neuromyelitis optica

ICD-10 Codes

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Description

Neuromyelitis Optica (NMO): A Rare Autoimmune Disease

Neuromyelitis optica, also known as Devic disease, is a rare autoimmune condition that affects the central nervous system. It's characterized by severe inflammation and damage to the optic nerves and spinal cord.

Symptoms of NMO:

  • Optic Neuritis: Pain in the eye and vision loss
  • Transverse Myelitis: Weakness or paralysis of arms and legs
  • Numbness: Loss of sensation in various parts of the body

Key Facts about NMO:

  • It's a rare autoimmune disease, affecting only a small percentage of people worldwide.
  • The exact cause is unknown, but it's believed to be an immune-mediated disorder.
  • There's no cure for NMO, but treatments can help manage symptoms and slow disease progression.

Understanding the Central Nervous System:

NMO affects the central nervous system (CNS), which consists of the brain and spinal cord. The CNS is responsible for controlling various bodily functions, including movement, sensation, and vision.

References:

  • [1] Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. Less often, it affects the brain and other parts of the CNS.
  • [2] NMO is characterized by severe inflammation and damage to the optic nerves and spinal cord, leading to symptoms such as pain in the eye, weakness or paralysis of arms and legs, and numbness.
  • [3] The exact cause of NMO is unknown, but it's believed to be an immune-mediated disorder.
  • [4] There's no cure for NMO, but treatments can help manage symptoms and slow disease progression.

Note: The above information is based on the search results provided in the context.

Additional Characteristics

  • numbness
  • loss of sensation
  • optic neuritis
  • transverse myelitis
  • weakness or paralysis of arms and legs
  • pain in the eye
  • severe inflammation
  • damage to optic nerves and spinal cord
  • immune-mediated disorder

Signs and Symptoms

Neuromyelitis optica (NMO), also known as Devic's disease, is a rare autoimmune disorder that affects the central nervous system. The signs and symptoms of NMO can vary from person to person, but here are some common ones:

  • Eye problems: Pain in the eyes, loss of vision, blurry vision, or blindness in one or both eyes are common symptoms of NMO.
  • Weakness or paralysis: Weakness or numbness in the arms and legs, as well as paralysis of the arms and legs, can occur due to damage to the spinal cord.
  • Balance difficulties: Many people with NMO experience balance problems, which can lead to difficulty walking or maintaining posture.
  • Numbness or tingling: Numbness or tingling sensations in the hands and feet are also common symptoms of NMO.
  • Painful spasms: Painful muscle spasms can occur due to inflammation and damage to the nerves.
  • Loss of bladder and bowel control: In severe cases, NMO can cause loss of bladder and bowel control due to damage to the spinal cord.
  • Severe nausea and vomiting: Some people with NMO may experience severe nausea and vomiting, which can be accompanied by hiccups.

It's essential to note that these symptoms can vary in severity and may not occur simultaneously. If you or someone you know is experiencing any of these symptoms, it's crucial to seek medical attention promptly.

References:

  • [2] Eye pain and problems with your eyesight or colour vision, in one or both eyes · sharp, burning or shooting pain (nerve pain) can be a symptom of NMO.
  • [3] Pain in the eyes · Loss of vision · Weakness or numbness in the arms and legs · Paralysis of the arms and legs are symptoms of NMO.
  • [4] A telltale sign of neuromyelitis optica is sudden loss of vision in both eyes. Other symptoms may include: Balance difficulties; Problems with bladder and bowel control.
  • [5] Blurry vision or blindness in one or both eyes · Weakness or paralysis in the legs or arms · Painful spasms · Numbness or loss of sensation are symptoms of NMOSD.
  • [6] Weakness or paralysis in the legs or arms · Difficulty walking · Numbness or tingling · Painful spasms · Uncontrollable hiccups are signs and symptoms of neuromyelitis optica.

