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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
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.
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