Lupus is an autoimmune disease that can affect many parts of the body, including the brain. When lupus affects the brain, it is called neuropsychiatric systemic lupus erythematosus (NPSLE). Up to 80% of people with lupus experience some neurological or psychiatric symptoms. These symptoms can range from mild to severe and may come and go over time. Some of the main ways that lupus can impact the brain include:
Cognitive Dysfunction
Many people with lupus experience issues with concentration, memory, learning, and processing speed. This cluster of symptoms is often referred to as “lupus fog” or “brain fog.” Studies estimate 40-80% of lupus patients have cognitive problems. The exact causes are not fully understood but likely involve inflammation, autoantibodies, and microvascular damage in the brain.
Some common cognitive symptoms of lupus include:
- Forgetfulness
- Difficulty focusing and paying attention
- Problems learning and remembering new information
- Difficulty finding the right words
- Slower thinking speed
- Trouble multitasking or processing complex information
Cognitive dysfunction can significantly impact daily life and make it hard to function at home, work, or school. Fortunately, some treatments may help, which will be discussed later.
Headaches and Migraines
Many people with lupus frequently get severe headaches or migraines. Studies estimate 20-95% of lupus patients experience frequent headaches. The wide range is likely because headaches have many causes. Headaches related to lupus may be due to:
- Inflammation of blood vessels in the brain
- Blood clots blocking blood flow
- Side effects of lupus medications
- Fatigue and stress from dealing with lupus
- Pain caused by inflammation of the membranes covering the brain (meninges)
Lupus headaches can feel like throbbing, pounding, or stabbing pain on one or both sides of the head. Migraines usually involve additional symptoms like nausea, sensitivity to light and sound, or visual disturbances.
Mood Disorders
Many people with lupus experience psychiatric symptoms like anxiety, depression, and mood swings. Studies estimate 20-95% of lupus patients have mood disorders. Possible causes include:
- The stress and chronic pain of dealing with lupus
- Hormonal factors
- Side effects of medications
- Brain inflammation
- Imbalances in neurotransmitters like serotonin due to autoantibodies
Symptoms of mood disorders can include:
- Persistent sad or “low” mood
- Loss of enjoyment and interest in normal activities
- Excessive worrying or fears
- Irritability or restlessness
- Changes in appetite and sleep habits
- Difficulty concentrating
- Thoughts of suicide
If mood symptoms are significantly impacting quality of life, medications or counseling may help.
Delirium and Psychosis
In rare cases, people with lupus can develop sudden onset confusion, disorientation, and even psychosis. This is believed to be caused by inflammation reducing blood flow to parts of the brain. Symptoms may include:
- Confusion and disorientation
- Memory loss
- Difficulty paying attention
- Hallucinations and delusions
- Paranoia
- Agitation and strange behavior
Delirium and psychosis require urgent medical treatment to identify the underlying cause and prevent long-term brain damage.
Seizures
A small percentage of lupus patients (5-10%) experience seizures. Seizures occur when brain cells misfire and overload. Causes may include:
- Inflammation and autoantibodies damaging brain cells
- Blood clots blocking blood flow to parts of the brain
- Bleeding into the brain
- Scarring on the brain from prior lupus flares
- Side effects of immune-suppressing drugs
Seizures may cause muscle spasms, loss of consciousness, sensory changes, or confusion. Seeking prompt treatment is crucial to prevent complications like falls or status epilepticus, a dangerous seizure that doesn’t stop.
Stroke
Patients with lupus have a higher risk of stroke compared to the general population. Lupus can cause stroke through:
- Blood clots blocking blood vessels in the brain
- Bleeding into the brain from damaged blood vessels (hemorrhagic stroke)
- Inflammation damaging blood vessels
- Autoantibodies promoting blood clotting
Signs of stroke include sudden numbness, weakness, vision loss, dizziness, and difficulty speaking. Stroke is a medical emergency requiring immediate treatment to limit brain damage.
Demyelinating syndrome
Some lupus patients develop demyelinating neurological disorders like multiple sclerosis, optic neuritis, and transverse myelitis. These occur when the myelin coating around nerve fibers becomes damaged, disrupting signal transmission. Symptoms depend on the location but may include:
- Weakness or paralysis of limbs
- Vision loss
- Numbness
- Tingling
- Difficulty walking
- Bladder or bowel dysfunction
Demyelinating disorders can potentially cause permanent impairments. Immunosuppressive treatment aims to control inflammation and prevent further damage.
Movement disorders
Some lupus patients experience disorders that impair control of movement, such as:
- Chorea: Involuntary, jerky “dance-like” movements, often affecting the face and hands.
- Tremors: Shaking, especially in the hands.
- Myoclonus: Sudden, brief muscle jerks.
- Dystonia: Sustained muscle contractions causing abnormal postures or twisting.
- Parkinsonism: Slow movements, rigidity, and problems with balance and coordination.
These disorders result from autoantibodies or inflammation damaging the basal ganglia, cerebellum, and other motor control regions. Symptoms may come and go over time. Immunosuppressive or anti-seizure medications can help in some cases.
Impact on Thinking and Behavior
In addition to specific neurological disorders, lupus can cause milder cognitive, emotional, and behavioral changes including:
- Slowed thinking, planning and decision making
- Impulsiveness and poor judgment
- Apathy and loss of initiative
- Social disinhibition
- Memory lapses
- Loss of focus and difficulty multitasking
- Irritability, anxiety or sadness
- Withdrawing from others
These changes can impair relationships, work/school performance, and quality of life. Support from loved ones helps cope.
