Journal Reading Neuromyelitis Optica

8/7/2019 Journal Reading Neuromyelitis Optica

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Presented By : dr.ZakiSupervised By : dr.Nurdjaman Nurimaba Sp.S(K)Taken From : J Neuro-Ophthalmol, Vol. 27, No. 1, 2007


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Abstract :

Neuromyelitis optica (NMO), or Devic disease, has been

distinguished from multiple sclerosis (MS) by the presence of optic

neuritis that is usually bilateral, simultaneous, and often

severe,myelopathic ndings accompanied by longitudinally extensive

spinal cord imaging abnormalities, no brain imaging abnormalities

typical of MS, and often rapid progression to debility and even death.

Researchers at the Mayo Clinic have identied an immunoglobulin

marker of NMO (the NMO antibody) that binds selectively to the

aquaphorin-4 water channel and may play a causative role.


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This marker has been found in Japanese patients with opticospinal MS,

prompting the suggestion that NMO and Japanese opticospinal MS are

the same disorder. The NMO antibody, which predicts frequent relapse

of mye-lopathy and optic neuritis, is also found in patients with lupus

erythematosus and Sjogren syndrome who also have severe optic

neuritis and longitudinally extensive myelitis. Because this antibody is

also found in patients with optic neuritis and myelitis who have brain

signal abnormalities atypical of MS, the diagnosis of NMO has been

revised to allow inclusion of these brain imaging abnormalities.

Abstract :


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THE THREE PHENOTYPIC FORMS OF NEUROMYELITIS OPTIC

1.Multiple Sclerosis

2.True Neuromyelitis Optica

3.Other Autoimmune Diseases


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WHY IS THE DISTINCTION BETWEEN NEUROMYELITISOPTICA AND MULTIPLE SCLEROSIS IMPORTANT ?

1. Neuromyelitis Optica has a worse outcomethan Multiple Sclerosis

2. Neuromyelitis Optica responds toimmunosuppressive therapy


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THE NEUROMYELITIS OPTICA ANTIBODY

Beginning in 2003, Mayo Clinic investigators began publishing articles on NMO that

described a serum autoantibody marker and redefined the clinical criteria for NMO

diagnosis.

In 2004, Lennon et al identified an IgG marker of NMO that is purported to allow distinction

from MS. The investigators prospectively tested serum samples from 124 patients with

clinical NMO or high risk for NMO. In the first subgroup were 102 North American patients.

Of these, 45 had definite NMO, or remained at high risk for NMO and 22 were found to haveclassic MS. NMO was defined as optic neuritis, acute myelitis, and no imaging evidence of

demyelinating disease except in the optic nerves and spinal cord. Supportive evidence

included either one major criterion or two minor criteria. The major criteria were 1) normal

brain MRI at outset, 2) a spinal cord MRI signal abnormality extending three or more

vertebral segments, and 3) CSF pleocytosis >50 10 -6 white blood cells/L or >5 10-6

neutrophils/L. The minor criteria were 1) bilateral optic neuritis, 2) severe optic neuritis

with irreversible visual acuity loss worse than 20/200 in at least one eye, and 3) severe

weakness in at least one limb.


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THE NEUROMYELITISOPTICA ANTIBODYANDTHE AQUAPORIN-4

WATERCHANNEL

The NMO IgG autoantibody was subsequently shown to bind selectively

to the aquaporin-4 (AQP4) water channel, a component of the

dystroglycan protein complex located in astrocytic foot processes at

the blood-brain barrier. Because of the location of the antigen, it isspeculated that this NMO IgG is not merely a marker but a causative

agent, as is the case, for example, with the muscle acetylcholine

receptor antibodies of myasthenia gravis and the P/Q-type calcium

channel antibodies associated with Lambert-Eaton syndrome. AQP4 isthe first water channel-specific autoantibody to be identified.


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LUPUS ERYTHEMATOSUS, SJGREN SYNDROME, AND THE

NEUROMYELITIS OPTICA ANTIBODY

Pittock et al and Weinshenker et al have identified an NMO-like disorder in some

patients with SLE and SS and in some patients who are antinuclear antibody

(ANA)-positive or extractable nuclear antibody (ENA)-positive without fulfilling

the clinical criteria for SLE or SS. These patients have optic neuritis and

extensive spinal cord lesions and are NMO IgG-positive. Pittock et al tested

serum samples from patients with NMO (79), recurrent longitudinally extensivetransverse myelitis (rLETM) (44), SLE (2), and SS (14) for NMO IgG, ANA, and

ENA. Approximately three fourths of patients with NMO and rLETM were NMO

IgG-positive. Half of these patients were also ANA-positive and about one

seventh were ENA-positive. Most did not fulfill the clinical criteria for SLE or SS.

There was a higher frequency of nonorgan-specific autoantibodies in NMO IgG-

positive patients than in NMO IgG-negative patients. Nonorgan-specific

autoantibodies may reflect a more intense autoimmune response.


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DEFINITION OF NEUROMYELITIS OPTIC


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REVISING THE DEFINITION OF NEUROMYELITIS OPTIC


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Journal Reading Neuromyelitis Optica

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