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Original Contributions |

Brain Involvement in Neuromyelitis Optica Spectrum Disorders

Koon Ho Chan, MD, FRCP; C. T. Tse, MBBS, MRCP; C. P. Chung, MBBS, MRCP; Raymand L. C. Lee, FRCR; J. S. C. Kwan, MPhil; P. W. L. Ho, PhD; J. W. M. Ho, PhD
Arch Neurol. 2011;68(11):1432-1439. doi:10.1001/archneurol.2011.249.
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Background Neuromyelitis optica spectrum disorders (NMOSDs) are severe inflammatory demyelinating disorders of the central nervous system. Brain involvement is increasingly recognized.

Objective To study brain involvement in NMOSDs among Hong Kong Chinese patients.

Design Retrospective study of patients with NMOSDs.

Setting Tertiary medical center in Hong Kong.

Patients Thirty-four Hong Kong Chinese patients with NMOSDs of 2 years or longer were recruited.

Interventions Brain and spinal cord magnetic resonance imaging was performed during NMOSD attacks and was repeated yearly for the first 3 years.

Main Outcome Measures We evaluated clinical features of NMOSDs associated with brain involvement and brain lesions on magnetic resonance imaging.

Results Among 34 patients with NMOSDs of 2 years or longer, 20 (59%) had brain involvement. The mean age at onset among these 20 patients was 45.6 years (age range, 19-67 years); 18 were women. Eleven patients (32% of all the patients with NMOSDs) had clinical manifestation of brain involvement, 19 patients (56%) had brain abnormalities on magnetic resonance imaging consistent with inflammatory demyelination, and 2 patients (6%) fulfilled criteria for multiple sclerosis. Clinical manifestation of brain involvement included the following: trigeminal neuralgia; vomiting, vertigo, ataxia, dysphagia, and tetraparesis from lesions around the third and fourth ventricles and aqueduct; homonymous hemianopia, aphasia, hemiparesis, and cognitive impairment from extensive hemispheric white matter lesions; and ataxia, diplopia, hiccups, facial sensory loss, internuclear ophthalmoplegia, hemisensory loss, and hemiparesis from other lesions in the midbrain, pons, cerebellar peduncles, and medulla. Eight patients (24%) developed brainstem encephalitis clinically, and brainstem encephalitis was the initial clinical manifestation in 6 patients (18%). Brain abnormalities on magnetic resonance imaging were detected in brainstem in 15 patients (44%), hemispheric periventricular white matter in 7 patients (21%), deep white matter in 7 patients (21%), corpus callosum in 4 patients (12%), subcortical white matter in 3 patients (9%), thalamus in 2 patients (6%), hypothalamus in 1 patient (3%), basal ganglia in 1 patient (3%), internal capsule in 1 patient (3%), periaqueductal gray matter in 1 patient (3%), and around the third and fourth ventricles in 1 patient (3%); large confluent lesions were detected in 2 patients (6%).

Conclusion Brain involvement manifesting clinically as brainstem encephalitis is common among Hong Kong Chinese patients with NMOSDs.

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Figure 1. Brain magnetic resonance images in patient 1. Axial T2-weighted images revealed hyperintense lesions in the periaqueductal gray matter and region around the fourth ventricle (A and B), and coronal fluid-attenuated inversion recovery images revealed hyperintense lesions around the aqueduct and fourth ventricle (C) and in the medulla (D).

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Figure 2. Brain magnetic resonance images in patient 2. On initial presentation, a sagittal T2-weighted image revealed a hyperintense pontine lesion (A), which was at the midline of the anterior pons, as shown on an axial T2-weighted image (B). During a relapse 8 years after clinical onset, a T2-weighted image revealed a hyperintense lesion at the left cerebral peduncle (C); bilateral middle cerebellar peduncles were also involved, as shown on a coronal fluid-attenuated inversion recovery image (D).

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Figure 3. Brain magnetic resonance image of a patient with recurrent brainstem encephalitis and subclinical myelitis who was seen initially with brainstem encephalitis. A T2-weighted image revealed a hyperintense lesion at the right dorsolateral pons involving the right superior cerebellar peduncle (A), which was also shown on a coronal fluid-attenuated inversion recovery image (B). The lesion was enhanced with gadolinium (C).

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Figure 4. Brain magnetic resonance imaging of a patient with extensive hemispheric white matter involvement. An axial T2-weighted image revealed a large confluent white matter lesion over the left occipital region (A), which was also shown on a fluid-attenuated inversion recovery (FLAIR) image (B). Multiple white matter lesions in bilateral hemispheres were shown on a T2-weighted image (C) and a FLAIR image (D); some of the lesions were cystic (D). Gadolinium enhancement was noted in some hemispheric white matter lesions, which were compatible with cloud-like enhancement (E). The corpus callosum was involved, with an enhancing lesion shown on a sagittal T1-weighted image (F). This patient fulfilled Barkhof criteria for multiple sclerosis and was seropositive for aquaporin-4 autoantibodies.

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