Eight cases of idiopathic hypertrophic meningitis with ophthalmic manifestations as the first symptom_Chinese Journal of Ocular Fundus Diseases

Authors：

Sun Mingming ^1,2 , Zhou Huanfen ¹ , Yang Mo ¹ , Song Honglu ¹ , Xu Xintong ¹ , Wei Shihui ¹ , Zhou Ming ² ,  Xu Quangang ¹

1. Department of Ophthalmology, The First Medical Center, Chinese PLA General Hospital, Beijing 100853, China;
2. Department of Ophthalmology, Affiliated Zhongshan hospital of Dalian University, Dalian 116001, China;

Corresponding author：

Xu Quangang, Email: xuquangang@126.com

Keywords：

Meningitis; Vision, low; Diplopia; Glucocorticoids; Disease modifying therapy

DOI：

10.3760/cma.j.cn511434-20200204-00037

Video：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

Objective To investigate the clinical, laboratory and imaging evaluation, treatment and prognosis of patients with idiopathic hypertrophic pachymeningitis (IHP) with ophthalmic manifestations as the first symptom.Methods A retrospective case analysis. Eight patients displaying symptoms of IHP were recruited from the Neuro-ophthalmology Department in the First Medical Center of Chinese PLA General Hospital from January 2016 to April 2019 were inculed in this study. There were 6 males and 2 females, aged from 11 to 65 years, with an average age of 48.00±19.08 years. The course of disease ranged from 30 days to 7.5 years, with an average course of 17.00±30.08 months. The age, symptoms and signs of all patients were recorded. All patients underwent ophthalmic examination, orbit or brain MRI or CT examinations, blood routine examination, biochemistry, tumor markers, immunity, hepatitis B, syphilis, HIV, thyroid function and other laboratory tests, and lumbar puncture was performed to measure the cerebrospinal fluid (CSF) pressure and indicators. The clinical manifestations, orbital or brain MRI imaging and laboratory examination characteristics were summarized. Treatment and prognosis were also observed.Results In total of 8 patients, visual loss was presented in 6 patients, visual loss and diplopia were presented in 1 patient, and diplopia was presented in 1 patient. Binocular involvement in 7 patients and monocular involvement in 1 patient. Other symptoms including headache and hear loss and so on. Optic disc edema in 1 eye and optic disc pallor in 6 eyes were reviewed by fundus examination. The laboratory examination showed that the angiotensin converting enzyme abnormal in 4 patients, the anti-thyroid peroxidase antibody abnormal in 3 patients and immunoantibodies positive in 3 patients. CSF measurements showed that the protein level elevated in all patients. Orbit and/or brain MRI and CT examination showed that optic nerve involvement in 6 patients, oculomotor nerve involvement in 1 patient, and cavernous sinus region involvement in 2 patients. Glucocorticoid was effective in all patients, and the visual acuity significantly improved in 4 patients, the diplopia was completely resolved in 2 patients, and the disease modifying therapy (DMT) was combined to prevent recurrence in 7 patients. No recurrence was observed in an average follow-up time of 26.63±16.55 months.Conclusions IHP patients may be first visit an ophthalmologist due to vision loss in bilateral eyes simultaneous or sequentially. IHP patients are often associated with headache and other cranial nerve paralysis symptoms. Definitive diagnosis of IHP depends on imaging examination. Glucocorticoid treatment is effective in early phase, but it is tendency to progress and relapse, suggesting combined with DMT as early as possible.

Citation： Sun Mingming, Zhou Huanfen, Yang Mo, Song Honglu, Xu Xintong, Wei Shihui, Zhou Ming, Xu Quangang. Eight cases of idiopathic hypertrophic meningitis with ophthalmic manifestations as the first symptom. Chinese Journal of Ocular Fundus Diseases, 2020, 36(4): 262-268. doi: 10.3760/cma.j.cn511434-20200204-00037 Copy

