Reappraisal of brain MRI features in patients with multiple sclerosis and neuromyelitis optica according to anti-aquaporin-4 antibody status

Devic's neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by predominant involvement of the optic nerves and spinal cord. In Asian countries relapsing NMO has been known as opticospinal multiple sclerosis. It has long been debated if NMO is a variant of multiple sclerosis (MS) or a distinct disease. Recent studies have shown that NMO has more frequently a relapsing course, and results from attack to aquaporin-4 which is the dominant water channel in the central nervous system, located in foot processes of the astrocytes. Distinctive pathological features of NMO include perivascular deposition of IgG and complement in the perivascular space, granulocyte and eosinophil infiltrates and hyalinization of the vascular walls. These features distinguish NMO from other demyelinating diseases such as MS and acute demyelinating encephalomyelopathy. An IgG-antibody that binds to aquaporin-4, named NMO-IgG has high sensitivity and specificity. Magnetic resonance imaging (MRI) studies have revealed that more frequently there is a long spinal cord lesion that extends through three or more vertebral segments in length. Brain MRI lesions atypical for MS are found in the majority of cases. Treatment in the acute phase includes intravenous steroids and plasma exchange therapy. Immunosupressive agents are recommended for prophylaxis of relapses.

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The expanded spectrum of neuromyelitis optica: evidences for a new definition

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2012001000010 ◽

2012 ◽

Vol 70 (10) ◽

pp. 807-813 ◽

Cited By ~ 20

Author(s):

Marco A Lana-Peixoto ◽

Dagoberto Callegaro

Keyword(s):

Optic Nerve ◽

Optic Neuritis ◽

Neuromyelitis Optica ◽

Brain Mri ◽

Transverse Myelitis ◽

Aquaporin 4 ◽

Longitudinally Extensive Transverse Myelitis ◽

Index Event ◽

Aquaporin 4 Antibody ◽

Extensive Transverse Myelitis

Neuromyelitis optica (NMO) has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM). Identification of aquaporin-4 antibody (AQP4-IgG) has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD) has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM). Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

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Atypical Neuromyelitis Optica Spectrum Disorder With Diffuse Cerebral Abnormalities: A Case Report

Journal of Central Nervous System Disease ◽

10.1177/1179573520973819 ◽

2020 ◽

Vol 12 ◽

pp. 117957352097381

Author(s):

Braeden D Newton ◽

Orhun Kantarci ◽

Darin T Okuda

Keyword(s):

Neuromyelitis Optica ◽

African American Woman ◽

Brain Mri ◽

American Woman ◽

Spectrum Disorder ◽

Aquaporin 4 ◽

High Signal ◽

Neuromyelitis Optica Spectrum Disorder ◽

Radiological Criteria ◽

Mri Features

The recent expansion of the radiological criteria and the use of a highly specific biomarker, anti-aquaporin 4-IgG (AQP4 IgG), has significantly improved the ability of clinicians to provide a timely and accurate diagnosis for neuromyelitis optica spectrum disorder (NMOSD), especially when faced with an abnormal disease presentation. Here, we report on the 5-year clinical experience of a 69-year-old right-handed African American woman who initially presented following symptoms suggestive of transient global amnesia. Her clinical history was only remarkable for a single episode of visual decline with poor recovery experienced 35 years prior, with prior unrevealing serological investigations. Brain MRI features were significant for diffuse, bilateral white matter abnormalities throughout the supratentorial, deep gray matter, and infratentorial regions. Spinal cord imaging studies were within normal limits with no intramedullary high-signal abnormalities identified. Serological studies were significant for the presence of anti-aquaporin 4-IgG. The clinical features were supportive of the diagnosis of NMOSD. The data provided here highlight both the clinical and radiological heterogeneity of NMOSD.

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Cortical oscillopsia without nystagmus, an isolated symptom of neuromyelitis optica spectrum disorder with anti-aquaporin 4 antibody

Multiple Sclerosis Journal ◽

10.1177/1352458511414149 ◽

2011 ◽

Vol 18 (2) ◽

pp. 244-247 ◽

Cited By ~ 8

Author(s):

Sung-Min Kim ◽

Ji-Soo Kim ◽

Young Eun Heo ◽

Hye-Ran Yang ◽

Kyung Seok Park

Keyword(s):

Spinal Cord ◽

Neuromyelitis Optica ◽

Spectrum Disorder ◽

Aquaporin 4 ◽

Neuromyelitis Optica Spectrum Disorder ◽

Ocular Manifestation ◽

Initial Manifestation ◽

Ocular Symptoms ◽

Head Tremor ◽

Aquaporin 4 Antibody

Neuromyelitis optica (NMO), mainly affecting optic nerve and spinal cord, can also manifest diverse ocular symptoms due to brain abnormalities. We present a cortical oscillopsia without nystagmus or head tremor in a patient with neuromyelitis optica spectrum disorder (NMOSD) with anti-aquaporin 4 antibody. This rare ocular manifestation, which is easily underestimated owing to absence of the typical nystagmus, can be an initial manifestation of NMOSD.

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