Was Cavum Septum Pellucidum the Cause of Intractable Seizure in a 17-Year-Old Boy with Wilson Disease?

Abstract

Background: Cavum septum pellucidum (CSP), which is often found incidentally in a few populations, occasionally becomes symptomatic if enlarged significantly. Wilson disease (WD) is an uncommon autosomal recessive inborn defect in copper metabolism characterized by abnormal accumulation of copper in various tissues, particularly in the liver and the brain. Seizure disorder, although rare both in CSP and WD, may happen in a few patients with either of the conditions.

Case description: We report a case of 17-year-old boy, a patient with known WD, who developed intractable seizure for a year, which was not controlled with a large amount of antiepileptics. Magnetic resonance imaging showed enlargement of his preexisting CSP, which was small and asymptomatic at the time of diagnosis of WD. His WD was in a state of remission when he developed the seizure disorder. On endoscopic cyst fenestration, he was relieved of the seizure.

Conclusions: Symptomatic CSP is a rare disorder, but the coexistence of WD is even rarer. Endoscopic cyst fenestration is a novel procedure that can be successful in properly selected cases. To the best of our knowledge, CSP associated with WD has not been reported in any English literature. We present this case for its rarity along with a relevant literature review.

Keywords: Cavum septum pellucidum; Endoscopic cyst fenestration; Seizure; Wilson disease.