Neuropsychiatric systemic lupus erythematosus
Neuropsychiatric systemic lupus erythematosus refers to the neurological and psychiatric manifestations of systemic lupus erythematosus. SLE is an autoimmune disease that can affect many of the body's organs and systems. It is estimated that over half of people with SLE have neuropsychiatric involvement.
Classification
The American College of Rheumatology has outlined 19 syndromes that are seen in NPSLE. These syndromes encompass disorders of the central and peripheral nervous systems:- Aseptic meningitis
- Cerebrovascular disease
- Demyelinating syndrome
- Headache
- Movement disorder
- Myelopathy
- Seizure disorders
- Acute confusional state
- Anxiety disorder
- Cognitive dysfunction
- Mood disorder
- Psychosis
- Acute inflammatory demyelinating polyradiculoneuropathy
- Autonomic disorder
- Mononeuropathy
- Myasthenia gravis
- Cranial neuropathy
- Plexopathy
- Polyneuropathy
The majority of cases involve the central nervous system, which consists of the brain and spinal cord. The most common CNS syndromes are headache and mood disorder.
Though neuropsychiatric lupus is sometimes referred to as "CNS lupus", it can also affect the peripheral nervous system. Between 10–15% of people with NPSLE have PNS involvement. Mononeuropathy and polyneuropathy are the most common PNS syndromes.
Other syndromes
Some neurological syndromes outside of the ACR classification may also be considered NPSLE manifestations. These include neuromyelitis optica, posterior reversible encephalopathy syndrome, [small fiber peripheral nervous system|peripheral neuropathy|small fiber neuropathy], and Lambert–Eaton myasthenic syndrome.Pathogenesis
There are several possible mechanisms that underlie the nervous system manifestations of lupus. Specific syndromes may be vasculopathic, autoantibody-mediated, or inflammatory in nature.There is evidence that the blood–brain barrier, which protects the CNS, is compromised in patients with NPSLE. As a result, autoantibodies are able to infiltrate the CNS and cause damage.