Panayiotopoulos Syndrome Presenting With Status Epilepticus and Cardiorespiratory Arrest

Abstract

Panayiotopoulos syndrome is early-onset benign childhood epilepsy, now classified as an electroclinical syndrome. The original description in 1989 focused on the triad of nocturnal seizures, tonic eye deviation, and vomiting. With available data from the long-term studies, a wide variety of manifestations have been described, with recognition of autonomic features as being the most prominent aspect of this epilepsy. The presenting symptoms are usually focal seizures comprising autonomic symptoms and/or behavioral changes. Majority of these seizures are in sleep, with half of the seizures progressing to become secondarily generalized. Occasionally, these seizures can present with prominent autonomic features such as ictal vomiting, pallor, flushing/cyanosis, and tachycardia with prolonged thermoregulatory changes lasting for hours, constituting autonomic status epilepticus. Recovery from this autonomic status epilepticus is within hours and is always complete. Autonomic status epilepticus has been previously reported in this epilepsy syndrome, but ictal cardiorespiratory arrest is extremely rare, with only 4 cases being reported in literature. All 4 cases reported in literature recovered spontaneously and did not require resuscitation. Here we present a 3½-year-old male child with Panayiotopoulos syndrome who presented with status epilepticus and ictal cardiorespiratory arrest requiring cardiopulmonary resuscitation for revival.

Panayiotopoulos syndrome with convulsive status epilepticus at the onset: A long-term study

“…18,19 Sanders et al reported a case of a 3-year-old male child with PS who presented a status epilepticus at the onset followed by a cardiorespiratory arrest, requiring cardiopulmonary resuscitation. 9 However, most of the signs and symptoms reported were autonomic without any description of seizures at the onset of PS. Furthermore, cardio-respiratory arrest was reported in four out of around 1000 cases that recovered completely.…”

“…1,8 The main features of PS are the autonomic seizures and occasionally these autonomic symptoms can present with a prominent impact on cardiac and respiratory functions. Mujawar et al 9 reported the case of a 3 year-old child with a diagnosis of PS who presented an ictal cardiorepiratory arrest requiring cardiopulmunary resuscitation. However, this is an extremely rare event, with only 4 cases being reported in the literature.…”

“…However, this is an extremely rare event, with only 4 cases being reported in the literature. 9 The incidence of sudden unexplained death in epilepsy (SUDEP) is estimated to be 0.35-1.8 per 1000 patient-years and the risk is significantly increased (about 3-9 per 1000 patient-years) in those with intractable epilepsy. 10,11 The pathophysiology of this severe complication of epilepsy remains unclear; however, some ictal autonomic changes, particularly cardiac, respiratory and cerebral mechanisms, are probably involved in the etiology of SUDEP.…”

Seizure-Related Autonomic Changes in Children

“…This included at least 1 seizure in 8.2% of children (Moseley et al, 2010). In the literature, only case reports of such profound changes in heart rate exist for children (Dubois-Teklali et al, 2006;Millichap et al, 2011;Mujawar et al, 2011;Venugopalan et al, 2001;Verrotti et al, 2005). When captured in children, ictal bradycardia has been associated with male sex, prolonged seizure duration, concurrent desaturations, and antiepileptic drug polytherapy.…”

Pediatric Focal Epilepsy Syndromes

“…The most frequent and often initial ictal autonomic manifestation is vomiting that occurs in 60% to 80% of the seizures (Caraballo et al, 2007;Chahine and Mikati, 2006;Kivity et al, 2000;Oguni et al, 1999;Lada et al, 2003;Panayiotopoulos et al, 2008). While brief irregularities in breathing and cardiac rhythm are frequent, cardiorespiratory arrest has been reported in very few patients (Ferrie et al, 2006;Grosso et al, 2007;Mujawar et al, 2011;Verrotti et al, 2005). Pallor (28-94%), cyanosis (12%), urinary (19%) and/or fecal (3%) incontinence, hypersalivation (10%), mydriasis (7%), myosis (2%), altered intestinal movements (3%), and thermoregulatory alterations may occur (Caraballo et al, 2007;Covanis, 2006;Oguni et al, 1999;Panayiotopoulos et al, 2008).…”