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Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) in Children

What Is NPSLE?

Lupus is an autoimmune disease that can affect nearly any part of a child’s body. “Autoimmune” means the immune system sees the body as something foreign and attacks it. When lupus affects the brain, spinal cord or other nerves, it is called neuropsychiatric systemic lupus erythematosus (NPSLE) or central nervous system (CNS) lupus. This happens in about 40% of all people who have systemic lupus erythematosus, or SLE. It is a lifelong disease with quiet spells and flare-ups. It can occur at any age but is rare in children. SLE occurs in less than one in 100,000 children. SLE is the most common form of lupus in children.

Causes of NPSLE

The cause of NPSLE (and all types of lupus) remains unknown. Some factors may combine to trigger this disease, including:

  • Genes: About half of people who have lupus also have a close relative who has lupus. A close relative means a mother, father, sister or brother. No single lupus gene has yet been found.
  • Hormones: A link to hormones could explain why lupus occurs more often in females. It most often starts between ages 15 to 44.
  • Environment: Some factors are already known to make lupus symptoms worse. These include some prescription drugs and exposure to sunlight.

Other factors that may contribute to NPSLE include:

  • Stress
  • Infections
  • Cigarette smoke and secondhand smoke

NPSLE Symptoms

Symptoms of NPSLE may persist or may come and go. They can range from mild to severe. Some of the most common symptoms that may affect the brain and spine include:

  • Headaches not improved by over-the-counter medication
  • Anxiety
  • Depression
  • Confusion
  • Attention problems
  • Seizures
  • Stroke
  • Abnormal gait (walking)
  • Tremors
  • Psychosis
  • Vision problems

Some children have “lupus fog.” This is a combination of memory loss, fatigue, confusion and trouble expressing thoughts.

Blood vessel inflammation (called vasculitis) is a very rare form of NPSLE and can cause high fevers, seizures and stroke. It also causes problems with thinking, attention and memory.

NPSLE Diagnosis

There is no single test for NPSLE. The doctor will start by asking about symptoms. Doctors that treat lupus include neurologists and rheumatologists. A neurologist is a doctor who specializes in conditions of the brain and spine. A rheumatologist is a doctor who specializes in autoimmune diseases affecting the joints, muscles, connective tissues and skin. Then, the doctor may order lab and imaging tests, such as:

  • Blood tests
  • Magnetic resonance imaging (MRI) to detect brain or spine changes
  • Spinal tap to test fluid around the brain and spine for signs of inflammation
  • Electroencephalogram (EEG) to record the brain’s electrical activity – an EEG looks for seizures caused by inflammation
  • Tests for behavior and thinking to study how well a child’s brain is working

NPSLE Treatment

The most common treatments for pediatric NPSLE include:

  • Steroids
  • Conventional and biologic immunosuppressive medications
  • Antimalarial-like drugs

These medicines are used to calm the immune system. They prevent and control inflammation. Children may take these long-term to keep the immune system quiet.

If NPSLE is severe, most children go to the hospital for treatment. A child may respond right away to medication. For other children, it takes a while for medication to work. A child should have routine doctor visits to check progress.

NPSLE Long-Term Outlook

If NPSLE is treated early, most children have a normal lifespan and good quality of life. When one treatment doesn’t work, doctors will keep trying other options.

The Multiple Sclerosis and Neuroimmunology Center at Cincinnati Children’s is home to specially trained doctors who treat NPSLE in children. Our team is prepared to care for your child’s unique needs, from diagnosis to treatment to long-term follow-up. Receiving this well-coordinated care in one location can improve outcomes.

Last Updated 09/2021

Reviewed By Veeral Shah, MD

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For more information or to schedule an appointment, contact the Multiple Sclerosis and Neuroimmunology Center.

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