Neuromyelitis optica spectrum disorder (NMOSD)
Two years ago, my mother was first diagnosed with neuromyelitis optica spectrum disorder (NMOSD).
She has been managing her condition with corticosteroids and immunosuppressants, and there have been no relapses during this time.
However, due to long-term corticosteroid use, she has developed avascular necrosis of the hip and is scheduled for a hip replacement next week.
After discussing with her attending physician, her corticosteroid dosage was reduced from two pills to one (which she has been taking for nearly a month).
Recently, she has experienced symptoms such as limb weakness, painful spasms, severe lower back pain, and gastrointestinal discomfort.
We are very concerned that these might be signs of a relapse of NMOSD.
Could you please clarify if these symptoms are indicative of an impending relapse? The information available online is unclear about what constitutes a relapse.
Since there is still some time before her next appointment, should we go to the emergency room? Will the emergency department be able to quickly assess if a relapse has occurred? How can we determine if it is a relapse? Is blood work sufficient, or is an MRI necessary? Aside from the initial MRI during her first episode, her doctors have advised against further imaging over the past two years.
Would it be prudent to have regular MRI scans every six months or annually? We are genuinely worried about the possibility of a relapse.
Thank you for your patience in addressing my concerns.
Xiao Qi, 20~29 year old female. Ask Date: 2022/06/03
Dr. Jiang Junyi reply Neurology
Dear Miss Hsiao-Chi,
You mentioned that your mother has some time until her next follow-up appointment and asked whether she should go to the emergency department.
Can the emergency department quickly check if there is a relapse? How can we determine if there is a relapse? Is blood work sufficient, or is an MRI necessary?
Acute exacerbations of NMOSD (Neuromyelitis Optica Spectrum Disorder) are typically assessed in conjunction with clinical symptoms and MRI imaging.
During an exacerbation, increased signal intensity in the optic nerve can be observed on MRI T2 fat-suppressed and T1-weighted images (with contrast).
MRI imaging cannot be scheduled on a 24-hour basis.
Emergency examinations can be conducted during regular hospital hours if necessary.
For acute exacerbations of NMOSD, treatment generally involves high-dose corticosteroid pulse therapy or plasmapheresis to remove abnormal antibodies from the patient's system.
Another option is the administration of intravenous immunoglobulin (IVIG) to neutralize the abnormal antibodies.
I recommend discussing treatment options with your mother's neurologist.
Thank you for your inquiry, and feel free to reach out with any further questions.
Wishing you good health and peace,
Dr.
Chien-Yi Chiang
Neurology, Taoyuan Hospital
Reply Date: 2022/06/03
More Info
Neuromyelitis Optica (NMO), also known as Devic's disease, is a rare autoimmune disorder that primarily affects the optic nerves and spinal cord. It is characterized by episodes of inflammation that can lead to severe neurological deficits. Understanding the symptoms, potential relapses, and management strategies is crucial for patients and their families, especially when dealing with the complexities of long-term treatment.
In your mother's case, it is important to recognize that while she has been stable for two years on steroids and immunosuppressants, the recent symptoms of limb weakness, pain, and gastrointestinal discomfort could indeed be concerning. In NMO, relapses can manifest in various ways, often resembling the initial symptoms of the disease. Common signs of a relapse may include:
1. Visual Disturbances: This could range from blurred vision to complete vision loss in one or both eyes.
2. Motor Weakness: Weakness in the limbs, which can be unilateral or bilateral, is a significant indicator.
3. Sensory Changes: Patients may experience numbness, tingling, or pain in the limbs or torso.
4. Bowel or Bladder Dysfunction: Changes in bowel or bladder control can also indicate a relapse.
5. Severe Pain: This can include back pain or neuropathic pain, which may be exacerbated by changes in medication.
Given your mother's symptoms of weakness and pain, it is prudent to consider these as potential indicators of a relapse. However, it is also essential to differentiate between a relapse and side effects from medication, particularly since she has recently reduced her steroid dosage. Long-term steroid use can lead to various complications, including joint issues like avascular necrosis, which may explain her hip problems.
Regarding your concerns about whether to seek emergency care, it is advisable to err on the side of caution. If her symptoms are severe or worsening, visiting the emergency department could provide immediate assessment and relief. Emergency physicians can perform initial evaluations, including blood tests and possibly imaging studies, to determine if there is a relapse or if the symptoms are due to other causes.
In terms of diagnostic imaging, while MRI is the gold standard for assessing NMO, it may not always be necessary unless there are clear signs of a relapse. Blood tests can help rule out other conditions and assess inflammatory markers, but they may not definitively indicate a relapse of NMO. Regular follow-ups with her neurologist are essential, and discussing the need for periodic MRIs is a good idea, especially if there are changes in her clinical status.
As for management, if a relapse is confirmed, treatment typically involves high-dose corticosteroids to reduce inflammation. Other options may include plasmapheresis or additional immunosuppressive therapies, depending on the severity of the relapse and the patient's overall health.
In conclusion, while your mother's symptoms could indicate a potential relapse of NMO, they could also be related to her recent medication changes or other health issues. It is crucial to communicate these concerns with her healthcare provider promptly. Regular monitoring and open communication with her medical team will help manage her condition effectively and address any emerging issues.
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