Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in a Girl Presented with Complex Partial Seizures

Area of Science:

• Neurology
• Pediatric Neurology
• Neuroscience

Background:

• The precise mechanisms of Hemiconvulsion-Hemiplegia-Epilepsy (HHE) syndrome are not fully understood.
• Current hypotheses suggest neuronal injury due to venous thrombosis and/or hypoxia as the primary cause.
• HHE syndrome typically manifests as hemispheric brain atrophy, hemiplegia, epilepsy, and cognitive deficits in children.

Observation:

• A 33-month-old female presented with right unilateral brain edema and left-sided hemiplegia and aphasia.
• The patient experienced complex partial seizures evolving to generalized tonic-clonic seizures, leading to status epilepticus.
• Diagnostic and therapeutic interventions for status epilepticus posed significant challenges.

Findings:

• Progressive atrophy of the right cerebral hemisphere was observed during a 3-month follow-up period.
• The case demonstrated a unique presentation of unilateral brain edema preceding atrophy in HHE syndrome.
• The patient's symptoms included intractable epilepsy and neurological deficits.

Implications:

• HHE syndrome should be considered in pediatric cases presenting with unilateral cerebral hemisphere brain edema and hemiplegia.
• Early suspicion aids in providing accurate prognoses for epilepsy development and cognitive outcomes.
• This case underscores the complexity of HHE syndrome and the need for further research into its pathogenesis.