Is it seizures? Non-epileptic events in a child with Tay-sachs disease

Case presentation: Male, 4 years old, diagnosed with Tay-Sachs syndrome. Patient with neuropsychomotor developmental delay, presented with polymorphic behaviors such as arrests, tonic posturing and laughter that were treated in another facility with a series of anti-crisis medications with no response. At first evaluation patient was in use of Levetiracetam, Clobazan, Phenobarbital, Oxcarbazepine and Cannabidiol. A 24-hour prolonged videoelectroencephalogram (VEEG) was performed, and 18 clinical events were recorded, however, none of them were accompanied by electrographic changes. Progressive and gradual withdrawal of anticrisis medication was performed and patient evolved with improvement in sedation, without significant modification of events previously considered as epileptic seizures.

Discussion: Mental retardation is a condition that can be present in several conditions in children and adolescents, usually associated with some comorbidity. The condition encompasses a series of behaviors, whether motor or non-motor, which can be confused with epileptic seizures. It is important to differentiate such events form epileptic seizures to avoid overtreatment that can worsen the patient´s clinical condition. Prolonged VEEG is an available diagnostic method and should be indicated in patients with cognitive impairment who have a history of refractory epileptic seizures, being the best method to identify non-epileptic events.

Final comments: Non-epileptic events are common in patients treated with suspected epilepsy. In patients with cognitive impairment unspecific movements are usually confused with epileptic seizures. Studies have demonstrated that almost 40% of children treated as having epilepsy may have no-epileptic events. Correct diagnosis may avoid unnecessary use of anticrisis medication and consequently its side effects.

 

MissingLinkText

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
18 September 2023