Skull base aspergillosis in an immunocompetent elderly man with early response to steroid.

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.