Cavernous sinus syndrome with pachymeningitis

Abstract

A 67-year-old white man presented, in 2010, with 6 weeks of double vision and left-sided periorbital pain with altered sensation. There was an incomplete third nerve palsy and altered sensation in the first and second trigeminal dermatomes. A malignant melanoma had been excised from his right forearm in 2003, with negative sentinel axial lymph node biopsy. In 2006, he had developed bilateral submandibular salivary gland enlargement, which histologically was chronic inflammatory sialadenitis.Plasma viscosity was persistently raised at 2.28 mPa/s (1.5-1.72) but serum C-reactive protein and serum ACE levels were normal. Chest x-ray showed left hilar prominence.Magnetic resonance scan of the brain showed extensive gadolinium-enhancing pachymeningeal thickening, including in the left Meckel's (trigeminal) cave ( figure 1A,B). Cerebrospinal fluid (CSF) was normal on two occasions, with negative cytology and absent oligoclonal bands.CT of the chest, abdomen and pelvis showed mediastinal and hilar lymphadenopathy. A soft tissue rim encased the distal aorta and the proximal common iliac arteries, suggesting either inflammatory tissue or retroperitoneal fibrosis. Positron-emission tomography with CT scan showed high uptake of radioactive 2-deoxy-2-[18F] fluoro-D-glucose in the hilar and right paratracheal lymph nodes Figure 1 T1-weighted gadolinium-enhanced axial magnetic resonance images of brain showing: (A) thickened meninges of the posterior fossa and left cavernous sinus; (B) marked meningeal thickening involving the falx and convexity meninges, without gyral or sulcal enhancement.

Probable neuromyelitis optica spectrum disorder presenting with recurrent cranial neuropathy

“…Brain MRI showed meningeal and eighth cranial nerve enhancement; therefore her palsy of the eighth cranial nerve might have had a peripheral origin due to pachymeningitis. Although no focal cavernous sinus lesion has been reported in NMO so far, in light of the previously reported cases, it seems reasonable to assume that a cavernous sinus lesion by focal pachymeningitis could occur as a result of NMOSD …”

“…Saba et al . reported a patient with idiopathic pachymeningitis who presented with multiple cranial neuropathy involving the third and fifth nerves with orbital pain that mimic Tolosa–Hunt syndrome; and asymmetric cavernous sinus and diffuse pachymeningeal lesions by gadolinium‐enhanced MRI . Wattamwar et al .…”

“…10 Terada et al reported a patient with idiopathic pachymeningitis who presented with multiple cranial neuropathy involving the third, fourth, fifth and sixth nerves with orbital pain that mimic Tolosa-Hunt syndrome; and an isolated asymmetric cavernous sinus lesion by contrast-enhanced CT. 11 Saba et al reported a patient with idiopathic pachymeningitis who presented with multiple cranial neuropathy involving the third and fifth nerves with orbital pain that mimic Tolosa-Hunt syndrome; and asymmetric cavernous sinus and diffuse pachymeningeal lesions by gadolinium-enhanced MRI. 12 Wattamwar et al reported a patient with pachymeningitis due to Takayasu arteritis who presented with multiple cranial neuropathy involving the third and fourth nerves with headache that mimic Tolosa-Hunt syndrome; and an isolated asymmetric cavernous sinus lesion by contrast-enhanced CT. 13 Thus, pachymeningitis (idiopathic or other etiology) could present a focal cavernous sinus lesion.…”