8433711141 , 8850517344, 8169558281

Poorly Controlled seizure – unusual presentation of long QT sydrome

Poorly Controlled seizure – unusual presentation of long QT sydrome
  • Seizure disorder are frequent paediatric age group problem.
  • They are result of abnormal neuronal activity.
  • Most of them are controlled with anti-epileptic.
  • However, a few mimics of seizure are easy to miss and potentially fatal.

Seizure disorder

  • 3yr girl
  • Recurrent episodes up rolling of eyeballs with loss of consciousness
  • Short lasting episodes with spontaneous recovery
  • A few times tonic posturing
  • Precipitated by excitement, physical activity
  • Poorly controlled on various medications with appropriate dosage single and combination : valproic acid, carbamazepin, levetiracetam etc
  • EEG: no abnormal activity
  • CT brain: with in normal limits
  • BERA: e/o hearing impairment

ECG

Arrow: Poly morphic ventricular tachycardia, Tick: sinus rhythm, Note
long QT
Arrow: Polymorphic VT spontaneously terminate
into sinus rhythm with ectopic , T wave alternance
Arrow: Sinus rhythm degenerate into polymorphic VT, T
wave alternance
Sinus rhythm, PR 130ms, QT interval: 480ms, QT corrected
( QTc= 610ms), T wave inversion

Therapy for Long QT

  • Patient was started on Propranolol 1mg/kg/dose BID
  • There was no recurrence of episodes on medication.
  • Other options include cervical sympathectomy, AICD, pacemaker etc.

How can long QT present at seizure?

  • Patients with long QT have runs as polymorphic ventricular tachycardia.
  • During VT, heart is not able to pump blood and leads to cerebral anoxia.
  • This can manifest as seizure or syncope.
  • As VT terminates spontaneously or with therapy, circulation is restored and consciousness is regained.

What is the risk with polymorphic VT?

  • Any episode of polymorphic VT can degenerate into ventricular fibrillation and lead to cardiac arrest and sudden death.
  • Long QT has prevalence of 1 per 2000to 4000.
  • Rare disease. Many varieties.
  • Sudden death and syncope are common presentation

Factors suggesting alternate etiology for seizure in this case

  • EEG: Normal
  • Poorly controlled episodes on medication
  • Brain imaging normal
  • Hearing loss ( associated with Long QT)

Take home message

  • Rule out alternate cause of seizure in case of abnormal presentation.
  • 12 lead ECG and QTc reported by a cardiologist is strongly recommended.
  • Long QT is potentially fatal condition
  • Prevention of VT with medication is the treatment.
  • Beta blockers are effective therapy.
Share

REPLY COMMENT

© 2021 sujyotheartclinic.com