Association of Child Neurology (AOCN) — Indian Epilepsy Society (IES) Consensus Guidelines for the Diagnosis and Management of West Syndrome

Justification West syndrome is one of the commonest causes of epilepsy in infants and young children and is a significant contributor to neurodevelopmental morbidity. Multiple regimens for treatment are in use. Process An expert group consisting of pediatric neurologists and epileptologists was cons...

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Bibliographic Details
Published in:Indian pediatrics 2021-01, Vol.58 (1), p.54-66
Main Authors: Sharma, Suvasini, Kaushik, Jaya Shankar, Srivastava, Kavita, Goswami, Jyotindra Narayan, Sahu, Jitendra Kumar, Vinayan, Kollencheri Puthenveettil, Mittal, Rekha
Format: Article
Language:English
Subjects:
Maternal and Child Health
Medicine
Medicine & Public Health
Pediatric Surgery
Pediatrics
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Summary:Justification West syndrome is one of the commonest causes of epilepsy in infants and young children and is a significant contributor to neurodevelopmental morbidity. Multiple regimens for treatment are in use. Process An expert group consisting of pediatric neurologists and epileptologists was constituted. Experts were divided into focus groups and had interacted on telephone and e-mail regarding their group recommendations, and developed a consensus. The evidence was reviewed, and for areas where the evidence was not certain, the Delphi consensus method was adopted. The final guidelines were circulated to all experts for approval. Recommendations Diagnosis should be based on clinical recognition (history/home video recordings) of spasms and presence of hypsarrhythmia or its variants on electroencephalography. A magnetic resonance imaging of the brain is the preferred neuroimaging modality. Other investigations such as genetic and metabolic testing should be planned as per clinico-radiological findings. Hormonal therapy (adrenocorticotropic hormone or oral steroids) should be preferred for cases other than tuberous sclerosis complex and vigabatrin should be the first choice for tuberous sclerosis complex. Both ACTH and high dose prednisolone have reasonably similar efficacy and adverse effect profile for West syndrome. The choice depends on the preference of the treating physician and the family, based on factors of cost, availability of infrastructure and personnel for daily intramuscular injections, and monitoring side effects. Second line treatment options include anti-epileptic drugs (vigabatrin, sodium valproate, topiramate, zonisamide, nitrazepam and clobazam), ketogenic diet and epilepsy surgery.
ISSN:0019-6061
0974-7559
DOI:10.1007/s13312-021-2097-6