West syndrome - Diagnostic dilemma in a month old infant without typical electroencephalographic features: A case report

Authors

  • Kiran Subedi Registrar, International Friendship Children’s Hospital, Kathmandu, Nepal.
  • Rydam Basnet Lecturer, Department of Paediatrics, Kathmandu Medical College Teaching Hospital, Kathmandu, Nepal.
  • Bikash Bhandari Lecturer, Department of Paediatrics, Devdaha Medical College and Research Institute, Rupandehi, Nepal.
  • Sonsy Gongal Lecturer, Department of Paediatrics, KIST Medical College, Lalitpur, Nepal.
  • Ashik Rajak Intern Doctor, Department of Paediatrics, Kathmandu Medical College Teaching Hospital, Kathmandu, Nepal. https://orcid.org/0000-0002-2219-3427

DOI:

https://doi.org/10.3126/jkmc.v8i2.28175

Keywords:

Hypsarrhythmia; Infantile spasms; West syndrome.

Abstract

Infantile spasm constitutes 2% of childhood epilepsies but 25% of epilepsy with onset in the first year of life. The rate of infantile spasm is estimated to be 2.5-6.0 cases per 10,000 live births. Its prevalence rate is 1.5-2.0 cases per 10,000 children aged 10 years or younger. Ninety percent of infantile spasms begin in infants younger than 12 months with a peak onset at age 4-6 months.  We report a case of 45 days old male child who had onset of flexor spasms since one month of age which is an unusual age of onset of West syndrome. The symptoms were initially thought as a simple startle response by the mother. This is a rare case because of its age of onset and lack of typical electroencephalographic features. Although the electroencephalography showed abnormal pattern suggesting seizure disorder, the frequently encountered pattern of hypsarrhythmia seen in children with infantile spasm was not seen in this case. It was difficult to assess developmental regression in a month old child who in subsequent visits had some degree of developmental delay. Hence fulfilling two of the three criterias for the diagnosis.

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Author Biography

Kiran Subedi, Registrar, International Friendship Children’s Hospital, Kathmandu, Nepal.

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Published

2019-06-30

How to Cite

Subedi, K., Basnet, R., Bhandari, B., Gongal, S., & Rajak, A. (2019). West syndrome - Diagnostic dilemma in a month old infant without typical electroencephalographic features: A case report. Journal of Kathmandu Medical College, 8(2), 116–117. https://doi.org/10.3126/jkmc.v8i2.28175

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Section

Case Reports

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