# Clinical features of Infantile Epileptic Spasms Syndrome: a systematic review

**Authors:** Xiao Meng, Danielle S. Takacs, Yashaswini Kelagere, Zihan Zhang, Sanjanaa Seshadri, Jiyeon Cha, Ming Li, Lei-Shih Chen

PMC · DOI: 10.1186/s13023-026-04229-1 · Orphanet Journal of Rare Diseases · 2026-02-02

## TL;DR

This study reviews clinical features of Infantile Epileptic Spasms Syndrome to help doctors identify and refer cases more effectively.

## Contribution

The study provides a comprehensive list of clinical features of IESS based on a systematic review of 140 studies.

## Key findings

- 25 clinical features were identified among 3,786 children with IESS.
- No single feature was reported in over half of the children.
- Flexor spasms were the most commonly reported feature at 48.28%.

## Abstract

Infantile Epileptic Spasms Syndrome (IESS) is a severe epilepsy in children between one month and two years of age. Urgent referral of possible IESS cases from pediatricians and family physicians who care for children to neurologists for diagnosis and treatment has been shown to significantly improve patient health outcomes. Yet, a comprehensive list of IESS clinical features is lacking. We conducted a systematic review of studies to report IESS clinical features.

Articles from the MEDLINE Complete and Embase databases were searched from the earliest years of databases availability (1979) to July 31, 2024. We followed the Preferred Reporting for Systematic Reviews and Meta-Analysis guidelines to conduct this systematic review with 140 articles, all of which were descriptive studies.

We identified 25 clinical features among the 3,786 children with IESS who were included in 140 studies. No single clinical descriptor was reported in over half of the children. The six most commonly reported clinical features included flexor spasms (48.28%), mixed spasms (30.19%), extensor spasms (10.49%), symmetric spasms (8.49%), asymmetric/focal spasms (6.50%), and head nodding (2.48%).

There were a wide variety of IESS presentation descriptions. This review will help future researchers to develop an IESS screening tool that can assist pediatricians and family physicians who care for children in making urgent referrals.

The online version contains supplementary material available at 10.1186/s13023-026-04229-1.

## Linked entities

- **Diseases:** Infantile Epileptic Spasms Syndrome (MONDO:0018097)

## Full-text entities

- **Diseases:** Epileptic Spasms Syndrome (MESH:D013035)

## Full text

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## Figures

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## References

3 references — full list in the complete paper: https://tomesphere.com/paper/PMC12955028/full.md

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Source: https://tomesphere.com/paper/PMC12955028