This review describes the clinical features that distinguish between major immune-mediated and inflammatory syndromes affecting the brainstem, many of which have ophthalmic effects.

Study design

The authors searched the literature for studies describing the MRI features or clinical characteristics of individuals with multiple sclerosis, neuromyelitis optica spectrum disorder, neuro-Behçet disease, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, neurosarcoidosis, Susac syndrome or histiocytic disorders.

Outcomes

Patients presenting with brainstem symptoms and signs may inspire a number of common and less common differential diagnoses, and arriving at an accurate diagnosis can be challenging. The authors’ analysis focused on clinical features and MRI clues that help to distinguish among the different brainstem conditions. They conclude that accurate diagnosis is key to determining appropriate treatments and to limiting neurologic disability.

Limitations

This is a review of the literature, which limits the amount of data available to analyze and can complicate comparison of findings across studies with different designs.

Clinical significance

A better understanding of these various disorders is essential to the practice of neuro-ophthalmology, neurology and ophthalmology because many of these conditions have associated ophthalmologic signs and symptoms.