. For classification of seizure types, see Figure 1 in Epileptic seizures.
Pointers for the identification of an epilepsy syndrome:
- Early-onset seizures (<1 year old)
- Neuroregression (loss of previously acquired milestones or skills)
- Global developmental delay
- Intellectual disability
- Specific seizure types
- Drug-resistant epilepsy
- Associated neurological symptoms, e.g. facial or limb weakness, ataxia, movement disorders
- Strong family history of epilepsy
- Associated neurocutaneous lesions
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Figure 1: International League Against Epilepsy classification of seizure types
(Taken From: Scheffer IE, Berkovic S, Capovilla G, Connolly MB, French J, Guilhoto L. ILAE classification of epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017, 58 (4): 512-521.)
Table 1: Epilepsy syndromes
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Note:
West syndrome (IESS), Dravet syndrome, myoclonic atonic epilepsy and Lennox-Gastaut syndrome are regarded as developmental and epileptic encephalopathies (DEEs) and are associated with neuroregression/neuroplateauing, and behavioural problems.
If valproate is used, acknowledgment of risk must be obtained annually, even if not of child-bearing potential. https://www.sahpra.org.za/wp-content/uploads/2025/05/GLF-CEM-PV-S01_v2-Valproate-Annual-Risk-Acknowledgement-FormARF.pdf
Girls aged 10 years and older should receive effective family planning and consider a long-acting form of contraception.
MEDICINE TREATMENT
Discuss initiation of ASM with specialist.
REFERRAL
All suspected cases of epilepsy syndromes.
Click here to access NEMLC Report.
Standard Treatment Guidelines and Essential Medicines List for South Africa, Paediatric Hospital Level_Updated May 2025
Chapter 13: The Nervous System_13.4 Epilepsy_13.4.1 Epilepsy in Children <13 Years of Age_13.4.1.1 Epilepsy Syndromes