Cogan’s Syndrome Combined with Hypertrophic Pachymeningitis: A Case Report

Abstract

Cogan’s syndrome (CS) is a rare chronic inflammatory disease, characterized by interstitial keratitis and vestibular auditory dysfunction. Hypertrophic pachymeningitis (HP) is a rare chronic aseptic inflammatory disease of the central nervous system. This article reports a patient with CS coexisting with HP. The patient was a 66-year-old male with fever, headache, red eyes, hearing loss, and significantly elevated inflammatory markers. Cerebrospinal fluid examination, blood culture, and tests for autoantibodies such as antinuclear antibodies were negative. Pure tone audiology (PTA) indicated bilateral sensorineural deafness. Both Positron emission tomography–computed tomography (PET/CT) and vascular color Doppler ultrasound suggest the presence of vasculitis. Considering Cogan’s syndrome, the patient received 40 mg of methylprednisolone intravenously once daily. The brain’s magnetic resonance imaging (MRI) revealed slightly thickened and enhanced dura mater, suggesting HP. The dose of methylprednisolone was increased to 40 mg intravenously every 8 hours, leading to the patient’s improved symptoms and decreased inflammatory markers. Both CS and HP are rare chronic inflammatory diseases, and their coexistence is even rarer, with only two reported cases in literature up to date. The coexistence of CS and HP should be considered when the CS patients with headaches do not respond well to treatment.

Keywords:

• Cogan’s syndrome
• hypertrophic pachymeningitis
• vasculitis

Data Sharing Statement

The data and materials used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Ethics Approval and Consent to Participate

Ethical approval is not necessary because this is a case report based on the clinical data during the patient’s hospitalization other than research and/or experiment. The patient has given his consent and authorized his clinical data in our report and signed the Patient Consent Form.

Consent for Publication

The patient has given his consent and authorized his clinical data in our report to be published.

Acknowledgments

The authors thank the patient for their commitment to this research.

Author Contributions

All authors made a significant contribution to the work reported, whether that is in the conception, study design, execution, acquisition of data, analysis and interpretation, or in all these areas; took part in drafting, revising or critically reviewing the article; gave final approval of the version to be published; have agreed on the journal to which the article has been submitted; and agree to be accountable for all aspects of the work.

Disclosure

The authors declare that there is no conflict of interest.

Additional information

Funding

This work was supported by Grants from the Educational Commission of Zhejiang Province (No. Y201941232).