Diagnosing Neuropsychiatric Lupus
Doctors use several approaches to diagnose NPSLE and pinpoint the type and location of any brain involvement, including:
- Medical history – Asking about neurological and psychiatric symptoms and their timing in relation to lupus flares.
- Physical and neurological exam – Testing reflexes, coordination, balance, vision, etc.
- Cognitive testing – Paper or computerized tests assessing memory, processing speed, executive function, etc.
- Blood tests – Checking antibody levels and markers of inflammation.
- Brain imaging – CT, MRI, or PET scans visualizing brain structure, function, and blood flow.
- EEG – Recording electrical activity in the brain to detect seizures.
- CSF analysis – Testing cerebrospinal fluid for antibodies and inflammation. Requires a spinal tap.
Doctors also need to rule out other possible causes like infections, side effects of medications, or unrelated neurological illness. Accurate diagnosis guides appropriate treatment.
Treating Neuropsychiatric Lupus
Treatment aims to control inflammation and autoantibody activity in the brain and nervous system. Options may include:
- Corticosteroids – Potent anti-inflammatories like prednisone, often used for acute flares.
- Immunosuppressants – Drugs like azathioprine, mycophenolate, methotrexate, or cyclophosphamide to suppress the overactive immune system long-term.
- Biologics – Newer drugs like belimumab that target specific parts of the immune response.
- Plasmapheresis – Filtering out autoantibodies from the blood.
- Rituximab – An infusion that depletes B cells that produce antibodies.
- Blood thinners – Used to prevent clots and stroke.
- Anti-seizure drugs – Help control seizures and nerve pain.
- Antidepressants or anti-anxiety medication – Can improve mood disorders, pain, and sleep.
Lifestyle measures like stress reduction, counseling, cognitive rehabilitation, gentle exercise, and brain-healthy nutrition may also help manage NPSLE.
Coping with Cognitive Symptoms
Cognitive dysfunction can be one of the most frustrating aspects of lupus. Some tips for coping include:
- Get plenty of rest and avoid exhausting yourself mentally.
- Reduce stress and multi-tasking as much as possible.
- Ask for help from loved ones with challenging mental tasks.
- Use calendars, to-do lists, reminders and notes to compensate for memory issues.
- Focus on one task at a time and take breaks as needed.
- Carry around a small notebook to write everything down.
- Let people know you have memory issues so they understand if you forget things.
- Give yourself more time to learn new skills.
- Engage in stress-reducing hobbies, social activities and light exercise to get mental stimulation.
Be patient with yourself. With the right treatment and support, many people find their thinking improves over time.
When to Seek Urgent Care
In most cases, neurological symptoms of lupus come on gradually. But some require emergency care, including:
- Sudden, severe headache or head pain
- Dizziness or loss of balance
- Blurry or double vision
- Weakness or numbness on one side of the body
- Slurred speech
- Confusion
- Seizures
- Hallucinations or psychosis
Seek immediate medical attention if you experience these signs, as they may indicate a stroke, seizure, or other medical emergency.
Preventing Flares
While lupus can never be cured, the following strategies help reduce NPSLE flares:
- Take medications consistently as prescribed.
- Learn your personal lupus flare triggers and try to avoid them.
- Use sun protection, avoid infections, and get enough rest.
- Manage stress levels with tools like meditation, therapy, exercise, or support groups.
- Live a generally healthy lifestyle with a nutritious diet, exercise, and sound sleep habits.
- Avoid smoking and limit alcohol, which increase inflammation.
- Seek prompt treatment for any emerging lupus symptoms.
While flares can’t always be prevented, minimizing avoidable triggers reduces overall risk and severity.
Long-Term Outlook
The long-term outlook for NPSLE varies significantly between patients. Some people experience mild cognitive or psychiatric symptoms that respond well to treatment. Others deal with recurrent flares, progressive impairment, and permanent disability. General predictors of outcome include:
- Type and severity of initial symptoms – Stroke, seizure disorders and diffuse brain inflammation tend to cause more lasting damage than headaches or mood disorders.
- Level of ongoing disease activity – Patients with frequent lupus flares usually fare worse.
- Timeliness of treatment – Early aggressive treatment prevents cumulative damage.
- Medication compliance – Patients who take medications consistently tend to have better control of symptoms.
- Lifestyle factors – Overall health habits like diet, exercise, sleep, and stress management support the best outcomes.
While some impairment may be irreversible, starting appropriate therapies early and actively working to prevent flares provides the best chance of living well with lupus.
Conclusion
Lupus is a complex autoimmune disease that can affect the brain and nervous system in a variety of ways. Common neurological manifestations include headaches, cognitive dysfunction, mood disorders, movement disorders, stroke, seizures and more. Diagnosis involves testing for antibodies, inflammation and neuropsychiatric symptoms. Treatment centers on controlling inflammation and autoantibody activity with immunosuppressive drugs. Supportive therapies and lifestyle changes also help manage symptoms. While some patients experience mild impairment, others deal with recurrence flares, cumulative damage and disability. Seeking prompt treatment and diligently trying to prevent flares offers the best opportunity for a positive long-term outcome.
