Abstracts

A constellation of early-onset electroclinical seizures alternating between the left and right hemispheres and subsequent death or profound neurodevelopmental abnormalities was first described by Coppola et. al.¹ Our report describes the clinical and electroencephalographic characteristics of five new patients encountered at this institution and the prospect of a more optimistic developmental outcome in a few.
A retrospective chart review was performed on five infants encountered between 1996 and 2002 at the Children[rsquo]s Hospital of Philadelphia who presented with unprovoked focal or multifocal seizures electrographically migrating and alternating between the hemispheres before 3 months of age. Clinical and electrographic characteristics and descriptions of long term follow-up were recorded.
All five developed unexpected, early-onset (range from 0 to 4 weeks; mean 1.5 weeks for age at onset) intractable electroclinical seizures that alternated between the two hemispheres. Each patient underwent comprehensive brain imaging and neuro-metabolic work-ups which were unrevealing. They subsequently displayed intractable partial seizures during infancy and childhood. All became significantly hypotonic after or during the neonatal period. Each demonstrated a progressive decline in head circumference percentile with age. Three demonstrated severe developmental delay. All survived and 7yr follow up on one patient is quite favorable.
Coppola et. al.¹ described fourteen infants evaluated over an eleven year period and generated seven major diagnostic criteria to define a new seizure syndrome. All of these characteristics were satisfied by our patients. The prognosis of this rare neonatal onset epilepsy syndrome from Coppola[rsquo]s original description and subsequent case reports^2,3 is very poor with a 35 percent mortality and the majority of survivors described as profoundly retarded and non-ambulatory. Our patient data validate the diagnostic criteria of this syndrome in support of its existence. These data also suggest that the prognosis of this syndrome, although poor, is not as grim as the few cases reported previously in the literature. Finally, our observations suggest that additional criteria including progressive decline of head circumference percentile with age and a hypotonic exam should be added to this neonatal epilepsy syndrome.
1. Coppola, G. et. al., Migrating Partial Seizures in Infancy: A Malignant Disorder with Developmental Arrest. Epilepsia (10) 1017-1024; 1995
2. Wilmshurst, J.M., et. al., Migrating Partial Seizures in Infancy: Two New Cases. Journal Of Child Neurology. 15(11): 717-22, 2000
3. Venselli, E. et.al., Malignant Migrating Partial Seizures In Infancy. Epilepsy Research 46: 27-32; 2001