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Neuromyelitis Optica (NMO)

Name of disease:

Neuromyelitis Optica (NMO)

ICD-10 diagnosis code:

G36.0

Causes:

Neuromyelitis Optica (NMO) is a rare disease with inflammation in optical nerve and spinal cord, Nervous system diseases, autoimmune disease The disease is autoimmune in nature which patients' self- antibodies mistakenly attack his/her own healthy cells and myelin material in the eyes and spinal cord.

Mode of inheritance:

The exact cause of neuromyelitis optica for the autoimmunity remains unknown Likely to be a combination of certain genetic variations, immune system, certain viral infection such as syphilis, HIV, chlamydia etc and unknown environmental factors. May relate to genetic variations in AQP4 gene. Genetic : de novo

Prevalence:

NMO has a worldwide distribution and estimated prevalence of 1-2/100,000.

Diagnosis:

A diagnosis of NMOSD is made based upon a detailed patient history, a thorough clinical evaluation, identification of characteristic physical findings, and a Variety of specialized tests.1.Magnetic resonance imaging (MRI) and computed tomography (CT or CAT) scans examine the condition of brain, spinal cord and eyes 2.Optical coherence tomography examines the retina of eyes. 3.Blood tests investigate the presence of aquaporin-4 antibody.4.Examination of the cerebrospinal fluid (CSF) to reveal presence of high levels of white blood cells and proteins in patients with neuromyelitis optica. Nervous system diseases

Age of onset:

Individuals of any age may be affected.

Common signs and symptoms:

The most common symptoms of include Optic neuritis: vision loss, eye pain, vision impairment, etc Spinal myelitis: paralysis of the lower or fourth limbs, urinary disorders, long periods of itching, nerve pain, etc Encephalitis: non-stop hiccups, nausea, vomiting,narcolepsy symptoms. Attacks of cerebral edema that may cause phycological confusion or coma, respiratory failure. Patients with NMO frequently have other systemic autoimmune disorders, such as systemic lupus erythematosus (SLE), Sjogren’s syndrome or myasthenia gravis

Available treatments (medicinal and non-medicinal):

Acute attacks are treated with high dose intravenous corticosteroids and if this fails, with plasma exchange. Long-term maintenance treatments are immunosuppressive drugs (azathioprine or mycophenolate mofetil), combined with corticosteroids in some patients, or rituximab therapy. Anti-spasticity agents to treat long term complication of spasticity that frequently develops in those with permanent motor deficits. Through symptomatic supportive treatment, patients' dysfunction can be improved and their quality of life can be improved

Disease management tips:

NMO has a long course and is prone to recurrence. Patients and their families should understand the importance of adherence to medication and improve medication compliance Avoid factors that can easily lead to recurrence, including cold, fever, infection, fertility, trauma, cold, tooth extraction,overwork, tiredness, heat irritation, mood swings, and random drug suspension, Vaccination should not be carried out at will, Keep an optimistic attitude and a cheerful mood, enhances patients’ confidence in overcoming the disease, thereby maximizing the patient’s quality of life and receive treatment with positive emotions. Cultivate the habit of exercise to maintain mobility Safe care Individual with NMO may have functional impairments, such as visual impairment and limb weakness. It is prone to accidents such as bumps, falls and falling from bed.1.Therefore, the layout of the home should be safe and reasonable, with sufficient light, flat ground, clean, no water, no obstructions, handrails in the bathroom, protective frames on both sides of the patient bed, lower the height of the bed, wear no slippers, wear flat shoes Or non-slip shoes. Medication care Follow the doctor's advice to take the medicine regularly, and do not reduce or stop using it at will. Diet care - Balanced nutrition and a simple diet Avoid crude fiber and hot hard foods and irritating foods. Eat low-fat, high-protein, vitamin-rich, high-potassium, and high-calcium diets, and foods rich in linoleic acid are appropriate. Drink more water and eat more meat, vegetables and fruits to increase protein and vitamin intake. Infection prevention care①Prevention of lung infection. Paralyzed patients are bedridden for a long time, prevent the occurrence of falling pneumonia. ②Keep your mouth clean, ③Keep the room clean and ventilate. ④Prevent bedsore care and turn over regularly, clean or replace dirty and wet mattresses and clothes in time, ⑤Prevention of urinary tract infections. For patients with mild urinary retention, to induce urination, and catherization when fail,⑥Prevent constipation. Due to spinal cord injury, bed paralysis, should eat more fruits, vegetables and crude fiber foods, and develop a regular bowel habit, to promote bowel movements. If necessary, give enema to help defecation.

References:

GARDhttps:https://rarediseases.org/gard-rare-disease/6267/neuromyelitis-optica/ https://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=10986&Disease_Disease_Search_diseaseGroup=neuromyelitis-optica&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Neuromyelitis-optica-spectrum-disorder&title=Neuromyelitis%20optica%20spectrum%20disorder&search=Disease_Search_Simple

Other useful websites:

More about the disease:https://www.massgeneral.org/cn/treatments/neurology/about-nmo Patient groups: HK Neuromyelitis Optica Association https://www.nmohk.com Hong Kong Neuro-Muscular Disease Association https://hknmda.org.hk/category/nmo