Anti NMO-IgG (Aquaporin-4)Antibodies

Any Other Information

1) What other tests can be done to diagnose Neuromyelitis Optica?

Other tests are

1. Myelin oligodendrocyte glycoprotein(MOG) antibodies
2. Oligoclonal Bands in CSF.
    

2) What is the sample required for testing Myelin oligodendrocyte glycoprotein(MOG) antibodies?

Myelin oligodendrocyte glycoprotein(MOG) antibodies can be tested from both the serum and CSF.


3) What is role of MOG antibodies in Neuromyelitis Optica?

Myelin oligodendrocyte glycoprotein(MOG) is a membrane protein expressed on the oligodendrocyte cell surface, located on the outermost surface of myelin sheaths. It is primary target antigen involved in immune mediated demyelination in patients with NMOSD- Neuromyelitis Optica Spectrum Disorder. Approximately 80% of patients with Neuromyelitis Optica are seropositive for Aquaporin -4(AQP4 /NMOIgG). Of the remaining 20%, about to one third harbor MOGIgG .30 TO 40% of pediatric patients with inflammatory demyelinating disease of the CNS exhibit autoantibodies against MOG at disease onset. Seropositivity with MOG IgG predicts a relapsing phenotype and warrants immunosuppressive therapy. It is important to differentiate NMOSD from Multiple Sclerosis as MS therapies may worsen MOG IgG associated inflammatory demyelinating disorders.


4) What is Neuromyelitis Optica Spectrum Disorder?

NMOSD is an etiologically heterogenous syndrome characterized predominantly by acute optic neuritis ON ( optic nerve inflammation) and spinal cord myelitis. Symptoms of ON and myelitis can occur at the same time or be successively. If patients remain untreated, it can present as a relapsing disease. In rare conditions, it can be associated with other autoimmune disorders like Connective tissue disorders, paraneoplastic diseases or infections. Sometimes cause may remain unknown (idiopathic NMO).


5) What are the symptoms of Neuromyelitis Optica?

Commonly initial manifestations are of myelitis spinal cord myelitis which can present as muscle weakness and reduced sensations. Patients also present with loss of bladder and bowel control as well as erectile dysfunction. This myelitis can be transverse that is involving the cross –section of spinal cord resulting in bilateral symptoms.

Second common manifestations of Optic Neuritis (ON) include visual impairment with decreased visual acuity. Visual field defects or loss of color vision can also be seen. On comparing Idiopathic ON and ON due to multiple sclerosis, ON due to NMOSD has often severe visual loss, involves bilaterally and also permanent visual deficits. Involvement of the brainstem or upper cervical spinal cord can cause respiratory distress.


6) What are the risk factors for Neuromyelitis Optica?

Risk factors include female sex and genetic factors.
 

7) Which age group is more susceptible for Neuromyelitis Optica?

Median age for AQP-4 IgG related NMO is 40 years.
Median age for MOGIgG related NMO is 31 years.

 

8) What are the differential diagnosis of Neuromyelitis Optica?

Most common differential is multiple sclerosis and less often other autoimmune disorders like connective tissue disorders, paraneoplastic disorders, etc.


9) What is demyelination?

Damage to the protective coating of nerve cells ( myelin) is known as demyelination.


10) What is the treatment for NMO?

Acute attacks are treated with short courses of high dose IV corticosteroids like methylprednisolone. If initiated in early course of disease, it has shown to improve vision related changes following acute attacks. Plasmapheresis has shown to be effective in patients who do not respond to steroids or to prevent the progress of disease following steroid administration. Other drugs used are monoclonal antibodies like Eculizumab, Rituximab, etc.

11) What is the prognosis of NMO?

Though this condition is treatable, but chances of complete cure are very rare.
Usually, some amount of improvement is seen, however residual signs and disability may persist.
This disease can be monophasic that is single episode with permanent remission. However, many patients (approximately 85%) relapse with repeated attacks of transverse myelitis and optic neuritis. In monophasic condition, myelitis and neuritsis can occur simultaneously or within a gap of few days. However, relapsing form usually takes weeks or months after initial attacks and motor recovery is better tham the initial transverse myelitis episode.
Frequency of relapse in 55% patients is within one year and in 90% patients in the first five years.


12) What can you interpret from oligoclonal band testing in CSF?

Oligoclonal bands are bands of immunoglobulins analyzed in a patient’s serum or CSF. They are useful in diagnosis of various neurological disorders, especially in multiple sclerosis. Normally it is assumed that those proteins present in CSF but not in serum, are produced intrathecally( inside CNS). The intrathecal synthesis is indicated by the presence of IgG band in the immunofixation pattern of CSF that are not present in the serum pattern of the patient.

 

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