Neuropsychiatric Manifestations in Antiphospholipid Syndrome (APS) – Prof Graham Hughes

Posted on: May 29, 2020
Last Updated: August 11, 2020

Professor Graham Hughes is a Consultant Rheumatologist and the founder and editor of the international journal LUPUS,’ and Head of London Lupus Centre. In 1983 he described the clotting disorder now known as Hughes Syndrome for which he received the World Rheumatology (ILAR) Research Prize in 1993. 

 Author Quotes 

One hypothesis could be that maybe a number of autonomic features including POTS could be associated with Hughes Syndrome.

So I think, in summary, that the ripples spread of antiphospholipid syndrome and they touch upon psychiatry as well as neurology – I am quite convinced as a clinician.

Summary

Professor Hughes begins this section of his presentation with a discussion of neuropsychiatric manifestations in SLE and APS, moving on to cognitive dysfunction in APS.  

He presents research on antiphospholipid with dementia and psychosis, highlighting research on cognitive and antiphospholipid impairment. Discussing patients with dementia, he explains the MRI changes and their meaning.

Although stroke and transient ischemic attack are the most common neurologic manifestations of APS, both cognitive dysfunction and magnetic resonance imaging (MRI) white matter hyperintensities can occur in aPL-positive patients (with or without APS). [Erkan D et al., 2010]

Learn more about white matter hyperintensities and their significance.

He moves on to explain the relevance of cerebral blood flow may in APS and ends with the less recognized features of APS. 

Prof Hughes takes a look at postural orthostatic tachycardia syndrome (POTS), discussing features common to both autoimmune and autonomic disorders, and presents the hypothesis that POTS might also be associated with Hughes Syndrome. 

Concluding his presentation with a discussion of heparin as a treatment option, patients reported a considerable improvement in symptoms whereas they had not responded to conventional POTS treatment. 

Read about the case of a young female diagnosed with conversion syndrome who was diagnosed with APS. She had complete remission of her symptoms after a heparin trial.

Take-Home Points  

  • Antiphospholipid antibodies are associated with recurrent stroke and dementia. 
  • Patients with POTS respond better to heparin than conventional POTS treatment. 
  • Cerebral blood flow may be the most relevant characteristic of Hughes Syndrome.

Dr. Sanil Rege covers APS in Psychiatry.

Quiz


Learn more

  1. APS and CNS Lupus: Anticoagulation Treatment By Prof Graham Hughes
  2. Antiphospholipid Syndrome (APS) Case Files (Stillbirths and Epilepsy) By Prof Graham Hughes
  3. Antiphospholipid Syndrome (APS) Case Files (Stroke and Thrombosis) By Prof Graham Hughes
  4. Antiphospholipid Syndrome (APS) Case Files (Gut, Tinnitus and Migraine) – Prof Graham Hughes
  5. Antiphospholipid Syndrome (APS):The Clinical Experience – Dr. Graham Hughes
  6. Antiphospholipid Syndrome: 30 Years On – Dr Graham Hughes
  7. Central Nervous System (CNS) Involvement in Antiphospholipid (Hughes) Syndrome – Prof Graham Hughes
  8. Clues to the Diagnosis of Antiphospholipid Syndrome – Prof Graham Hughes
  9. Neuropsychiatric Lupus and The Curious Case of Molly – What Does This Case Teach Us? – Prof Hughes
  10. Pathogenesis of Neuropsychiatric Systemic Lupus Erythematosus – Prof Graham Hughes
  11. What is Lupus? – Prof Graham Hughes
  12. Prof Graham Hughes Introduces You To The World of Lupus and Antiphospholipid Syndrome – Prof Graham Hughes
  13. Q & A session on Lupus, Hughes Syndrome and the Brain – Prof Hughes

References

  1. Hanly JG, Hong C, Smith S, Fisk JD. A prospective analysis of cognitive function and anticardiolipin antibodies in systemic lupus erythematosus. Arthritis Rheum. 1999;42(4):728-734.
  2. Asherson RA, Mercey D, Phillips G, et al. Recurrent stroke and multi-infarct dementia in systemic lupus erythematosus: association with antiphospholipid antibodies. Ann Rheum Dis. 1987;46(8):605-611.
  3. Medina G, Molina-Carrión LE, Angeles-Garay U, et al. Cerebral blood flow abnormalities in neurologically asymptomatic patients with primary antiphospholipid syndrome. Lupus. 2012;21(6):642-648.