From the Institute of Vision Research, Department of Ophthalmology, Yonsei University College of Medicine, Seoul, Republic of Korea.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Gong Je Seong, MD, PhD, Institute of Vision Research, Department of Ophthalmology, Yonsei University College of Medicine, 712 Eonjuro, Gnangnam-gu, Seoul 135-720, Republic of Korea.
Idiopathic hypertrophic pachymeningitis (IHP) is a rare chronic inflammatory process of unknown etiology that can cause various neurological deficits. Even though patients with IHP commonly present with cranial neuropathy and complain of headache, ophthalmologic manifestations such as ophthalmoplegia and optic neuropathy have occasionally been reported.1–7 There are only a few studies of IHP associated with optic neuritis and acute visual loss were the initial sign of optic nerve involvement in all reported cases.
We report a patient who initially presented with only uveitic glaucoma and subsequently developed optic neuritis associated with IHP. Because we overlooked his headache, neuroimaging study was delayed until development of optic neuritis.
A 73-year-old man presented with a 3-month history of left-sided progressive visual loss associated with chronic left temporal headache. In his left eye, visual acuity was counting fingers and intraocular pressure was 32 mm Hg. Even though there were no signs of active inflammation, almost 360° of posterior synechiae with a small distorted pupil and dense cataract, and extensive peripheral anterior synechiae of the iris were found. Due to the small distorted pupil, the posterior fundus could not be seen. Examination of the right eye was not remarkable. Systemic examinations including abdominal ultrasonography and serologic tests to seek any underlying causes of uveitis found no evidences of malignant, autoimmune, or infectious diseases. A clinical diagnosis of left secondary angle-closure glaucoma and cataract due to uveitis was made. After synechiolysis, cataract extraction was performed by phacoemulsification and a foldable intraocular lens was implanted in the bag. Postoperatively, his vision improved to 20/30 and intraocular pressure fell to 16 mm Hg. Even though the optic nerve head looked slightly pale and had large disc cupping with thinning of the peripapillary retinal nerve fiber layer, these findings were presumed to be due to the long-standing uveitic glaucoma. Even after surgery, he still complained of headache. We recommended neuroimaging, but the patient refused due to the cost. There were no other significant problems and it seemed that his postoperative course was stable.
Three months later, he returned to our clinic due to sudden bilateral visual loss. Visual acuity decreased to hand motion in the right eye and 20/200 in the left eye. Bilateral papillitis was noted. Deficits in color vision and decrease in light sensitivity over the entire central visual field were also found. Fluorescein angiography revealed diffuse hyperfluorescence around the disc margin in both eyes. Gd-DTPA-enhanced cranial magnetic resonance imaging (MRI) (Figure) showed abnormal diffuse thickening and enhancing dura in both frontoparietal areas. Furthermore, this enhancement extended to the bilateral optic nerve sheath and the right eye was more severely affected than the left. However, the cerebrospinal fluid study was normal in pressure and composition. All of these findings were compatible with IHP combined with optic neuritis. After systemic steroid pulse therapy, vision in his left eye recovered to 20/30, but the right eye remained at 20/200 and a pale disc at 2-year follow-up.
Figure. Representative Gd-DPTA-Enhanced Cranial Magnetic Resonance Imaging Showed Thickened Enhancement of the Right Optic Nerve Sheath.
Chronic inflammatory processes play a crucial role in the pathogenesis of IHP. Hypertrophic thickening of the dura is the main pathologic characteristic of IHP. However, its clinical manifestations are very diverse and the exact pathogenesis remains unknown.
In our case, uveitic glaucoma was the initial manifestation of IHP. Even though he complained the only 3-month visual impairment, his initial ophthalmic findings implied the more chronic intraocular inflammation. IHP usually shows a chronic course of inflammation with the repetition of relapse and recovery. Though we do not understand the mechanism, it is possible that inflammation of the dura spread to the intraocular tissue or may be it took place synchronously. Initially, a small distorted pupil disturbed to see the posterior fundus. However, some optic neuropathy may already have been present at that time.
Considering headache, he repeatedly complained of headache, even after sufficiently lowering intraocular pressure. Although he did not agree to undergo neuroimaging, we also overlooked this problem. If we more strongly alerted him of the seriousness of the situation, he might agreed to further studies.
IHP is an exclusive disease and systemic evaluation is needed to rule out other possible causes such as malignant, autoimmune, or infectious diseases. The confirmed diagnosis of IHP usually needs brain MRI and meaningeal biopsy. In our case, we could not find any evidence of other underlying diseases. We did not perform the meningeal biopsy, but all circumstantial evidences supported the diagnosis of IHP.
In conclusion, intraocular inflammation such as uveitis may lead to an associated optic neuritis that may be associated with IHP. Inductively, uveitis might precede IHP. Therefore, when clinicians encounter patients who present uveitic glaucoma accompanied by headache, they should consider neuroimaging study.
- Hamilton SR, Smith CH, Lessell S. Idiopathic hypertrophic cranial pachymeningitis. J Clin Neuroophthalmol. 1993;13:127–134.
- Shintani S, Shiigai T, Tsuruoka S. Hypertrophic cranial pachymeningitis causing progressive unilateral blindness: MR findings. Clin Neurol Neurosurg. 1993;95:65–70. doi:10.1016/0303-8467(93)90095-X [CrossRef]
- Harada T, Ohashi T, Ohki K, et al. Optic neuropathy associated with hypertrophic cranial pachymeningitis. Br J Ophthalmol. 1996;80: 574–575. doi:10.1136/bjo.80.6.574-a [CrossRef]
- Hatano N, Behari S, Nagatani T, et al. Idiopathic hypertrophic cranial pachymeningitis: clinicoradiological spectrum and therapeutic options. Neurosurgery. 1999;45:1336–1342. doi:10.1097/00006123-199912000-00014 [CrossRef]
- de Deus-Silva L, Queiroz Lde S, Zanardi Vd Vde A, et al. Hypertrophic pachymeningitis: case report. Arq Neuropsiquiatr. 2003;61:107–111.
- Hosler MR, Turbin RE, Cho ES, Wolansky LJ, Frohman LP. Idiopathic hypertrophic pachymeningitis mimicking lymphoplasmacyte-rich meningioma. J Neuroophthalmol. 2007;27:95–98.
- Rojana-udomsart A, Pulkes T, Viranuwatti K, Laothamatas J, Phudhichareonrat S, Witoonpanich R. Idiopathic hypertrophic cranial pachymeningitis. J Clin Neurosci. 2008;15:465–469. doi:10.1016/j.jocn.2006.11.020 [CrossRef]