A case of neuromyelitis optica-associated rhombodiencephalic encephalitis

Abstract

Background

Neuromyelitis optica spectrum disorder (NMOSD) classically presents with the triad of optic neuritis, transverse myelitis, and area postrema syndrome, while brainstem encephalitis and diencephalon involvement are rare but recognized manifestations of the disease.

Case presentation

A 59-year-old female with type 1 diabetes mellitus presented to the comprehensive ophthalmology clinic with acute headache and oblique diplopia, and later that week developed confusion and hypersomnolence. MRI brain with contrast demonstrated prominent T2 hyperintensity in the dorsal midbrain and brainstem, and the patient was ultimately found to be positive for Aquaporin-4 antibodies and diagnosed with NMOSD.

Case report

Very few reports exist of bilateral ptosis secondary to NMOSD, and this case also represents a novel presentation of cranial nerve III nuclear involvement. Thus, this case offers a unique diagnostic challenge due to the uncommon presentation of the disease.

Conclusions

Diplopia and ptosis in conjunction with somnolence, nausea, or vertigo, all signs of diencephalon involvement, should prompt consideration of the diagnosis of NMOSD.