Display options
Cite
Dragoumi P, Chivers F, Brady M, et al. Epilepsy with myoclonic-atonic seizures (Doose syndrome): When video-EEG polygraphy holds the key to syndrome diagnosis. Epilepsy Behav Case Rep. 2015;5:31-3doi: 10.1016/j.ebcr.2015.10.001.
Dragoumi, P., Chivers, F., Brady, M., Craft, S., Mushati, D., Venkatachalam, G., Cross, J. H., & Das, K. B. (2016). Epilepsy with myoclonic-atonic seizures (Doose syndrome): When video-EEG polygraphy holds the key to syndrome diagnosis. Epilepsy & behavior case reports, 531-3. https://doi.org/10.1016/j.ebcr.2015.10.001
Dragoumi, Pinelopi, et al. "Epilepsy with myoclonic-atonic seizures (Doose syndrome): When video-EEG polygraphy holds the key to syndrome diagnosis." Epilepsy & behavior case reports vol. 5 (2016): 31-3. doi: https://doi.org/10.1016/j.ebcr.2015.10.001
Dragoumi P, Chivers F, Brady M, Craft S, Mushati D, Venkatachalam G, Cross JH, Das KB. Epilepsy with myoclonic-atonic seizures (Doose syndrome): When video-EEG polygraphy holds the key to syndrome diagnosis. Epilepsy Behav Case Rep. 2015 Oct 28;5:31-3. doi: 10.1016/j.ebcr.2015.10.001. eCollection 2016. PMID: 26958468; PMCID: PMC4773482.
Copy Download .nbib Format: NLM
Share it on

Epilepsy Behav Case Rep. 2015 Oct 28;5:31-3. doi: 10.1016/j.ebcr.2015.10.001. eCollection 2016.

Epilepsy with myoclonic-atonic seizures (Doose syndrome): When video-EEG polygraphy holds the key to syndrome diagnosis.

Epilepsy & behavior case reports

Pinelopi Dragoumi, Fiona Chivers, Megan Brady, Sheila Craft, David Mushati, Gopalakrishnan Venkatachalam, Judith Helen Cross, Krishna B Das

Affiliations

  1. Young Epilepsy, Lingfield, Surrey, UK; Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
  2. Young Epilepsy, Lingfield, Surrey, UK.
  3. Young Epilepsy, Lingfield, Surrey, UK; Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; UCL - Institute of Child Health, London, UK.

PMID: 26958468 PMCID: PMC4773482 DOI: 10.1016/j.ebcr.2015.10.001

Abstract

An electroclinical epilepsy syndrome diagnosis enables physicians to predict outcomes as well as select appropriate treatment options. We report a child who presented with reflex myoclonus at the age of 9 months and was initially diagnosed with myoclonic epilepsy in infancy. After 9 years of medically resistant myoclonic seizures, extensive investigations, and emerging learning difficulties, she was referred for video-telemetry to characterize her seizures in an attempt to make a syndromic diagnosis. A three-day video-telemetry assessment was performed to document seizures. Neck and deltoid EMG channels were applied from the onset of the recording. Frequent generalized bursts of 3- to 5-Hz spike/polyspike and slow wave discharges, associated with clinical manifestations, mostly myoclonic seizures, were noted. In addition, definite atonic components were noted on the neck EMG as well as the deltoids associated with the slow component of the ictal discharges. The EEG and polygraphy findings are suggestive of a generalized epilepsy characterized by predominantly myoclonic seizures with atonic components. This raises the possibility whether a variant of epilepsy with myoclonic-atonic seizures (Doose syndrome) may be the underlying diagnosis for this girl. A trial of the ketogenic diet would therefore be considered as an option in her future management in view of its beneficial effect in this condition.

Keywords: Childhood; Doose syndrome; Myoclonic epilepsy; Myoclonic–astatic epilepsy; Myoclonic–atonic seizures; Video-telemetry

References

  1. Epilepsia. 2013 Sep;54(9):1605-12 - PubMed
  2. Dev Med Child Neurol. 2010 Nov;52(11):988-93 - PubMed
  3. Epileptic Disord. 2003 Jun;5(2):121-2 - PubMed
  4. Brain Nerve. 2011 Apr;63(4):393-400 - PubMed
  5. Seizure. 2012 May;21(4):300-3 - PubMed

Publication Types