Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

Abstract

Sturge-Weber syndrome (SWS) is a rare congenital disease associated with venous angiomatosis of the leptomeninges and ipsilateral, cutaneous facial port-wine stain (facial angiomatosis). Seizures are seen in more than 80% of cases, cognitive impairment in 50% and frequently glaucoma. 1-2 Seizures are the result of leptomeningeal angioma and underlying calcifications. These abnormalities are usually considered to be limited to one hemisphere. Frequently, seizures in SWS are refractory to medical treatment. In those cases surgical resection of the affected areas is recommended. Some form of hemispherectomy is usually performed for SWS-associated intractable epilepsy and most patients become seizure-free after surgery. 3-5 Another possible benefit of hemispherectomy is a better cognitive profile in patients that became seizure-free after such a procedure. However, very long follow-ups addressing seizure control and cognitive deficits in hemispherectomized patients are rare and they usually show good outcomes. Here we report on a SWS patient who become seizure-free after hemispherectomy performed in the first year of life, however, seizures recurred 29 years later without any identifiable cause. She also had cognitive decline before the onset of seizures. To the best of our knowledge this is the longest seizure-free interval between hemispherectomy and seizure recurrence in SWS.

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

“…49 Whether such cognitive preservations are long-lasting or are followed by a long-term decline in some cases (as reported in at least one patient following early hemispherectomy) remains to be determined. 50 Increased cortical metabolism contralateral to severely hypometabolic cortex may be an imaging marker of such reorganization (Fig. 2B).…”

Endoscopy-Assisted Interhemispheric Transcallosal Hemispherotomy

“…Hemispherotomy can achieve excellent outcomes when performed in optimally indicated patients. 3,[6][7][8]10,14,16,18,20,28,35,[43][44][45][46][47][48][49][50][51][52][53] Since the introduction of functional hemispherectomy by Rasmussen, morbidity and mortality have steadily decreased. 8,10,11,13,14,28,[43][44][45][46][47]54 However, this procedure still cannot be considered as a trivial surgery because most of the surgeries are performed in children who cannot tolerate blood loss and are prone to other perioperative morbidities such as hypothermia, electrolyte disturbances, and other problems associated with pediatric patients.…”