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Sarcoidosis of orbita and central nervous system presenting as a non‐arteritic ischemic optic neuropathy
Author(s) -
De Bruyn D.,
Ninclaus V.,
Leroy B.P.,
De Schryver I.,
De Zaeytijd J.
Publication year - 2015
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.2015.0485
Subject(s) - medicine , sarcoidosis , neurosarcoidosis , uveitis , optic nerve , optic neuropathy , optic chiasm , ischemic optic neuropathy , pathology , radiology , ophthalmology
Purpose To describe a patient who presented with non‐arteritic ischemic optic neuropathy ( NA ‐ AION ) as an initial manifestation of neurosarcoidosis. Methods A case of a 70‐year‐old woman who presented with loss of vision in the left eye and normal neuroimaging, was initially diagnosed as a NA ‐ AION . An extensive workup included MRI orbita and PET / CT scan. Results At the initial presentation, the left eye showed loss of vision and papilloedema. Further ophthalmological examination was normal, as was a CT scan of the brain. A diagnosis of NA ‐ AION was entertained. After 4 months, she had no light perception vision. Fundoscopy of the LE showed papilloedema with retinal infiltrates and severe ischaemia. MRI of the brain showed mass infiltration of the intraorbital and the intracranial optic nerve up to the optic chiasm. A complete systemic workup revealed a monoclonal gammopathy with cervical and hilar lymph nodes noted on PET / CT scan. Mediastinoscopy with biopsy of the hilar lymph nodes demonstrated a non‐caseating granulomatous lesion and a diagnosis of sarcoidosis was confirmed. Conclusions Sarcoidosis is a multisystemic disease characterized by granulomatous inflammation. Ocular involvement is seen in approximately 25% of patients with sarcoidosis. Uveitis is the most common ocular manifestation. Orbital and CNS manifestations of sarcoidosis are uncommon. Involvement of the optic nerve, chiasm and visual tract only represent 1–5% of neurosarcoidosis cases. In the latter, sarcoidosis can be initially misdiagnosed as NA ‐ AION , before involvement of the orbit and the CNS . Systemic involvement should be ruled out in any case of AION with an atypical evolution.