Eales' disease with neurological involvement. Part 2. Pathology and pathogenesis

Area of Science:

• Neuroscience
• Ophthalmology
• Pathology

Background:

• Eales' disease is a rare condition characterized by inflammation of retinal blood vessels.
• Central nervous system (CNS) involvement is an uncommon but severe manifestation.
• This case presents a detailed neuropathologic examination of Eales' disease with extensive CNS sequelae.

Observation:

• The patient exhibited retinal vasculopathy, progressing to brain stem, cerebellar, and spinal cord disease over four years.
• Neuropathologic findings included chronic retinal inflammation and optic nerve demyelination.
• The brain stem and cerebellum showed significant vasculopathy affecting venous structures, with associated demyelination in surrounding tissues.

Findings:

• Extensive vasculopathy and venous changes were observed in the brain stem and cerebellum.
• Perivenular demyelination, with relative axon preservation, was noted, particularly in the cerebellum.
• Spinal cord examination revealed similar, though less pronounced, venopathy, with characteristic ascending and descending degeneration patterns.

Implications:

• This case underscores the potential for Eales' disease to cause severe and widespread neurological damage.
• Understanding the neuropathologic basis of CNS involvement is crucial for managing patients with Eales' disease.
• Further research into the mechanisms of vasculopathy and demyelination in Eales' disease is warranted.