Journal Reading Neuromyelitis Optica
-
Upload
badbiggyboy -
Category
Documents
-
view
226 -
download
0
Transcript of Journal Reading Neuromyelitis Optica
-
8/7/2019 Journal Reading Neuromyelitis Optica
1/20
Presented By : dr.ZakiSupervised By : dr.Nurdjaman Nurimaba Sp.S(K)Taken From : J Neuro-Ophthalmol, Vol. 27, No. 1, 2007
-
8/7/2019 Journal Reading Neuromyelitis Optica
2/20
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.
-
8/7/2019 Journal Reading Neuromyelitis Optica
3/20
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 :
-
8/7/2019 Journal Reading Neuromyelitis Optica
4/20
THE THREE PHENOTYPIC FORMS OF NEUROMYELITIS OPTIC
1.Multiple Sclerosis
2.True Neuromyelitis Optica
3.Other Autoimmune Diseases
-
8/7/2019 Journal Reading Neuromyelitis Optica
5/20
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
-
8/7/2019 Journal Reading Neuromyelitis Optica
6/20
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.
-
8/7/2019 Journal Reading Neuromyelitis Optica
7/20
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.
-
8/7/2019 Journal Reading Neuromyelitis Optica
8/20
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.
-
8/7/2019 Journal Reading Neuromyelitis Optica
9/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
10/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
11/20
DEFINITION OF NEUROMYELITIS OPTIC
-
8/7/2019 Journal Reading Neuromyelitis Optica
12/20
REVISING THE DEFINITION OF NEUROMYELITIS OPTIC
-
8/7/2019 Journal Reading Neuromyelitis Optica
13/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
14/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
15/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
16/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
17/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
18/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
19/20
-
8/7/2019 Journal Reading Neuromyelitis Optica
20/20