Epilepsy in Wolf-Hirschhorn Syndrome (4p-)
Article first published online: 11 JAN 2005
Volume 46, Issue 1, pages 150–155, January 2005
How to Cite
Kagitani-Shimono, K., Imai, K., Otani, K., Kamio, N., Okinaga, T., Toribe, Y., Suzuki, Y. and Ozono, K. (2005), Epilepsy in Wolf-Hirschhorn Syndrome (4p-). Epilepsia, 46: 150–155. doi: 10.1111/j.0013-9580.2005.02804.x
- Issue published online: 11 JAN 2005
- Article first published online: 11 JAN 2005
- Accepted August 27, 2004.
- Wolf-Hirschhorn syndrome;
- Status epilepticus;
Summary: Purpose: We investigated the evolution of epilepsy, seizure types, and effective drugs in Wolf-Hirschhorn syndrome, which is a malformation syndrome often with refractory seizures and status epilepticus.
Methods: We reviewed 11 cases of Wolf-Hirschhorn syndrome (age range, 2–25 years; SD, 7.2 years) and who were treated in Osaka University or Osaka Medical Center of Research Institute for Maternal and Child Health.
Results: In all patients, febrile or afebrile convulsions had developed. Epileptic seizures included alternative hemiconvulsions, generalized tonic–clonic seizures, focal clonic seizures, tonic seizures, and epileptic spasms. Seizures were often induced by a high fever or a hot bath. Status epilepticus occurred in all patients, including one patient who died at the first status epilepticus. In some cases, intratracheal intubation was needed because of respiratory insufficiency. The effective antiepileptic drugs for long-term use were sodium bromide (four of four), followed by clorazepate (CLP; one of two), and nitrazepam (NZP; two of four). Sodium bromide was particularly effective for preventing status epilepticus. The mean age of last status epilepticus in patients receiving sodium bromide (1 year 8 months) was significantly younger than that in those not treated with sodium bromide (3 year 4 months).
Conclusions: We identified that, in most patients of Wolf-Hirschhorn syndrome, the frequency of both seizures and status epilepticus decreased gradually after age 5 years. However, during infancy, status epilepticus sometimes resulted in permanent disability or even death. We propose that sodium bromide should be used as the initial treatment for the prevention of the development of status epilepticus associated with Wolf-Hirschhorn syndrome.