Neurosarcoidosis, Coccidioidomycosis, or Both!

Abstract

Purpose: To report a case of neurosarcoidosis (NS) who was initially diagnosed as Coccidioidomycosis immitis (CI) infection.

Observations: A 57-year-old diabetic man presented with sudden painless diminution of vision, metamorphopsia, and color vision deficits in the left eye (OS) for one month. His vision was 20/20 in the right eye (OD) and 20/40 OS. Ophthalmic examination revealed left relative afferent pupillary defect, blurred optic nerve margin, creamy chorioretinal infiltration around the optic disc, and mild macular edema. OD examination was non-revealing. Chest CT scan with contrast showed calcified mediastinal lymph nodes, but biopsy of the lymph nodes was normal. Brain and orbit MRI demonstrated soft tissue abnormality with enhancement in left orbital apex with involvement of the extraocular muscles. CSF culture was negative, but complement fixation had positive titer of 1:2 for CI. The patient was diagnosed with CI meningitis, and antifungal therapy was initiated. Slight visual and symptomatic improvement was observed, which was not completely satisfactory. Biopsy of extraocular orbital muscle five months later revealed non-caseating granulomatous inflammation, leading to initiation of prednisone trial therapy. Nine months later, the patient was referred to a tertiary center owing to persistence of optic disc edema OS. PET CT was consistent with a diagnosis of sarcoidosis. Antifungal treatment was discontinued, and oral prednisone with methotrexate was initiated. Subsequently, methotrexate was replaced by infliximab to further manage ocular inflammation and neurologic symptoms which was effective. Vision was 20/20 OD and 20/30 OS at the most recent visit.

Conclusion and importance: Signs and symptoms of neurosarcoidosis and coccidioidomycosis can be similar and deceiving. The index case underscores importance of considering appropriate differential diagnoses in patients with similar symptoms and signs who may respond to preliminary designated treatment but not to the optimal extent. Considering such possibility could assist clinicians in managing the patients timely and efficiently.

Keywords: coccidioidomycosis; neurosarcoidosis; ocular involvement; sarcoidosis.

Figure 1

Axial view of magnetic resonance imaging (MRI) showed intraconal soft tissue and optic nerve sheath complex swelling [obscured the territories of different segments (Orange arrow)] (A). The positron emission tomography (PET) computed tomography demonstrated multiple fluorodeoxyglucose (FDG) avid lymph nodes above and below the diaphragm (red arrows) (B). MRI improved following the treatment [clear borders of segments (blue arrow)] (C).

Figure 2

At the baseline, standard and ultra-wide color fundus photographs of the right eye had a normal appearance; however, the left eye showed disc edema with blurred margin and peripapillary chorioretinal atrophy (A–D). Although macular SD-OCT of the right eye was normal (E and F), the left eye showed preserved foveal contour but intraretinal edema (yellow arrow) and disruption of the ellipsoid zone (EZ) line (G and H). Spectral-domain optical coherence tomography (SD-OCT) through the optic disc showed elevated optic disc and subretinal fluid (red arrow) at temporal (I and J). In addition to the SD-OCT of optic disc improvement, intraretinal edema decreased in size and echogenicity (blue and Orange arrows), and the disrupted EZ line partially improved following the treatment at 2 months, 5 months, and the most recent visit (K–S).

Figure 3

Early and late phase wide-angle fundus fluorescein angiography showed window defect on chorioretinal atrophy lesions with moderate optic disc hyperfluorescence (green arrow) at baseline (A and B), which slightly improved following treatment at 2 months (C and D).