{Reference Type}: Journal Article
{Title}: Seizure course of PCDH19 clustering epilepsy in female children: A multicentre cohort study in China.
{Author}: Chen Y;Liu A;Zhang X;Ma X;Sun D;Tian X;Wu W;Zeng Q;Jiang Y;Zhang Y;
{Journal}: Dev Med Child Neurol
{Volume}: 66
{Issue}: 6
{Year}: 2024 Jun 13
{Factor}: 4.864
{DOI}: 10.1111/dmcn.15803
{Abstract}: <B>OBJECTIVE: </B>To investigate the seizure course of PCDH19 clustering epilepsy (PCDH19-CE) in a cohort of female children in China.<BR><B>METHODS: </B>This ambidirectional cohort study examined 113 female patients with PCDH19-CE through multicentre collaboration. Prognostic factors for seizure freedom were evaluated by multivariate Cox regression analysis.<BR><B>RESULTS: </B>The median seizure course period from seizure onset was 6 years 6 months. Of 113 patients, 78% and 56% experienced seizure freedom for at least 1 year and at least 2 years respectively. In patients younger than 5 years (n = 30), 5 to 10 years (n = 52), and older than 10 years (n = 31), 57%, 81%, and 94% experienced at least 1 year of seizure freedom, and 32%, 52%, and 84% experienced at least 2 years of seizure freedom, respectively. However, 58% (65 out of 113) relapsed at least once after more than 1 year of seizure freedom without trigger exposure (40%) or because of common triggers, including fever (43%) and antiseizure medication (ASM) reduction (29%). There was an 84% risk of seizure relapse after ASM reduction attempts. The likelihood of seizure freedom decreased with early age at seizure onset and developmental delay.<BR><B>CONCLUSIONS: </B>Patients with PCDH19-CE exhibit increasing seizure freedom with age, but there is a risk of relapse. ASM reduction in children younger than 10 years old requires caution. Patients with early seizure onset and developmental delay have a reduced chance of seizure freedom.<BR><B>CONCLUSIONS: </B>The seizure freedom rate in PCDH19 clustering epilepsy gradually increases with age. The disease course is characterized by relapsing-remitting seizures. Antiseizure medication reduction requires caution for patients younger than 10 years of age. Patients with early seizure onset and developmental delay are less likely to achieve seizure freedom.