Abstracts

Alternating hemiplegia of childhood is a rare neurologic disorder presenting in infancy or early childhood with abnormal eye movements and paroxystic motor phenomenae of alternating hemiplegia. Epilepsy is associated in up top 50% of cases, which may cause diagnostic difficulty between seizures and hemiplegic episodes in these cases. We discuss the role of video-EEG monitoring in the diagnosis of epileptic and non-epileptic events in two cases of alternating hemiplegia of childhood and discuss implications in management. Two unrelated boys with developmental delay and ataxia presented with paroxysmal spells in the first year. In the first patient, intermittent nystagmus and dystonic posturing started at age 6 months, followed by episodes of alternating transient hemiplegia lasting hours to days, worsened by stress. At age 2, brief blinking episodes and loss of body tone. Flunarizine was started. Video-EEG documented episodes of hemiplegia and complex focal seizures. Brain MRI disclosed left mesial temporal sclerosis, which had not been seen in a previous, good quality exam. Antiepileptic therapy was started, with reduction of seizure frequency. The second patient presented episodes of global hypotonia and abnormal eye movements at age 4 months. At age 2, these symptoms disappeared, and episodes of alternating or bilateral hemiplegia ensued. Antiepileptic drugs were started, with poor response. The child had two ICU admissions for presumed status epilepticus. At age 2 1/2, video-EEG monitoring was performed. Typical events of hemiplegia were registered, during which no epileptiform activity was seen.Brain MRI was normal. Antiepileptic drugs were stopped and flunarizine was introduced, with clinical improvement. Video-EEG monitoring may provide useful information in establishing a precise diagnosis of paroxysmal epileptic and nonepileptic events in patients with alternating hemiplegia, with impact in management.