Epilepsy and drug-resistant epilepsy in neurocutaneous syndromes

Abstract

Aim: Epilepsy frequently accompanies neurocutaneous syndromes. Due to the comorbid conditions that epileptic seizures may cause, their frequency, type, and duration also affect the prognosis of neurocutaneous diseases. The purpose of this study was to examine the frequency of epilepsy and drug-resistant epilepsy in neurocutaneous syndromes, and factors affecting the development of drug-resistant epilepsy. Methods: In our clinic, nine of the 24 patients who were under follow-up due to neurocutaneous syndromes were diagnosed with Tuberous Sclerosis Complex (TSC), six with Sturge-Weber syndrome, and nine with Neurofibromatosis type 1. We examined the age at diagnosis, findings at the time of the diagnosis, the presence of accompanying epilepsy, age at onset of seizures, seizure type and frequency, antiepileptic drugs used, responses to antiepileptic drugs, electroencephalography and brain magnetic resonance imaging findings, neurological-neuro-ophthalmological and physical examination results, and systemic findings. Results: Age at onset of seizures ranged between four months and four years. A pre-diagnosis history of seizure was present in all the patients with TSC, and the first seizure in 55% of the patients was epileptic spasm type. Hemiparesis and port wine stain were present at the time of diagnosis in Sturge-Weber syndrome, while accompanying seizures were observed later. Conclusion: The frequency of accompanying epilepsy and drug-resistant epilepsy is higher in the TSC.

Keywords

• Drug-resistant epilepsy
• epilepsy
• neurocutaneous syndromes