The visual outcome and efficacy of current therapies for neurosarcoidosis with anterior visual pathway involvement: A systemic review

Purpose

To examine the clinical characteristics of neurosarcoidosis with anterior visual pathway involvement (NSAVP), assess the effectiveness of current treatments, and provide insights into the demographics, symptoms, and treatment outcomes to better guide clinical management of NSAVP.

Methods

The databases PubMed, Embase, MEDLINE, CINAHL and Ovid were searched to identify relevant studies. Statistical analyses were performed in R.

Results

Thirty-three studies involving 334 patients and 404 eyes were included in the meta-analysis. Our findings indicate that NSAVP predominantly affects females. Bilateral involvement was observed in 46 % of cases. Visual loss was the most common presenting symptom, affecting 94 % of patients; 78 % had no prior symptoms of systemic involvement. Three percent had isolated AVP manifestations. Fewer than 17 % had co-existing orbital or cranial nerve signs and 29 % had co-existing uveitis.

Sixty-three percent had an MRI abnormality of the AVP. The most frequently reported laboratory abnormality was an elevated white cell count in cerebrospinal fluid (CSF), followed by elevated protein in CSF and elevated serum ACE level.

Following treatment, 57 % experienced visual improvement. The visual improvement rate was similar for patients receiving steroids alone (46 %) and those receiving combined steroid and immunosuppressive therapy (44 %). Combined therapy was associated with a lower the incidence of no light perception (NLP, 1 % vs. 16 %), a higher proportion with vision better than 20/200 (70 % vs. 62 %) and a lower incidence of relapse (16 % vs. 40 %).

Conclusion

NSAVP predominantly affects females, with visual loss being the most common presenting symptom. Combination therapy of steroids and immunosuppressants was associated with better outcomes than steroids alone, including lower incidence of relapse and fewer cases of no light perception.