Abstract

BackgroundNeuromyelitis 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.MethodsThe 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.ResultsBrainstem 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).ConclusionsNMO 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.

Highlights

  • 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

  • Brainstem lesions were observed in 25 patients, 18 in medulla oblongata (11 in area postrema)

  • Brainstem lesions were associated with anti-aquaporin 4 (AQP4) antibody positivity

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Summary

Introduction

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. Neuromyelitis optica (NMO) includes transverse myelitis, optic neuritis and brain lesions. Extensive transverse myelitis (LETM) is an important component of the clinical diagnosis of NMO. Neuromyelitis optica (NMO) is characterized by inflammation of the optic nerve and the spinal cord [1]. Discovery of serum immunoglobulin G autoantibody towards the water channel aquaporin 4 (AQP4) led to the recognition of NMO patients with clinical signs and/or lesions in the CNS outside of the optic nerve and spinal cord [2,3,4]. The demonstration of anti-AQP4 antibodies/NMO-IgG is obligatory in the diagnosis of the NMO spectrum disease, which includes patients with clinical signs and/or MRI lesions in the CNS outside of the optic nerve and spinal cord [4]. A number of studies have shown brain abnormalities as detected by MRI in

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