CNS lupus, Systemic Lupus (SLE) in the brain and / or spinal cord 4.33/5 (6)

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Cerebral Lupus / Neuropsychiatric SLE with MRI changes in the brain: Wang HP, Chin Med J, 2016. CC BY-NC-SA 3.0

Definition

Signs of disease in the brain and / or spinal cord may be due to Systemic lupus erythematosus (SLE). Changes are usually detected by various tests / examinations. Most neuropsychiatric manifestations occur early in the course of SLE. However, several of the symptoms are so common that in SLE they can only be related to the disease in about 30% of cases (Hanly JG, 2020).

Examinations

  • Antibody in serum and spinal fluid: Anti-ribosomal P and antifosfolipid antibodies are associated with neuropsychiatric SLE
  • MRI of the brain recommended for suspicion of focal neurological changes, seizures, chronic cognitive failure and Antiphospholipid syndrome. However, MRI changes are not easy to interpret (Reference: Kim KW, 2008)
    Changes in white matter are present in healthy population under 50 years in approx. 20%
    • At age over 70 years, 90% has such MRI changes
    • SLE and MRI changes in white matter:
      • For about. 50% is normal MR normal, although neuropsychiatric lupus is present
      • Small infarct changes may resemble Multiple sclerosis (MS). Increased incidence at Antiphospholipid syndrome. The changes correlate poorly with SLE activity
      • Major MRI lesions in the brain can correlate with SLE activity (SLEDAI score)
      • In case of acute convulsions, psychosis or coma there are signs of brain edema
      • "Dynamic susceptibility contrast-enhanced T2-weighted perfusion MRI (DSC-MRI)" is a special study used at some centers
      • More about MRI examination of the brain at SLE here (reference: Sarbu N, 2015)
    •  Electro encephalogram (EEG)
      • 80% with active CNS lupus have pathological changes (slow waves, focal changes)
    • Spinal Fluid examination
        • Routine examinations may be normal. Special tests are aDNA, IgG and immune complexes)
    • Neuropsychological tests
        • These may be useful for mapping and as a basis for later inspection.
    •  PET / CT can be used

 Different types of nerve manifestations

A total of 19 neuropsychiatric symptoms and manifestations of SLE were defined by ACR in 1999 (reference: ACR ad hoc committee, 1999). A selection:

  • Cognitive problems (attention, memory, control of emotions, reduced pace) described at 20-80% with SLE
  • Anxiety
  • Depressed mood / depression
  • Headache (most often migraine or tension headache) is common in the population as well. New headache at SLE is considered for sinus vein thrombosis (Especially at Antifosfolipid antibodies)
  • Psychosis
    • Serious manifestation with a loss of reality experience
    • SLE-conditioned psychosis is distinguished from psychosis triggered by corticosteroids (eg Prednisolone more than 40mg / day)
  • Epileptic cramps
    • Occurs among 10-20% with CNS lupus
    • May be debut symptom at SLE
    • Often related to SLE activity or old, larger scar changes in the brain
    • Increased incidence of antifosfolipid antibodies
  •  By Antiphospholipid syndrome (with increased risk of blood clots (thromboembolisms)) injury may occur independently of SLE activity. Neurological symptoms may be
    • TIA and / or stroke
    • Vascular dementia
    • Convulsions
    • Sinus-vein thrombosis (headache)
    • Coordination Problem
    • Headache
  •  Transverse myelitis
    • Sudden weakness in lower extremities and / or sensitivity loss
    • Loss of control over urine and faeces (sphincter paresis) occurs
    • Most often at the same time as high SLE activity
    • Investigation with MRI investigations showing disease signs
  • Fatigue (chronic fatigue) is considered not as a symptom of neuropsychiatric lupus

Diagnosis

  • Disease history
  • Investigational Findings
  • If investigations do not show signs of disease, the diagnosis depends on the expert's overall assessment

Incorrect diagnosis? (Similar diseases / differential diagnoses)

Literature


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