J Clin Neurol.  2016 Apr;12(2):188-193. 10.3988/jcn.2016.12.2.188.

Cerebral Cortex Involvement in Neuromyelitis Optica Spectrum Disorder

• Kim W
• Lee JE
• Kim SH
• Huh SY
• Hyun JW
• Jeong IH
• Park MS
• Cho JY
• Lee SH
• Lee KS
• Kim HJ

• KMID: 2354139
• DOI: http://doi.org/10.3988/jcn.2016.12.2.188

Abstract

BACKGROUND AND PURPOSE
Brain lesions involving the cerebral cortex are rarely described in patients with neuromyelitis optica spectrum disorder (NMOSD), in contrast to multiple sclerosis. We investigated cerebral cortex involvement using conventional brain magnetic resonance imaging (MRI) in anti-aquaporin-4 (AQP4)-antibody-positive NMOSD patients.
METHODS
The study enrolled 215 NMOSD patients who were seropositive for the anti-AQP4 antibody from 5 referral hospitals, and retrospectively analyzed their demographic, clinical, and MRI findings. Abnormal cerebral cortex lesions on brain MRI were identified by a neuroradiologist and two neurologists using consensus.
RESULTS
Most of the 215 enrolled patients (87%) were female. The median age at onset was 22.5 years (range: 15-36 years) and the mean follow-up duration was 123 months. Brain lesions were found in 143 of 194 patients (74%) in whom MRI was performed during follow-up. Brain lesions involving the cerebral cortex were identified in 6 of these 194 patients (3.1%). Five of the patients were female, and the six patients together had a median age of 29 years (range: 15-36 years) at the time of lesion presentation. Three of them showed leptomeningeal enhancement in the lesions. At presentation of the cortex-involving lesions, five of these patients were not being treated at the time of presentation, while the sixth was being treated with interferon-beta.
CONCLUSIONS
Although rare, cortical involvement occurs in NMOSD and is commonly combined with leptomeningeal enhancement. We speculate that this occurs only in patients who are not treated appropriately with immunosuppressant drugs.

Keyword

neuromyelitis optica; neuromyelitis optica spectrum disorder; magnetic resonance imaging; cerebral cortex

MeSH Terms

Brain
Cerebral Cortex*
Consensus
Female
Follow-Up Studies
Humans
Interferon-beta
Magnetic Resonance Imaging
Multiple Sclerosis
Neuromyelitis Optica*
Referral and Consultation
Retrospective Studies
Interferon-beta

Figure

• 

  Fig. 1 Brain abnormalities in neuromyelitis optica spectrum disorder (NMOSD) patients on fluid-attenuated inversion recovery (FLAIR) (upper rows) and T1-weighted enhanced (lower rows) magnetic resonance imaging (MRI) scans obtained at the time of cortical lesion presentation. The brain MRI scans of Patient 2 have been described previously.15 The lesions involving the cerebral cortex are accompanied by leptomeningeal (Patients 1, 2, and 3) or cortical (Patient 4) enhancement, or no enhancement (Patients 5 and 6). In addition to the cortex-involving lesions, other characteristic brain lesions of NMOSD are evident. In representative follow-up FLAIR images (Patients 1, 2, 4, 5, and 6), obtained 3 to 80 months after the presentation of the cortex-involving lesions, most of the lesions had disappeared or become faint—compared to the correlated slices in the previous images (*)—in all patients except Patient 5. mo: months.

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