Additional Symptoms

  • Difficulty walking
  • Blurry vision
  • Severe nausea and vomiting
  • Numbness or tingling sensations in the hands and feet
  • Balance difficulties
  • Painful muscle spasms
  • Pain in the eyes
  • Blindness in one or both eyes
  • Loss of bladder and bowel control
  • Sudden loss of vision in both eyes
  • Weakness or paralysis in the legs or arms
  • Uncontrollable hiccups
  • loss of vision
  • paralysis
  • weakness

Diagnostic Tests

Diagnostic Tests for Neuromyelitis Optica (NMO)

Neuromyelitis optica (NMO), also known as Devic's disease, is a rare autoimmune disorder that affects the central nervous system. Diagnosing NMO can be challenging, but several diagnostic tests can help confirm the condition.

Laboratory Testing

  • Aquaporin-4 receptor (AQP4) IgG and myelin oligodendrocyte glycoprotein (MOG) IgG serum antibody status: This test determines the presence of specific antibodies in the blood that are associated with NMO. [2]
  • Cell-based assays (CBAs): These tests, such as microscopy or flow cytometry-based detection, can optimize autoantibody detection and are strongly recommended for AQP4 testing. [6]

Imaging Studies

  • MRI scan of the brain and spinal cord: This test helps identify lesions in the central nervous system that may be indicative of NMO. [3]
  • Visual evoked responses (VER): This test assesses how well the optic nerves are functioning. [7]

Other Diagnostic Tests

  • Blood tests: These tests can help rule out other conditions with similar symptoms and signs. [1, 9]
  • Lumbar puncture: A sample of cerebrospinal fluid is taken to check for signs of NMO. [9]

It's essential to note that a combination of these diagnostic tests, along with a thorough medical evaluation, can help confirm an NMO diagnosis.

References: [1] Nov 23, 2022 — Diagnosis [2] Jun 13, 2024 — Laboratory testing involves determining aquaporin-4 receptor (AQP4) IgG and myelin oligodendrocyte glycoprotein (MOG) IgG serum antibody status; [3] MRI scan of your brain and spinal cord. Tests to check on how well your optic nerves are working. [6] Quest Diagnostics offers cell-based assays (CBAs) for testing AQP4 or MOG antibodies (Table 2). [7] Diagnosis of NMOSD. A doctor's evaluation. Magnetic resonance imaging. Visual evoked responses. Blood tests. [8] by DM Wingerchuk · 2015 · Cited by 4410 — The Panel recommended testing with cell-based serum assays (microscopy or flow cytometry-based detection) whenever possible because they optimize autoantibody ... [9] How neuromyelitis optica is diagnosed · blood tests · an MRI scan to check your brain and spinal cord · a lumbar puncture to take a sample of fluid from your ...

Treatment

Treatment Options for Neuromyelitis Optica (NMO)

Neuromyelitis optica (NMO), also known as Devic's disease, is a rare autoimmune disorder that affects the central nervous system. While there is no cure for NMO, various treatment options are available to manage symptoms and slow disease progression.

Monoclonal Antibodies

  • Eculizumab, a complement inhibitor, has been approved by the FDA for the treatment of NMO.
  • Satralizumab, an interleukin-6 (IL-6) inhibitor, has also shown promise in treating NMO.
  • Inebilizumab, an anti-CD19 agent, is another monoclonal antibody being investigated for its potential in treating NMO.

Corticosteroids

  • Corticosteroid medications such as methylprednisolone (Solu-Medrol) are often used to treat acute relapses of NMO.
  • These medications can help reduce inflammation and manage symptoms, but their long-term use is not recommended due to potential side effects.

Immunosuppressive Agents

  • Cyclophosphamide, a cytotoxic alkylating agent, has been evaluated in small studies for its potential in treating NMO.
  • Mycophenolate mofetil (CellCept), rituximab (Rituxan), and azathioprine (Imuran) are other immunosuppressive agents that have been used off-label to treat NMO.

