Background Severe laryngospasm, as a presenting symptom of epilepsy in children, is very uncommon and often misdiagnosed. To increase awareness, we present a literature review. The incentive was a 22 month old girl who presented with life threatening laryngospasm. The choking episodes progressed rapidly to frequent unprovoked episodes day and night; associated with severe oxygen desaturation, whist being fully aware and requiring paediatric intensive care interventions. Initially, investigations did not confirm a diagnosis of epilepsy. Repeat video EEG finally demonstrated cortical seizures, with focality over the right posterior temporal region. To achieve seizure control, high doses of carbamazepine and levetiracetam were necessary. Interestingly, the patient shares neurological features with family members, suggesting a genetic link.

Methods and results A literature review shows that the extent of epileptic laryngospasm in this case report has not previously been described amongst only 9 related case reports. Reviews concerning choking episodes during sleep debate a diagnostic challenge that stems from the frequent absence of recorded epileptiform activity, often causing a delay in adequate seizure treatment. Two recent reports on severe neonatal episodic laryngospasm describe a new phenotype of seizure disorder related to sodium channelopathy. In these patients, high dose carbamazepine was necessary to achieve seizure control.

Conclusions Despite being a rare phenomenon, severe laryngospasm in children should be suspicious as a symptom of epilepsy, even without EEG correlation. Treatment should no be postponed, while excluding other aetiology. Carbamazepine may be effective, however high doses may be necessary to achieve seizure control.