Titel | Sarcoidosis of orbita and central nervous system presenting as a non-arteritic ischemic optic neuropathy. |
Abstract Nr. | 2037 |
Doel | To describe a patient who presented with non-arteritic ischemic optic neuropathy (NA-AION) as an initial manifestation of neurosarcoidosis. |
Methodes | A 70-year-old woman who presented with loss of vision in the LE and normal neuroimaging, was initially diagnosed as a NA-AION. An extensive workup included MRI orbita and PET/CT scan. |
Resultaten | At the initial presentation, the LE showed loss of vision and papilloedema. Further ophthalmological examination and CT scan of the brain were normal. 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. |
Conclusie | Sarcoidosis is a multisystemic disease characterized by granulomatous inflammation. Uveitis is the most common ocular manifestation. 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. |
Naam | DE BRUYN |
Initialen | D |
Instituut | Dpt Ophthalmology, Ghent Univ Hosp |
Stad | Ghent |
Naam | NINCLAUS |
Initialen | V |
Instituut | Dpt Ophthalmology, Ghent Univ Hosp |
Stad | Ghent |
Naam | LEROY |
Initialen | BP |
Instituut | Dpt Ophthalmology & Ctr for Med Gen, Ghent Univ Hosp and Children's Hospital of Philadelphia |
Stad | Ghent and Philadelphia |
Naam | DE SCHRYVER |
Initialen | I |
Instituut | Dpt Ophthalmology, Ghent Univ Hosp |
Stad | Ghent |
Naam | DE ZAEYTIJD |
Initialen | J |
Instituut | Dpt Ophthalmology, Ghent Univ Hosp |
Stad | Ghent |