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Open Access Research article

Modifications of longitudinally extensive transverse myelitis and brainstem lesions in the course of neuromyelitis optica (NMO): a population-based, descriptive study

Nasrin Asgari156*, Hanne Pernille Bro Skejoe2, Soeren Thue Lillevang3, Troels Steenstrup4, Egon Stenager15 and Kirsten Ohm Kyvik5

Author Affiliations

1 The Multiple Sclerosis Clinic of Southern Jutland (Vejle, Sonderborg, Esbjerg), Jutland Vejle Hospital, Vejle, DK-7100, Denmark

2 Radiology Clinic, Aleris-Hamlet Hospital, Copenhagen, Denmark

3 Department of Clinical Immunology, Odense University Hospital, Odense, Denmark

4 Department of Biostatistics, University of Southern Denmark, Odense, Denmark

5 Institute of Regional Research, University of Southern Denmark, Odense, Denmark

6 Institute of Molecular Medicine, University of Southern Denmark, J.B. Winsloewsvej 25,2, Odense C, DK-5000, Denmark

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BMC Neurology 2013, 13:33  doi:10.1186/1471-2377-13-33

Published: 8 April 2013

Abstract

Background

Neuromyelitis optica (NMO) includes transverse myelitis, optic neuritis and brain lesions. Recent studies have indicated that the brainstem is an important site of attack in NMO. Longitudinally extensive transverse myelitis (LETM) is an important component of the clinical diagnosis of NMO. The frequency of brainstem and LETM lesions, changes over time of LETM and the clinical consequences in the course of NMO have only been sparsely studied.

Methods

The study was a population-based retrospective case series with clinical and magnetic resonance imaging (MRI) follow-up of 35 patients with definite NMO and a relapsing-remitting course.

Results

Brainstem lesions were observed in 25 patients, 18 in medulla oblongata (11 in area postrema). Lesions in the pons, mesencephalon and diencephalon occurred in 10, 7 and 7 patients, respectively. Lesions were symptomatic in medulla oblongata and pons, asymptomatic in mesencephalon and diencephalon. Brainstem lesions were observed significantly more often in anti-aquaporin-4 (AQP-4) antibody positive than in seronegative patients (p < 0.002).

LETM was demonstrated by MRI of the spinal cord in 30/36 patients, 23/30 of whom had follow-up MRI of the spinal cord. Recurrent LETM was observed in five patients. In nine patients the LETM changed into multiple lesions during remission or treatment. Spinal cord atrophy was observed in 12/23 (52%) patients, correlating to Expanded Disability Status Scale (r = 0.88, p < 0.001).

Conclusions

NMO patients had frequent occurrence of brainstem lesions and LETM. Brainstem lesions were associated with anti-AQP4 antibody positivity. LETM lesions differentiated over time and the outcome included relapses, fragmentation and atrophy. Correlation was observed between spinal cord atrophy and neurological disability.

Keywords:
Neuromyelitis optica; Brainstem lesions; Area postrema; Longitudinally extensive transverse myelitis; Anti-aquaporin-4 antibody; Magnetic resonance imaging