Elsevier

The Lancet Neurology

Volume 7, Issue 3, March 2008, Pages 278-284
The Lancet Neurology

Grand Round
Refractory, life-threatening status epilepticus in a 3-year-old girl

https://doi.org/10.1016/S1474-4422(08)70043-7Get rights and content

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Case presentation

A 3-year-old girl was admitted for the first time to our hospital because of status epilepticus. The girl was born to healthy, unrelated parents, who had an unremarkable family history, and her birth parameters were within the normal range. The girl had started to walk and had used single words at around 12 months. At 6 months, she had a right hemiclonic seizure 48 hours after being given pentavalent (pertussis, tetanus, diphtheria, inactivated polio, and haemophilus) vaccination. An

Differential diagnosis

During the past decades, diagnostic schemes have been developed for a rational clinical approach to management of patients with epilepsy.1, 2 An epileptic syndrome is a complex of signs and symptoms that define a unique epileptic condition;2 the epileptic syndromes of childhood include several conditions with different electroclinical features and variable outcomes. Although some syndromes are relatively easy to diagnose early in their course, others take time to develop, which hampers an

Treatment

Lamotrigine was slowly withdrawn in the intensive care unit, and the patient improved dramatically over 48 hours: the myoclonus disappeared and the laboratory parameters normalised. Because levetiracetam is well tolerated and has well known antimyoclonic properties, we considered levetiracetam therapy for our patient. The girl was discharged on levetiracetam (1000 mg/day) and phenobarbital (100 mg/day), which controlled her seizures satisfactorily.

SMEI

SMEI is a catastrophic epilepsy that starts during the first year of life and is characterised by early, prolonged febrile seizures followed by afebrile seizures of different types and psychomotor impairment.7, 8 Patients with SMEI can have nearly all seizure types, particularly myoclonic, absence, atonic, and partial seizures.

Simple motor-type partial seizures or complex partial seizures with prominent autonomic symptoms can manifest early and occur in 40–78% of patients, whereas tonic

Conclusions

Status epilepticus is a medical emergency in children, who are at high risk of mortality, particularly when the cause is not known.64, 65 Aggravation of epilepsy by the inappropriate use of antiepileptic drugs is a serious and common problem that is being increasingly recognised. However, it is rarely believed that the inappropriate use of an antiepileptic drug can induce status epilepticus. In patients with SMEI, there is a risk of aggravation with lamotrigine, as shown by the present case.

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References (65)

  • O Kanazawa

    Refractory grand mal seizures with onset during infancy including severe myoclonic epilepsy in infancy

    Brain Dev

    (2001)
  • RF Chin et al.

    Incidence, cause, and short-term outcome of convulsive status epilepticus in childhood: prospective population-based study

    Lancet

    (2006)
  • M Raspall-Chaure et al.

    Outcome of paediatric convulsive status epilepticus: a systematic review

    Lancet Neurol

    (2006)
  • Proposal for revised classification of epilepsies and epileptic syndromes

    Epilepsia

    (1989)
  • J Engel

    Report of International League Against Epilepsy (ILAE). A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology

    Epilepsia

    (2001)
  • C Dravet et al.

    Benign myoclonic epilepsy of infancy

  • R Nabbout et al.

    Epileptic encephalopathies: a brief overview

    J Clin Neurophysiol

    (2003)
  • U Stephani

    The natural history of myoclonic astatic epilepsy (Doose syndrome) and Lennox–Gastaut syndrome

    Epilepsia

    (2006)
  • KE Wisniewski et al.

    Pheno/genotypic correlations of neuronal ceroid lipofuscinoses

    Neurology

    (2001)
  • C Dravet et al.

    Severe myoclonic epilepsy in infancy: Dravet syndrome

  • R Guerrini et al.

    Antiepileptic drug-induced worsening of seizures in children

    Epilepsia

    (1998)
  • J Chaves et al.

    Seizure aggravation in idiopathic generalized epilepsies

    Epilepsia

    (2005)
  • P Thomas et al.

    Absence and myoclonic status epilepticus precipitated by antiepileptic drugs in idiopathic generalized epilepsy

    Brain

    (2006)
  • P Gelisse et al.

    Worsening of seizures by oxcarbazepine in juvenile idiopathic generalized epilepsies

    Epilepsia

    (2004)
  • P Striano et al.

    Life-threatening status epilepticus following gabapentin administration in a patient with benign adult familial myoclonic epilepsy

    Epilepsia

    (2007)
  • V Biton

    Pharmacokinetics, toxicology and safety of lamotrigine in epilepsy

    Expert Opin Drug Metab Toxicol

    (2006)
  • A Crespel et al.

    Lamotrigine associated with exacerbation or de novo myoclonus in idiopathic generalized epilepsies

    Neurology

    (2005)
  • A Biraben et al.

    Exacerbation of juvenile myoclonic epilepsy with lamotrigine

    Neurology

    (2000)
  • P Genton et al.

    Lack of efficacy and potential aggravation of myoclonus with lamotrigine in Unverricht-Lundborg disease

    Epilepsia

    (2006)
  • R Guerrini et al.

    Lamotrigine and seizure aggravation in severe myoclonic epilepsy

    Epilepsia

    (1998)
  • WA Catterall

    A 3D view of sodium channels

    Nature

    (2001)
  • D Higgins et al.

    CLUSTAL W: improving the sensitivity of progressive multiple sequence alignment through sequence weighting, position-specific gap penalties and weight matrix choice

    Nucleic Acids Research

    (1994)
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