COMORBIDITY IN HEMICONVULSION-HEMIPLEGIA-EPILEPSY SYNDROME IN CHILDREN

Abstract

Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a rare consequence of focal status epilepticus (SE) in childhood. The major discerning feature of the syndrome is the unilateral brain lesion during SE with subsequent development of hemiparesis and drug-resistant epilepsy. The unilateral brain damage in SE is not fully understood. We hypothesized that comorbidity could be the cause of this the hemisphere damage; to that end, we searched for the evidence of comorbidity in patients with HHE. Objective : to analyze the concomitant pathology in patients with HHE, which was the possible cause of unilateral brain damage. Materials and methods . Four patients with HHE syndrome (aged from 1 year 2 months to 6 years; three boys and a girl) were examined. Results . The course of the disease in all patients met the criteria of HHE; various manifestations of comorbidity were found in all four patients. Thus, patient A.A. (6 years old) had hypothyroidism, the history of consciousness loss, acetone smell from the mouth, and spontaneous generalized seizure attacks starting from the age of 11 months. Patient V.S. (6 years old) had adrenogenital syndrome and inborn glaucoma. Patient D.Sh. (1 year 10 months old) had a structural heart defect, a high degree of stigmatization, and suspected Wolf-Hirschhorn syndrome. Patient V.Kh. (3 years old) had cerebral palsy and post-hemorrhagic occlusion hydrocephaly. Conclusion . We propose that comorbidity can be the cause of hidden or obvious lesions in one hemisphere, which can explain the unilateral brain damage in HEE syndrome.