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

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An Erratum to this article was published on 28 March 2021

This article has been updated

Abstract

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.

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Acknowledgment

Dr Sushma Goyal and Dr Chaitanya Datar for helpful inputs in the EEG and genetic testing sections, respectively.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India

    Suvasini Sharma

  2. Department of Pediatrics, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India

    Jaya Shankar Kaushik

  3. Department of Pediatrics, Bharati Vidyapeeth Deemed University Medical College, Pune, Maharasthra, India

    Kavita Srivastava

  4. Department of Pediatrics, Army Hospital — Research and Referral, New Delhi, India

    Jyotindra Narayan Goswami

  5. Division of Pediatric Neurology, Advanced Centre of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Jitendra Kumar Sahu

  6. Department of Pediatric Neurology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

    Kollencheri Puthenveettil Vinayan

  7. Department of Pediatric Neurology, Madhukar Rainbow Children’s Hospital, Delhi, India

    Rekha Mittal

  8. Department of Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children Hospital, New Delhi, India

    Suvasini Sharma

Authors
  1. Suvasini Sharma
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  2. Jaya Shankar Kaushik
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  3. Kavita Srivastava
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  4. Jyotindra Narayan Goswami
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Consortia

AOCN-IES Expert Committee

Corresponding author

Correspondence to Suvasini Sharma.

Additional information

Contributors

SS and RM: conceptualized the idea; SS, JSK, KS, JS, JNG, RM, KPV: constituted the writing committee and drafted the manuscript; JSK devised and conducted Delphi process. All the authors approved the final version of the manuscript.

Funding

The logistics of faculty travel for the West syndrome Delphi Consensus meeting was funded by Intas Pharmaceutical Ltd. and Ferring Pharmaceuticals. The venue support was provided by Army Hospital, Research and Referral, Dhaula Kuan, New Delhi. None of the funding sources had any role in content of the discussion during meeting, or in report or manuscript preparation. They did not have access to any version of the manuscript.

Competing interests

None stated.

Association of Child Neurology — Indian Epilepsy Society Expert Committee

Razia Adam (Hyderabad, Telengana); Satinder Aneja (Noida, Uttar Pradesh); Biswaroop Chakrabarty (Delhi); Arijit Chattopadhyay (Kolkata); Saurabh Chopra (Delhi), Rajni Farmania (Delhi); Pradnya Gadgil (Mumbai, Maharashtra); Divyani Garg (Delhi); Jatinder Singh Goraya (Ludhiana, Punjab); Sheffali Gulati (Delhi); Aparajita Gupta (Kolkata, West Bengal); Rachana Dubey Gupta (Indore, Madhya Pradesh); Vineet Bhushan Gupta (Delhi); Anaita Udwadia Hedge (Mumbai, Maharashtra); Mary Iype (Trivandrum, Kerala); Vivek Jain (Jaipur, Rajasthan); Prashant Jauhari (Delhi); Veena Kalra (Delhi); Mahesh Kamate (Belgavi, Karnataka); Lakshminarayanan Kannan (Chennai, Tamil Nadu); Anupriya Kaur (Chandigarh); Gurpreet Kochar (Ludhiana, Punjab); Ramesh Konanki (Hyderabad, Telangana); Ajay Kumar (Patna, Bihar); PAM Kunju (Trivandrum, Kerala); Lokesh Lingappa (Hyderabad, Telengana); Priyanka Madaan (Chandigarh); Ranjith Kumar Manokaran (Chennai, Tamil Nadu); MM Mehendiratta (Delhi); Ramshekhar Menon (Trivandrum, Kerala); Devendra Mishra (Delhi); Debasis Panigrahi (Bhubneshwar, Orissa); Harsh Patel (Ahmedabad, Gujarat); Sandeep Patil (Pune, Maharashtra); Bijoy Patra (Delhi); Leema Pauline (Chennai, Tamil Nadu); KVN Raju (Bangalore, Karnataka); Arushi Gehlot Saini (Chandigarh); Lokesh Saini (Chandigarh); Naveen Sankhyan (Chandigarh); Rachna Sehgal (Delhi); Anita Sharma (Delhi); Pratibha Singhi (Gurgaon, Haryana); Vishal Sondhi (Pune, Maharasthra); Renu Suthar (Chandigarh); Sanjeev V Thomas (Trivandrum, Kerala); Manjari Tripathi (Delhi); Vrajesh Udani (Mumbai, Maharasthra); Prashant Utage (Hyderabad, Telengana); Nitish Vora (Ahmedabad, Gujarat); Sangeeta Yoganathan (Vellore, Tamil Nadu).

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Sharma, S., Kaushik, J.S., Srivastava, K. et al. Association of Child Neurology (AOCN) — Indian Epilepsy Society (IES) Consensus Guidelines for the Diagnosis and Management of West Syndrome. Indian Pediatr 58, 54–66 (2021). https://doi.org/10.1007/s13312-021-2097-6

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