A case report: Diagnosis and treatment of idiopathic hypertrophic pachymeningitis

Abstract

A 57-year-old man who suffered from a headache for 1 year, accompanied by blurred vision for 7 months and numbness in his left face for 1 week was admitted to the Affiliated Hospital of Zunyi Medical University on May 7, 2022. One year ago, the patient had no obvious precipitating factor of paroxysmal stabbing pain in the whole skull with dizziness, which could be relieved by oneself after lasting for 1–2 min each time, with about 20 episodes per day. The cranial magnetic resonance imaging revealed changes in bilateral frontal lobe ischemic foci, bilateral frontal, ethmoid, sphenoid and maxillary sinusitis, and retinal macular degeneration. After hormone shock treatment, the condition improved. He suffered from headaches again with blurred vision in the right eye 7 months ago and was initially diagnosed with multiple sclerosis. He then was discharged after improvement due to hormone shock therapy. Oral hormone therapy was continued outside the hospital, but he stopped it due to drug side effects (details remained unclear). After cutting off, he developed a headache and visited our hospital once more, the relevant tests were performed and the patient was diagnosed with idiopathic hypertrophic pachymeningitis (IHP). The symptoms were slightly abated after hormone therapy. We hope that through this case report, we can deepen the clinicians' understanding of IHP, and improve the diagnosis rate of the disease through relevant examinations in future clinical work, so that patients can receive timely treatment and the mental pressure and economic burden caused by the disease on patients are reduced.

Keywords

Autoimmune disease / Headache / Idiopathic hypertrophic pachymeningitis (IHP) / Magnetic resonance imaging (MRI) / Myelin oligodendrocyte glycoprotein (MOG)

References

[1] Yao A, Jia L, Wang B, Zhang J, Zhang J, Xu B. Idiopathic hypertrophic pachymeningitis mimicking meningioma with occlusion of superior sagittal sinus: case report and review of literature. World Neurosurg. 2019; 127: 534-537.

[2] Yonekawa T, Murai H, Utsuki S, et al. A nationwide survey of hypertrophic pachymeningitis in Japan. J Neurol Neurosurg Psychiat. 2014; 85(7): 732-739.

[3] Mazzurco M, Pavone P, Di Luca M, et al. Optic neuropathy, secondary to ethmoiditis, and onodi cell inflammation during childhood: a case report and review of the literature. Neuropediatrics. 2019; 50(6): 341-345.

[4] El Aoud S, Frikha F, Ben Salah R, Snoussi M, Loukil H, Bahloul Z. Multiple cranial nerve palsy revealing hypertrophic pachymeningitis with positive myeloperoxidase-antineutrophil cytoplasmic antibody. Reumatismo. 2013; 65(5): 248-252.

[5] Charleston L, Cooper W. An update on idiopathic hypertrophic cranial pachymeningitis for the headache practitioner. Curr Pain Headache Rep. 2020; 24(10): 57.

[6] De Virgilio A, de Vincentiis M, Inghilleri M, et al. Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res. 2017; 65(1): 386-394.

[7] Bi Z, Shang K, Cao J, et al. Hypertrophic pachymeningitis in Chinese patients: presentation, radiological findings, and clinical course. BioMed Res Int. 2020; 2020: 1-9.

[8] Xiao X, Fu D, Feng L. Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study. Front Neurol. 2020; 11:565088.

[9] Hahn LD, Fulbright R, Baehring JM. Hypertrophic pachymeningitis. J Neurol Sci. 2016; 367: 278-283.

[10] Wu CS, Wang HP, Sung SF. Idiopathic hypertrophic pachymeningitis with anticardiolipin antibody: a case report. Medicine. 2021; 100(2):e24387.

[11] Papathanasiou A, Yeo JM, Humberstone M, Hosseini AA. MOG-antibody-associated hypertrophic pachymeningitis. Multiple Sclerosis Related Disord. 2020; 42:102074.

[12] Oghalai JS, Ramirez AL, Hegarty JL, Jackler RK. Chronic pachymeningitis presenting as asymmetric sensorineural hearing loss. Otol Neurotol. 2004; 25(4): 616-621.

[13] Zhu R, He Z, Ren Y. Idiopathic hypertrophic craniocervical pachymeningitis. Eur Spine J. 2015; 24(suppl 4): 633-635.

[14] Huang Y, Chen J, Gui L. A case of idiopathic hypertrophic pachymeningitis presenting with chronic headache and multiple cranial nerve palsies: a case report. Medicine. 2017; 96(29):e7549.

[15] Zhao M, Geng T, Qiao L, et al. Idiopathic hypertrophic pachymeningitis: clinical, laboratory and neuroradiologic features in China. J Clin Neurosci. 2014; 21(7): 1127-1132.

[16] Nicho N, Nozawa T, Murase A, et al. Difficulties of diagnosing idiopathic hypertrophic pachymeningitis in children: case report and literature review. Modern Rheumatol Case Rep. 2023; 7(1): 233-236.

RIGHTS & PERMISSIONS

2023 The Authors. Ibrain published by Affiliated Hospital of Zunyi Medical University and Wiley-VCH GmbH.