Idiopathic Intracranial Hypertension Secondary to Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common immune-mediated inflammatory polyneuropathy, defined as progressive or relapsing symptoms for over two months with pathological or electrophysiological evidence of peripheral nerve demyelination. Papilledema is optic nerve head edema secondary to increased intracranial pressure or infiltrative/infectious etiologies. Regardless of the cause, visual loss is one of the feared manifestations due to optic nerve damage. We present a 50-year-old female patient with CIDP who developed papilledema that was secondary to increased intracranial pressure from high protein content in the cerebrospinal fluid (CSF) and elevated body mass index (BMI) secondary to prednisone use. Treatment with acetazolamide completely resolved the papilledema and headaches, and the patient was later maintained on mycophenolate, intravenous immunoglobulin (IVIG), rituximab, and prednisone. To the best of our knowledge, this is the first case that describes successful medical management of increased intracranial pressure in the setting of CIDP.

Keywords: acetazolamide; chronic inflammatory demyelinating polyradiculoneuropathy; hydrocephalus; idiopathic intracranial hypertension; papilledema.

Figure 1. MRI of the orbit. Top image: Axial T2, prominent CSF space along the optic nerve sheath on either side (red arrows). Bottom image: Axial T1, mild flattening of the posterior sclera bilaterally (yellow arrows). These findings can be seen with increased intracranial pressure.