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Sean J. Pittock, MD; Brian G. Weinshenker, MD; Claudia F. Lucchinetti, MD; Dean M. Wingerchuk, MD; John R. Corboy, MD; Vanda A. Lennon, MD, PhD

Arch Neurol. 2006;63:964-968.

Background  Neuromyelitis optica (NMO)–IgG is a specific autoantibody marker for NMO. It binds selectively to aquaporin 4 (AQP4), which is highly concentrated in astrocytic foot processes at the blood-brain barrier and is not restricted to optic nerve and spinal cord. Although it is conventionally believed that the brain is spared, brain imaging abnormalities are not uncommon in patients with NMO.

Objective  To investigate the location of brain lesions that are distinctive for NMO with respect to the localization of AQP4 in mammalian brain.

Design  Observational, retrospective case series.

Setting  Clinical serologic cohort of patients tested for NMO-IgG for whom brain MRI images were available.

Patients  We identified 120 patients seropositive for NMO-IgG for whom brain magnetic resonance images were available.

Main Outcome Measure  Magnetic resonance imaging abnormalities.

Results  In 8 patients we observed recurring and distinctive magnetic resonance imaging abnormalities in the hypothalamic and periventricular areas that corresponded to brain regions of high AQP4 expression.

Conclusion  The distribution of NMO-characteristic brain lesions corresponds to sites of high AQP4 expression.

Author Affiliations: Departments of Neurology (Drs Pittock, Weinshenker, Lucchinetti, and Lennon), Laboratory Medicine and Pathology (Drs Pittock and Lennon), and Immunology (Dr Lennon), Mayo Clinic College of Medicine, Rochester, Minn; Department of Neurology, Mayo Clinic, Scottsdale, Ariz (Dr Wingerchuk); and University of Colorado–Denver and Health Sciences Center and Department of Neurology, Denver Veteran's Affairs Medical Center, Denver (Dr Corboy).

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