A rare presentation of neurosarcoidosis as the superior orbital fissure syndrome

Abstract

We are reporting a rare case of neurosarcoidosis that presented with superior orbital fissure syndrome. A 51-year-old Indian woman who suffered from 2 weeks duration, insidious onset gradually progressive left sided headache limited to forehead, left periorbital pain, diplopia on looking toward left, and numbness on the left forehead was referred to our hospital. Ophthalmic findings included left oculomotor nerve, trochlear nerve and abducens nerve palsies, absent corneal reflex, and loss of touch, pain and temperature sensation in V1 distribution. Brain and orbit magnetic resonance imaging (MRI) showed contrast enhancing area at the left orbital apex with normal optic nerve. Normal CSF analysis. High ESR, and chest x-ray showing non-homogenous patchy opacities in bilateral lung fields leaded us for neoplastic screening. Chest computed tomography (CT) revealed multiple ill-defined irregular lung parenchymal nodular opacities of varying sizes involving bilateral lungs with enlarged mediastinal and bilateral axillary lymph nodes. Elevated angiotensin converting enzyme level was found. Biopsy of the axillary lymph node showed non-caseating granulomatous lymphadenitis with giant cells containing schaumann bodies and asteroid bodies in the cytoplasm. These findings indicating an inflammatory response induced by sarcoidosis. After the initiation of steroid treatment, the patient experienced complete remission without any recurrence. When examining a case presenting with superior orbital fissure syndrome with contrast enhancing MRI lesion at the orbital apex, the additional chest CT to exclude neoplastic lesions can detect pulmonary sarcoidosis and extraneural lesions. These may avoid delays in the diagnosis and management of neurosarcoidosis.