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Hypertrophic pachymeningitis with cranial nerve palsy as the initial symptom: A case report
1Department of Ophthalmology, The First Affiliated Hospital of Zhejiang Chinese Medical University (Zhejiang Provincial Hospital of Chinese Medicine), Hangzhou, China.
Rationale:
Hypertrophic pachymeningitis (HP) is a rare and complex condition marked by inflammation and thickening of the dura mater. It can occur either on its own or as a result of various underlying medical issues. This type of granulomatous meningitis is extremely rare and poorly understood, making diagnosis and treatment particularly challenging. Patients with HP may experience severe headaches and cranial nerve defects, but in some cases, the condition can occur without any symptoms, making it undetected. We detail the case of patient who was diagnosed with HP after presenting with abducens nerve palsy, a condition that significantly affected her vision and quality of life. Remarkably, she showed substantial recovery following a course of methylprednisolone pulse therapy, coupled with careful radiographic diagnosis and follow-up assessments. The decision to report this case stems from its rarity and the diverse clinical manifestations associated with this condition, which can vary widely among patients.
Patient Concerns:
A 26-year-old Asian female, sought medical attention at The First Affiliated Hospital of Zhejiang Chinese Medical University, where she reported experiencing diplopia, a troubling symptom indicative of abducens nerve palsy.
Diagnosis:
To establish a definitive diagnosis, second-generation sequencing biological detection was employed to rule out any infectious causes, while radiographic imaging provided confirmation of HP.
Interventions:
In terms of intervention, the patient was promptly initiated on a regimen of methylprednisolone pulse therapy, followed by a carefully monitored oral sequential reduction of the medication. Throughout her treatment, she underwent regular liver function tests to ensure her safety, and liver protective drugs were also administered as a precautionary measure.
Outcomes:
As of now, the patient has successfully completed her treatment and is reported to be doing well, marking a positive outcome in her recovery journey.
Lessons:
This case underscores the critical importance of imaging examinations in the clinical diagnosis of patients presenting with diplopia, as they play a vital role in both diagnosing and differentiating between various diseases. Furthermore, it is essential to rule out any infection-related factors in patients diagnosed with HP before commencing treatment with glucocorticoids, ensuring a comprehensive approach to patient care.
Insights
Hypertrophic pachymeningitis (HP) is a rare dural inflammation. A case study shows a patient with abducens nerve palsy recovered well after methylprednisolone pulse therapy, highlighting imaging importance.
Area of Science:
- Neurology
- Radiology
- Immunology
Background:
- Hypertrophic pachymeningitis (HP) is a rare inflammatory condition affecting the dura mater, often presenting with challenging diagnosis and varied symptoms.
- It can manifest with severe headaches or cranial nerve deficits, but can also be asymptomatic.
Observation:
- A 26-year-old female presented with diplopia due to abducens nerve palsy.
- Diagnostic workup included second-generation sequencing to exclude infections and radiographic imaging, which confirmed HP.
Findings:
- The patient received methylprednisolone pulse therapy followed by oral steroids, with regular liver function monitoring.
- Significant recovery was observed post-treatment, with the patient reported to be doing well.
Implications:
- This case highlights the crucial role of imaging in diagnosing diplopia and differentiating conditions like HP.
- It emphasizes the necessity of ruling out infections before initiating glucocorticoid therapy for HP to ensure patient safety.
