Progressive cerebral atrophy in neuromyelitis optica

Area of Science:

• Neuroimmunology
• Neurology

Background:

• Neuromyelitis optica (NMO) is an autoimmune disorder typically characterized by inflammatory attacks on the optic nerves and spinal cord.
• While NMO is primarily considered relapsing-remitting, its later stages can involve neurodegeneration.

Observation:

• Two elderly NMO patients presented with secondary progressive tetraparesis and cognitive impairment.
• Both patients exhibited elevated cerebrospinal fluid (CSF) protein and myelin basic protein, alongside extremely high serum anti-aquaporin-4 (AQP4) antibody titers.

Findings:

• NMO pathology rarely manifests as a secondary progressive clinical course due to axonal degeneration.
• Severe intrathecal inflammation and extensive neuroglial destruction in NMO can precipitate secondary progressive clinical courses with cerebral atrophy.

Implications:

• These cases highlight that severe inflammation in NMO can drive neurodegenerative changes, leading to progressive cerebral atrophy.
• Recognizing this degenerative potential is crucial for understanding NMO's long-term prognosis and management strategies.