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Case Report
A two-year-old male child with Doose syndrome: An unusual entity
1 Department of Paediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of Paediatrics, All India Institute of Medical Sciences, Gorakhpur, Uttar Pradesh, India
Address correspondence to:
Swathi Chacham
Department of Paediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand,
India
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Article ID: 100077Z06AS2020
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Singh A, Prabha R, Ahmad N, Chacham S, Mohan K, Gaurav K, Bhat N, Verma PK, Kumar M, Panda PK, Sharawat IK, Prakash B. A two-year-old male child with Doose syndrome: An unusual entity. Case Rep Int 2020;9:100077Z06AS2020.ABSTRACT
Introduction: Myoclonic-astatic epilepsy of early childhood (MAE)/Doose syndrome (DS) is an infrequent entity causing myoclonic-astatic seizures.
Case Report: A 2-year-old male child presenting with history of seizures from one year, inability to speak and choreiform movements. He had isolated speech delay with other quadrants being normal (speaks monosyllables at two years). Parents noticed brief jerky movements of both upper and lower limbs with increasing intensity over 2–3 months which became a daily occurrence. Also, the child had episodes of abrupt loss of contact with surroundings and prolonged staring (3–5 minutes) and few eye blinks. Subsequently, by 1.5 years, he started having episodes of sudden forward bending of head followed by frequent falls. Jerks either preceded or followed falls. Decreased attention and hyperactivity had started by one year of the illness. Electroencephalography depicted polysharp waves with maximum voltage of 180 μV and 3–4 Hz@ an interval of 20–30 seconds bilaterally. He responded to oral sodium valproate, levetiracetam, and ketogenic diet.
Conclusion: We report a 2-year-old male child with Doose syndrome, a distinct yet rare entity.
Keywords: Astatic seizures, Atonic seizures, Electroencephalographic correlate, Myoclonic-astatic epilepsy, Myoclonic-atonic seizures, Myoclonic seizures
SUPPORTING INFORMATION
Author Contributions
Rashmie Prabha - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Avinish Singh - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Najeeb Ahmad - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Swathi Chacham - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Kriti Mohan - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Kumar Gaurav - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Nowneet Bhat - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Prashant Kumar Verma - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Manish Kumar - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Prateek Kumar Panda - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Indar Kumar Sharawat - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Brahm Prakash - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2020 Avinish Singh et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