1.	Yonekawa T, Murai H, Utsuki S, et al. A nationwide survey of hypertrophic pachymeningitis in Japan[J]. J Neurol Neurosurg Psychiatry, 2014, 85(7): 732-739. DOI: 10.1136/jnnp-2013-306410.
2.	Kupersmith MJ, Martin V, Heller G, et al. Idiopathic hypertrophic pachymeningitis[J]. Neurology, 2004, 62(5): 686-694. DOI: 10.1212/01.wnl.0000113748.53023.b7.
3.	van Toorn R, Esser M, Smit D, et al. Idiopathic hypertrophic cranial pachymeningitis causing progressive polyneuropathies in a child[J]. Eur J Paediatr Neurol, 2008, 12(2): 144-147. DOI: 10.1016/j.ejpn.2007.07.013.
4.	Kanzaki S, Inoue Y, Watabe T, et al. Hypertrophic chronic pachymeningitis associated with chronic otitis media and mastoiditis[J]. Auris Nasus Larynx, 2004, 31(2): 155-159. DOI: 10.1016/j.anl.2004.01.004.
5.	Mamelak AN, Kelly WM, Davis RL, et al. Idiopathic hypertrophic cranial pachymeningitis: report of three cases[J]. J Neurosurg, 1993, 79(2): 270-276. DOI: 10.3171/jns.1993.79.2.0270.
6.	Martin N, Masson C, Henin D, et al. Hypertrophic cranial pachymeningitis: assessment with CT and MR imaging[J]. AJNR Am J Neuroradiol, 1989, 10(3): 477-484.
7.	Masson C, Hénin D, Hauw JJ, et al. Cranial pachymeningitis of unknown origin: a study of seven cases[J]. Neurology, 1993, 43(7): 1329-1334. DOI: 10.1212/wnl.43.7.1329.
8.	Goyal M, Malik A, Mishra NK, et al. Idiopathic hypertrophic pachymeningitis: spectrum of the disease[J]. Neuroradiology, 1997, 39(9): 619-623. DOI: 10.1007/s002340050479.
9.	Horiguchi T, Gotoh K, Yoshida K, et al. A successful case of hypertrophic cranial pachymeningitis treated by optic nerve decompression[J]. No Shinkei Geka, 1996, 24(3): 281-285.
10.	Nikoskelainen EK, Huoponen K, Juvonen V, et al. Ophthalmologic findings in Leber hereditary optic neuropathy, with special reference to mtDNA mutations[J]. Ophthalmology, 1996, 103(3): 504-514. DOI: 10.1016/s0161-6420(96)30665-9.
11.	Michel D, Girard PF, Tommasi M, et al. Intracranial granulomatous pachymeningitis with pseudotumoral symptomatology: apropos of 4 cases[J]. J Med Lyon, 1969, 50(162): 545-578.
12.	Feringa ER, Weatherbee L. Hypertrophic granulomatous cranial pachymeningitis causing progressive blindness in a chronic dialysis patient[J]. J Neurol Neurosurg Psychiatry, 1975, 38(12): 1170-1176. DOI: 10.1136/jnnp.38.12.1170.
13.	Hatano N, Behari S, Nagatani T, et al. Idiopathic hypertrophic cranial pachymeningitis: clinicoradiological spectrum and therapeutic options[J]. Neurosurgery, 1999, 45(6): 1336-1342. DOI: 10.1097/00006123-199912000-00014.
14.	Wallace ZS, Carruthers MN, Khosroshahi A, et al. IgG4-related disease and hypertrophic pachymeningitis[J]. Medicine, 2013, 92(4): 206-216. DOI: 10.1097/MD.0b013e31829cce35.
15.	Mathew RG, Hogarth KM, Coombes A. Idiopathic hypertrophic cranial pachymeningitis presenting as acute painless visual loss[J]. Int Ophthalmol, 2012, 32(2): 195-197. DOI: 10.1007/s10792-012-9536-2.
16.	Rossi S, Giannini F, Cerase A, et al. Uncommon findings in idiopathic hypertrophic cranial pachymeningitis[J]. J Neurol, 2004, 251(5): 548-555. DOI: 10.1007/s00415-004-0362-y.
17.	Hahn LD, Fulbright R, Baehring JM. Hypertrophic pachymeningitis[J]. J Neurol Sci, 2016, 367: 278-283. DOI: 10.1016/j.jns.2016.06.024.
18.	Jang Y, Lee ST, Jung KH, et al. Rituximab treatment for idiopathic hypertrophic pachymeningitis[J]. J Clin Neurol, 2017, 13(2): 155-161. DOI: 10.3988/jcn.2017.13.2.155.
19.	Riku S, Kato S. Idiopathic hypertrophic pachymeningitis[J]. Neuropathology, 2003, 23(4): 335-344. DOI: 10.1046/j.1440-1789.2003.00520.x.
20.	Sylaja PN, Cherian PJ, Das CK, et al. Idiopathic hypertrophic cranial pachymeningitis[J]. Neurol India, 2002, 50(1): 53-59.
21.	Nadgir DB, Ramdas R, Kulkarni RV, et al. Cavernous sinus syndrome due to syphilitic pachymeningitis[J]. Neurol India, 2003, 51(2): 289-290.
22.	Choi IS, Park SC, Jung YK, et al. Combined therapy of corticosteroid and azathioprine in hypertrophic cranial pachymeningitis[J]. Eur Neurol, 2000, 44(4): 193-198. DOI: 10.1159/000008235.
23.	Ruiz-Sandoval JL, Bernard-Medina G, Ramos-Gómez EJ, et al. Idiopathic hypertrophic cranial pachymeningitis successfully treated with weekly subcutaneous methotrexate[J]. Acta Neurochir (Wien), 2006, 148(9): 1011-1014. DOI: 10.1007/s00701-006-0775-8.
24.	Bosman T, Simonin C, Launay D, et al. Idiopathic hypertrophic cranial pachymeningitis treated by oral methotrexate: a case report and review of literature[J]. Rheumatol Int, 2008, 28: 713-718. DOI: 10.1007/s00296-007-0504-5.

