Lupus Cerebritis (Neuropsychiatric Lupus)

What neuropsychiatric lupus is

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect skin, joints, kidneys, blood cells and other organs. When it affects the nervous system, it is called neuropsychiatric lupus. The American College of Rheumatology recognizes 19 syndromes, including:

Central nervous system

• headache (including severe lupus headache)
• seizures
• stroke and other cerebrovascular disease
• acute confusional state (delirium)
• cognitive dysfunction ("lupus fog")
• psychosis
• mood disorders and anxiety
• movement disorders (such as chorea)
• myelopathy (spinal cord inflammation)
• aseptic meningitis and demyelinating syndromes

Peripheral nervous system

• peripheral neuropathy (numbness, tingling, weakness)
• cranial neuropathies (such as facial weakness or double vision)
• autonomic disorders and others

Causes

Neuropsychiatric lupus has several mechanisms, which guide treatment:

"Cerebritis" suggests inflamed brain tissue, but true vasculitis of brain vessels is rare.

Conditions that mimic NPSLE

Before attributing symptoms to lupus, doctors look for:

• infections, especially in people on immunosuppressants
• medicine side effects: steroids can cause mood changes and psychosis
• high blood pressure, kidney failure, low sodium or low blood sugar
• primary psychiatric illness, migraine, or unrelated neurological disease

Symptoms

• headaches that are new or different
• seizures
• confusion, disorientation or reduced alertness
• memory, attention and word-finding problems
• depression, anxiety or mood swings
• hallucinations or paranoia
• stroke symptoms
• numbness, tingling or weakness in the hands and feet
• abnormal movements

Diagnosis

• neurological and psychiatric assessment
• MRI of the brain or spine: looks for strokes, inflammation and demyelination; may be normal in diffuse forms
• lumbar puncture: rules out infection and assesses inflammation
• EEG for seizures or confusion
• blood tests: lupus activity (complement levels, anti-dsDNA), antiphospholipid antibodies, anti-ribosomal P, blood counts, kidney function
• nerve conduction studies for neuropathy
• neuropsychological testing for cognitive problems

Treatment

Inflammatory forms

• corticosteroids, often high-dose IV methylprednisolone in severe cases
• immunosuppressants: cyclophosphamide, mycophenolate, azathioprine
• rituximab for resistant cases
• plasma exchange or IV immunoglobulin in selected severe cases
• hydroxychloroquine as background therapy for most people with lupus

Clot-related forms

• anticoagulation (usually warfarin) for antiphospholipid syndrome with stroke
• antiplatelet drugs and control of blood pressure, cholesterol and smoking

Symptom treatment

• anti-seizure medicines
• antipsychotics, antidepressants and anti-anxiety treatment
• cognitive rehabilitation
• headache management

Outlook

Many neuropsychiatric events improve with treatment, particularly acute inflammatory events such as psychosis or confusion. Strokes and severe events can leave lasting effects. Mild cognitive problems may persist but often stabilize. Close follow-up with a rheumatologist, neurologist and mental health professionals gives the best outcomes.

Frequently asked questions

What is lupus cerebritis?

Lupus cerebritis is an older term for neuropsychiatric lupus, when systemic lupus erythematosus affects the brain or nerves. It can cause seizures, confusion, psychosis, stroke, headaches, memory problems and mood changes.

What are the symptoms of lupus affecting the brain?

Symptoms range from mild "brain fog," memory problems, headaches and low mood to severe events such as seizures, hallucinations, sudden confusion and stroke. Numbness or tingling can occur if peripheral nerves are involved.

How is neuropsychiatric lupus diagnosed?

There is no single test. Doctors combine neurological examination, brain MRI, lumbar puncture, EEG, blood tests for lupus activity and antiphospholipid antibodies, and cognitive testing, after ruling out infections, medicine effects and other causes.

Is lupus brain fog serious?

Mild cognitive problems are common in lupus and often fluctuate with fatigue, sleep, pain, mood and disease activity. They are usually not dangerous but should be discussed with your doctor. Sudden or severe confusion is an emergency.

Can neuropsychiatric lupus be treated?

Yes. Inflammatory forms respond to steroids and immunosuppressants, clot-related forms are treated with blood thinners, and symptoms are managed with specific medicines and therapy. Many people improve substantially.