Concomitant Idiopathic Intracranial Hypertension, Normal Pressure Hydrocephalus, and Pleomorphic Xanthoastrocytoma: A Case Report and Review of the Literature

Abstract

Background. Idiopathic intracranial hypertension (IIH) and normal pressure hydrocephalus (NPH) are disorders of the cerebrospinal fluid (CSF) flow dynamics. Pleomorphic xanthoastrocytoma (PXA) is a rare low-grade astrocytoma (World Health Organization grade II) representing <1% of astrocytomas. Combination of IIH and NPH with PXA is unheard of, with few published cases discussing the association of CNS tumors with either IIH or NPH, but never combined. We present a case of a 51-year-old woman with such a rare combination. Case Presentation. A fifty-one-year-old obese female presented with a progressive visual loss, abducens nerve palsy, and headache for three months. Further investigations revealed a right frontal tumor and ventriculomegaly on magnetic resonance imaging. Her symptoms improved remarkably after total surgical excision of the tumor with a ventriculoperitoneal (VP) shunt. Conclusion. The pathophysiology behind NPH and IIH is still not fully understood, yet their management is mostly dependent on CSF diversion. The concomitant development of two different CSF dynamic diseases with a PXA has not been reported in the literature. We hypothesize that PXA may have sparked an abnormal CSF circulation pattern and ventriculomegaly.