1. Yonekawa T, Murai H, Utsuki S, et al. A nationwide survey of hypertrophic pachymeningitis in Japan[J]. J Neurol Neurosurg Psychiatry, 2014, 85(7): 732-739. DOI: 10.1136/jnnp-2013-306410.
2. Kupersmith MJ, Martin V, Heller G, et al. Idiopathic hypertrophic pachymeningitis[J]. Neurology, 2004, 62(5): 686-694. DOI: 10.1212/01.wnl.0000113748.53023.b7.
3. van Toorn R, Esser M, Smit D, et al. Idiopathic hypertrophic cranial pachymeningitis causing progressive polyneuropathies in a child[J]. Eur J Paediatr Neurol, 2008, 12(2): 144-147. DOI: 10.1016/j.ejpn.2007.07.013.
4. Kanzaki S, Inoue Y, Watabe T, et al. Hypertrophic chronic pachymeningitis associated with chronic otitis media and mastoiditis[J]. Auris Nasus Larynx, 2004, 31(2): 155-159. DOI: 10.1016/j.anl.2004.01.004.
5. Mamelak AN, Kelly WM, Davis RL, et al. Idiopathic hypertrophic cranial pachymeningitis: report of three cases[J]. J Neurosurg, 1993, 79(2): 270-276. DOI: 10.3171/jns.1993.79.2.0270.
6. Martin N, Masson C, Henin D, et al. Hypertrophic cranial pachymeningitis: assessment with CT and MR imaging[J]. AJNR Am J Neuroradiol, 1989, 10(3): 477-484.
7. Masson C, Hénin D, Hauw JJ, et al. Cranial pachymeningitis of unknown origin: a study of seven cases[J]. Neurology, 1993, 43(7): 1329-1334. DOI: 10.1212/wnl.43.7.1329.
8. Goyal M, Malik A, Mishra NK, et al. Idiopathic hypertrophic pachymeningitis: spectrum of the disease[J]. Neuroradiology, 1997, 39(9): 619-623. DOI: 10.1007/s002340050479.
9. Horiguchi T, Gotoh K, Yoshida K, et al. A successful case of hypertrophic cranial pachymeningitis treated by optic nerve decompression[J]. No Shinkei Geka, 1996, 24(3): 281-285.
10. Nikoskelainen EK, Huoponen K, Juvonen V, et al. Ophthalmologic findings in Leber hereditary optic neuropathy, with special reference to mtDNA mutations[J]. Ophthalmology, 1996, 103(3): 504-514. DOI: 10.1016/s0161-6420(96)30665-9.
11. Michel D, Girard PF, Tommasi M, et al. Intracranial granulomatous pachymeningitis with pseudotumoral symptomatology: apropos of 4 cases[J]. J Med Lyon, 1969, 50(162): 545-578.
12. Feringa ER, Weatherbee L. Hypertrophic granulomatous cranial pachymeningitis causing progressive blindness in a chronic dialysis patient[J]. J Neurol Neurosurg Psychiatry, 1975, 38(12): 1170-1176. DOI: 10.1136/jnnp.38.12.1170.
