A Comprehensive Management of HHE Syndrome: A Case Report

Abstract

Hemiconvulsion Hemiplegia Epilepsy is a rare pediatric neurological disorder that is marked by prolonged unilateral convulsions, hemiplegia, and epilepsy. Clinical manifestations include progressive developmental delay and neurological deficits. The condition was classified in 1960 by Gastaut. This condition falls under the category of epileptic encephalopathies and post-ictal hemiplegia syndrome. The case report of a 15-year-old boy with HHE syndrome highlights the consequences of early seizure onset, non-compliance to treatment, and its impact on neurological development. The article provides a thorough insight into the management of the syndrome thereby improving the outcome and quality of life. Case Report: A 15-year-old boy came to the emergency with complaints of increased frequency of seizures. The child was born to consanguineous parents and experienced perinatal distress and measles at 7 months of age. At 9 months of age, the child experienced prolonged seizures which led to right hemiplegia. MRI results confirmed the left MCA infarct with encephalomalacia. The noncompliance to initial therapy resulted in developmental delay. The child was managed with drugs like brevetiracetam, quetiapine, and olanzapine. Discussion: HHE syndrome mainly affects children under 10 and is frequently preceded by febrile seizures. The pathophysiology involves prolonged seizures leading to cerebral edema, cortical atrophy, and vascular anomalies. The diagnostic evaluation involves clinical assessment, genetic testing, and imaging studies. The management includes drug therapy, rehabilitation, and surgical interventions in severe cases. Conclusion: Effective management of HHE syndrome involves timely diagnosis, and therapy to prevent complications and improve quality of life.