Table 3.

Proposed diagnostic guidelines for neuro-ophthalmic sarcoidosis.

Definite		Presence of non-caseating granulomatous inflammation on tissue biopsy and neuro-ophthalmic involvement with clinical features compatible with sarcoidosis
Probable	Clinical syndrome suggestive of neuro-ophthalmic sarcoidosis in the absence of a confirmatory biopsy with each of the following: Orbital or intracranial infiltrative or inflammatory findings including, but not limited to, optic neuropathy, EOM dysmotility, proptosis, or ptosis as presented in this case series Imaging evidence of inflammation suggestive of sarcoid (at least 1): a. Abnormal gallium-67 citrate body scan b. Enhancement of the ocular adnexa on MRI c. CXR or CT scan with lesions consistent with sarcoidosis Laboratory evidence of inflammation (at least 1): a. Elevated ACE b. Abnormal PFTs or elevated urine calcium c. Elevated levels of CSF protein and/or cells Exclusion of alternative diagnoses such as infectious, inflammatory (e.g., multiple sclerosis, idiopathic pachymeningitis, seronegative vasculitis, orbital pseudotumour), or neoplastic aetiologies

EOM extraocular muscle; MRI magnetic resonance imaging; CXR chest x-ray; CT computed tomography; PFT pulmonary function test; ACE angiotensin-converting enzyme