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Idiopathic Hypertrophic Cranial Pachymeningitis with Scleritis and Optic Disc Involvement: a Case Report and Literature Review.

Xuecheng CaiNan HongHuiyan LiBo JiangJianxia FangJiechao ShaoHui LiangYe Shen
Published in: Ocular immunology and inflammation (2023)
This report describes a case of idiopathic hypertrophic cranial pachymeningitis (IHCP) with scleritis and optic disc involvement. The patient was a 56-year-old woman with chief complaints of fever, headache, binocular pain, and redness. Biochemical and immunological indicators, cranial magnetic resonance imaging, and relevant ophthalmological examinations were employed for evaluation. Infectious and neoplastic causes were excluded. Typical meningeal thickening and enhancement on magnetic resonance imaging revealed IHCP. Diffuse hyperaemia and oedema of the conjunctiva and the T-shape sign on the B-scan suggested anterior and posterior scleritis, respectively. Abnormalities in fundus photography, optical coherence tomography, and visual field examination suggested optic disc involvement. After anti-infection and steroid therapy, the patient's temperature returned to normal and the symptoms of headache, binocular pain, and redness improved. Neurologists and ophthalmologists should consider the combination of IHCP with scleritis in their differential diagnosis when patients complain of headache accompanied by ocular pain and redness.
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