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Skull base aspergillosis in an immunocompetent elderly man with early response to steroid
Pamela Sarkar1, Christopher Price2, Mark Fish2
1Neurology Department, North Bristol NHS Trust, Bristol, UK.
Abstract:
We report the case of a previously well 80-year-old man who presented with subacute bilateral painful optic neuropathy with initial response to corticosteroids but ultimately progressed to a fatal skull base syndrome. Initial presentation of steroid-responsive painful bilateral posterior optic neuropathy, preliminary normal enhanced MRI, normal cerebrospinal fluid and inflammatory markers indicated atypical optic neuritis. However, this progressed to a bilateral orbital apex syndrome with ophthalmoplegia and evidence of abnormal skull base enhancement on subsequent MRI. Biopsy of radiologically abnormal dura was non-diagnostic and negative for fungal stains. He deteriorated and died 8 months after initial presentation. At postmortem, fungal skull base infection was diagnosed. This case demonstrates that chronic skull base fungal infection can: (1) present in elderly immunocompetent patients, (2) show initial improvement with corticosteroids and (3) evade diagnosis on biopsy. We encourage a high index of suspicion for fungal skull base infection in similar cases.
Insights
A rare fungal skull base infection mimicked optic neuritis in an elderly man, initially responding to steroids before proving fatal. This case highlights the need for high suspicion in similar presentations.
Area of Science:
- Neurology
- Infectious Diseases
- Ophthalmology
Background:
- Atypical optic neuritis can present with subacute bilateral painful optic neuropathy.
- Elderly, immunocompetent patients can develop chronic fungal skull base infections.
Observation:
- The patient presented with steroid-responsive optic neuropathy, normal initial MRI, and CSF findings.
- The condition progressed to orbital apex syndrome with ophthalmoplegia and abnormal skull base enhancement.
- Dural biopsy was non-diagnostic and negative for fungal elements.
Findings:
- Postmortem examination revealed a chronic fungal skull base infection.
- Fungal skull base infection can evade diagnosis via biopsy and mimic inflammatory conditions.
- Corticosteroid treatment can provide temporary improvement in fungal skull base infections.
Implications:
- A high index of suspicion for fungal skull base infection is crucial in elderly patients with atypical optic neuropathy.
- Diagnostic challenges include initial steroid responsiveness and negative biopsies.
- Prompt diagnosis and treatment of fungal skull base infections are vital to prevent fatal outcomes.
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