Article

Clinical and neuropathological findings in systemic lupus erythematosus: The role of vasculitis, heart emboli, and thrombotic thrombocytopenic purpura

  1. Dr. Orrin Devinsky MD1,†,*,
  2. Carol K. Petito MD2,
  3. Daniel R. Alonso MD2

Article first published online: 8 OCT 2004

DOI: 10.1002/ana.410230411

Issue

Annals of Neurology

Annals of Neurology

Volume 23, Issue 4, pages 380–384, April 1988

Additional Information

How to Cite

Devinsky, O., Petito, C. K. and Alonso, D. R. (1988), Clinical and neuropathological findings in systemic lupus erythematosus: The role of vasculitis, heart emboli, and thrombotic thrombocytopenic purpura. Ann Neurol., 23: 380–384. doi: 10.1002/ana.410230411

Author Information

  1. 1

    Department of Neurology, The New York Hospital—Cornell University Medical College, New York, NY

  2. 2

    Pathology, The New York Hospital—Cornell University Medical College, New York, NY

  1. †

    Medical Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke, Building 10, Room 5N248, Bethesda, MD 20892

*Department of Neurology, The New York Hospital—Cornell University Medical College, New York, NY

Publication History

  1. Issue published online: 8 OCT 2004
  2. Article first published online: 8 OCT 2004
  3. Manuscript Accepted: 29 OCT 1987
  4. Manuscript Revised: 29 SEP 1987
  5. Manuscript Received: 10 JUL 1987

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Abstract

We reviewed the medical records and autopsy reports of 50 patients with systemic lupus erythematosus to determine the clinical and neuropathological features of this disease. Neuropsychiatric disturbances were found in the majority (74%) of the patients, occurring as psychiatric illness only (5 patients), neurological idsorders only (15 patients), and both together (17 patients). Central nervous system (CNS) lesions were present in half the patients; embolic brain infarcts (10 patients) and CNS infections (8 patients) were the most common. Cardiac sources of emboli were Libman-Sacks endocarditis (5 patients), chronic valvulitis (2 patients), and left side of heart mural thrombus (2 patients). There was no evidence of active CNS vasculitis. Clinical features of thrombotic thrombocytopenic purpura (TTP) developed during the terminal illness in 14 patients, 7 of whom also had pathological evidence of TTP. Correlation between neuropsychiatric disorders and brain lesions could be made in approximately half the patients. This study indicates that cardiac emboli from Libman-Sacks endocarditis and TTP are common pathogenetic factors of CNS disease in systemic lupus erythematosus, whereas CNS vasculitis is rare.

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