Central Nervous System Lupus
Recently, a dear friend received the news that she had developed CNS (central nervous system) lupus in additional to her pre-existing SLE. This led me to 'hit the net' of course, and delve into the catacombs of articles and medical journals pertaining to the subject. What I discovered was a web of cold and clinical information about the condition. Additionally, there seemed to be a disconnect between the patient experience and the medical data. So, in typical More Than Lupus fashion, I decided to write a blog solely dedicated to “bridging the gap” between the patient and medical community, by providing not only updated information on CNS, but patient perspectives from those that are currently living with it.
“I have lots of frustration, and silent secret struggles and uncertainties with the degree of my dysfunction.” - Cathy
What Is CNS Lupus?
Lupus us an inflammatory autoimmune disorder where your body attacks healthy cells by mistake. This process can affect almost any part of the body, causing inflammation, tissue damage, and even death. Some of the most common areas lupus affects are the joints, skin, kidneys, heart, lungs, and blood. However, lupus can also affect the brain and nervous system. When it does, it is referred to as central nervous system lupus or CNS lupus.
CNS lupus is sometimes referred to as neurocognitive dysfunction, or neuropsychiatric lupus, and it can affect the three parts of the nervous system, which include:
The central nervous system (CNS): This includes the brain and spinal cord.
The peripheral nervous system (PNS)—A network of nerves that connects the brain and spinal cord to the rest of the body, and gives skin and muscles the signals needed for sensation and movement.
The autonomic nervous system (ANS)— The network that allows communication between spinal and peripheral nerves, to the brain and internal organs, and controls functions like breathing, blood flow, and heart rate.
Symptoms of CNS lupus vary depending on which area of the nervous system is affected. If the central nervous system is affected, a patient may experience:
Memory issues or “Brain Fog”
If lupus affects the peripheral nervous system, which controls motor responses and sensation, a patient may experience:
Carpal tunnel syndrome
Ringing In the ears
Drooping of the eyelid
If the autonomic nervous system is affected, which regulates heart rate, blood pressure, temperature, bowel functions, breathing, muscle movement, and release of adrenaline, a patient may experience:
Nausea, vomiting, diarrhea, and constipation
As you can see, symptoms may overlap - and some patients can have more than one part of the nervous system affected. You may have CNS lupus and also Raynaud’s Syndrome. Or, have burning and tingling (autonomic involvement) and carpal tunnel (peripheral involvement).
From the patient perspective, one of the most frustrating components of CNS lupus is the cognitive or lupus “brain fog” as one patient states,
“I struggle with memory, language both receptive and expressive, confusion, concentration. I struggle at times to express my thoughts, my memory is terrible both long term and short. I sometimes fake or I guess it’s lying when I say “ oh ya… I remember ...that.”
Let me talk a moment to address one of the above symptoms, seizures. Seizures are already known to occur in 14-25% of patients with lupus compared with 0.5-1% in the general population. Seizures may result from CNS Vasculitis (inflammation of blood vessel walls in the brain or spine), or from a cardiac embolism, infection, or drug reaction.
How is CNS Diagnosed?
“I personally have seen very little information regarding this type of lupus. Since I was just diagnosed, I am not aware of the prevalence of it but I would imagine many people are living with it without being diagnosed.” - Darien, CNS patient
CNS, like all forms of lupus, can be tricky to diagnosis. This is mainly because symptoms can come and go and present similarly to other diseases. If your doctor suspects that you are living with CNS lupus, they will most likely refer you to a neurologist. Neurologists are medical doctors who study how the nervous system functions, as well as different conditions that affect the nervous system.
Different diagnostic tools are used to determine in what ways the nervous system is being affected. For example: the most common manifestation of CNS is cognitive impairment, which includes “brain fog”, confusion, and impaired memory. Statistically, 80% of lupus patients who have lived with lupus for ten years or more will experience this type of cognitive dysfunction. Single positron emission computed tomography (SPECT) scans can be used to show abnormalities in blood flow, indicating that the cognitive impairment may be the result of decreased oxygen flow to certain parts of the brain.
Neurologists also use brain scans like an MRI (magnetic resonance imaging) or a CT (computerized tomography ) to view the brain and explore symptoms, like migraine-like headaches. About 20% of patients experience a true “lupus headache.” In rare cases, lumbar punctures and EEG’s are used to examine the spinal fluid and record brain waves if a condition like organic brain syndrome (cerebritis, encephalopathy, and acute confusional state) may be suspected.
“My experience upon diagnosis was a history of strokes, seizures, encephalitis, and severe cognitive and memory issues. Prior to my lupus diagnosis, they thought I had multiple sclerosis.” - Lupus patient
Cognitive and behavioral testing may also be performed to find out if your memory or other mental abilities have been affected. Blood tests can also be helpful to recognize CNS disease activity, since the erythrocyte sedimentation rate (ESR) or plasma viscosity can be elevated during neurologic episodes.
You may be asking yourself, “How common IS CNS lupus?” And the answer is not exactly black and white. Many people are believed to be living with CNS manifestations, however, are not diagnosed. However, according to 10 intensive studies by the American College of Rheumatology, which included 2,049 SLE patients, the prevalence of neurologic involvement was 56% with approximately 90% of that being CNS manifestations that included headache, cognitive dysfunction, and mood disorders. Thankfully, seizures and cerebrovascular disease were very low in prevalence.
Depending on the symptoms, your doctor may talk to you about a variety of different medications to treat you CNS lupus.
The most common treatments include:
Non-steroidal anti-inflammatory drugs (Ibuprofen, Motrin, Mobic, Naproxen)
Corticosteroids (Prednisone, Medrol, Millipred)
Immunosuppressants (CellCept, Methotrexate, Cyclophosphamide, Azathioprine)
Antidepressants (Cymbalta, Nortriptyline)
Anticonvulsants (Lyrica, Gabapentin)
Each person's response to treatment can vary, from rapid improvement or gradual over several months. But, for many with lupus, nervous system involvement can be reversible.
Today, with careful treatment, 80 to 90 percent of people with lupus can expect to live a normal lifespan. Central nervous system (CNS)-specific statistics are not available, however, neuropsychiatric events in systemic lupus erythematosus (SLE) have a more favorable outcome than events secondary to non-SLE causes. The concern with CNS lupus is, how high a person’s disease activity index is, if there is a positive antiphospholipid antibody, and a high ESR. Those variables can affect patient outcomes, as well as, patient medication compliance.
It is important for you to address your concerns promptly with your doctor if you suspect you may have CNS lupus. The more upfront you are about your symptoms, the sooner the treatment, and the better the outcome.
**All resources provided by this blog are for informational purposes only, not to replace the advice of a medical professional. Kelli encourages you to always contact your medical provider with any specific questions or concerns regarding your illness. All intellectual property and content on this site and in this blog is owned by morethanlupus.com. This includes materials protected by copyright, trademark, or patent laws. Copyright, More Than Lupus 2019.