13. Hatano N, Behari S, Nagatani T, et al. Idiopathic hypertrophic cranial pachymeningitis: clinicoradiological spectrum and therapeutic options[J]. Neurosurgery, 1999, 45(6): 1336-1342. DOI: 10.1097/00006123-199912000-00014.
14. Wallace ZS, Carruthers MN, Khosroshahi A, et al. IgG4-related disease and hypertrophic pachymeningitis[J]. Medicine, 2013, 92(4): 206-216. DOI: 10.1097/MD.0b013e31829cce35.
15. Mathew RG, Hogarth KM, Coombes A. Idiopathic hypertrophic cranial pachymeningitis presenting as acute painless visual loss[J]. Int Ophthalmol, 2012, 32(2): 195-197. DOI: 10.1007/s10792-012-9536-2.
16. Rossi S, Giannini F, Cerase A, et al. Uncommon findings in idiopathic hypertrophic cranial pachymeningitis[J]. J Neurol, 2004, 251(5): 548-555. DOI: 10.1007/s00415-004-0362-y.
17. Hahn LD, Fulbright R, Baehring JM. Hypertrophic pachymeningitis[J]. J Neurol Sci, 2016, 367: 278-283. DOI: 10.1016/j.jns.2016.06.024.
18. Jang Y, Lee ST, Jung KH, et al. Rituximab treatment for idiopathic hypertrophic pachymeningitis[J]. J Clin Neurol, 2017, 13(2): 155-161. DOI: 10.3988/jcn.2017.13.2.155.
19. Riku S, Kato S. Idiopathic hypertrophic pachymeningitis[J]. Neuropathology, 2003, 23(4): 335-344. DOI: 10.1046/j.1440-1789.2003.00520.x.
20. Sylaja PN, Cherian PJ, Das CK, et al. Idiopathic hypertrophic cranial pachymeningitis[J]. Neurol India, 2002, 50(1): 53-59.
21. Nadgir DB, Ramdas R, Kulkarni RV, et al. Cavernous sinus syndrome due to syphilitic pachymeningitis[J]. Neurol India, 2003, 51(2): 289-290.
22. Choi IS, Park SC, Jung YK, et al. Combined therapy of corticosteroid and azathioprine in hypertrophic cranial pachymeningitis[J]. Eur Neurol, 2000, 44(4): 193-198. DOI: 10.1159/000008235.
23. Ruiz-Sandoval JL, Bernard-Medina G, Ramos-Gómez EJ, et al. Idiopathic hypertrophic cranial pachymeningitis successfully treated with weekly subcutaneous methotrexate[J]. Acta Neurochir (Wien), 2006, 148(9): 1011-1014. DOI: 10.1007/s00701-006-0775-8.
24. Bosman T, Simonin C, Launay D, et al. Idiopathic hypertrophic cranial pachymeningitis treated by oral methotrexate: a case report and review of literature[J]. Rheumatol Int, 2008, 28: 713-718. DOI: 10.1007/s00296-007-0504-5.

Chinese Journal of Ocular Fundus Diseases

Eight cases of idiopathic hypertrophic meningitis with ophthalmic manifestations as the first symptom

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content