Other Treatment Options

  • Disease-modifying antirheumatic drugs (DMARDs) such as methotrexate have also been investigated for their potential in treating NMO.
  • Plasmapheresis, a process that removes antibodies from the blood, may be recommended in some cases to manage severe relapses.

References:

  • [1] The newly approved treatments are three monoclonal antibodies: Eculizumab, a complement inhibitor; Inebilizumab, an anti-CD19 agent ...
  • [3] Drugs used to treat Neuromyelitis Optica Spectrum Disorder ; Rate ; Generic name: satralizumab systemic; Brand name: Enspryng; Drug class: interleukin inhibitors ...
  • [4] Cyclophosphamide is a cytotoxic alkylating agent that is used in the treatment of severe autoimmune disorders. This drug has been evaluated in NMO in two small ...
  • [5] Satralizumab can be effective in the treatment of NMO/NMOSD due to potential spikes in IL-6 in the cerebral spinal fluid (CSF) in patients with ...

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnosis of Neuromyelitis Optica (NMO)

Neuromyelitis optica (NMO), also known as Devic's disease, is a rare and severe autoimmune disorder that affects the central nervous system. When diagnosing NMO, it's essential to consider several differential diagnoses to rule out other conditions with similar symptoms.

Key Differential Diagnoses:

  • Multiple Sclerosis (MS): MS is a common demyelinating disease of the CNS, but it can be challenging to distinguish from NMO. Both conditions can present with optic neuritis, cerebral and spinal cord lesions, and similar clinical features [6][9].
  • MOG-Associated Optic Neuritis (MOG-EM/MOGAD): MOG-associated optic neuritis is a distinct entity that shares some clinical and radiological features with NMO. However, it's characterized by the presence of anti-MOG antibodies [5].
  • Acute Disseminated Encephalomyelitis (ADEM): ADEM is an inflammatory CNS disorder that can present with seizures, altered mental status, and neurological deficits. While it shares some similarities with NMO, ADEM typically has a more acute onset and a different radiological appearance [10].
  • Systemic Lupus Erythematosus (SLE): SLE is a systemic autoimmune disease that can affect the CNS, leading to symptoms similar to NMO. However, SLE is characterized by its broader range of systemic manifestations and specific autoantibodies [10].

Other Differential Diagnoses:

  • Neurosarcoidosis: This condition presents with granulomatous inflammation in the CNS, which can mimic some features of NMO.
  • Paraneoplastic Neurological Syndromes (PNS): PNS are rare disorders caused by cancer-related immune responses that can affect the CNS and lead to symptoms similar to NMO.

References:

[1] SM Kim et al. (2017) - ATM can be a symptom of either MS, NMO, systemic connective tissue disease, infectious disease, radiation, or malignancy. [2] SM Kim et al. (2017) - Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis ... [3] Mar 22, 2023 - Differential Diagnosis and Types of NMOSD ... NMO/NMOSD (neuromyelitis optica spectrum disorder). [4] BG Dutra et al. (2018) - There are many differential diagnoses based on NMOSD imaging features; however, multiple sclerosis remains the main differential entity. [5] S Jarius et al. (2023) - The most important differential diagnoses include MOG-EM/MOGAD, MS, neurosarcoidosis, paraneoplastic neurological syndromes, and infectious ... [6] Sep 4, 2024 - The most important clinical and imaging differential is multiple sclerosis as both can present with optic neuritis, cerebral and spinal ... [7] JM Seok et al. (2023) - The differential diagnosis between MS and NMOSD is critical for initiating early effective therapy. [8] CC Glisson et al. - is an inflammatory disorder of the central nervous system characterized by severe, immune-mediated demyelination and axonal damage ... [9] Oct 20, 2021 - Are you a healthcare provider looking for information on Neuromyelitis Optica Spectrum Disorder differential diagnosis? Read about it here. [10] Oct 10, 2024 - Differential diagnosis. Multiple sclerosis · Acute disseminated encephalomyelitis; MOG associated optic neuritis; Systemic lupus erythematosus ...

Additional Information

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