Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
Book Review
Brief Report
Case Letter
Case Report
Case Series
Commentary
Current Issue
Editorial
Erratum
Guest Editorial
Images
Images in Neurology
Images in Neuroscience
Images in Neurosciences
Letter to Editor
Letter to the Editor
Letters to Editor
Letters to the Editor
Media and News
None
Notice of Retraction
Obituary
Original Article
Point of View
Position Paper
Review Article
Short Communication
Systematic Review
Systematic Review Article
Technical Note
Techniques in Neurosurgery
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
Book Review
Brief Report
Case Letter
Case Report
Case Series
Commentary
Current Issue
Editorial
Erratum
Guest Editorial
Images
Images in Neurology
Images in Neuroscience
Images in Neurosciences
Letter to Editor
Letter to the Editor
Letters to Editor
Letters to the Editor
Media and News
None
Notice of Retraction
Obituary
Original Article
Point of View
Position Paper
Review Article
Short Communication
Systematic Review
Systematic Review Article
Technical Note
Techniques in Neurosurgery
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
Book Review
Brief Report
Case Letter
Case Report
Case Series
Commentary
Current Issue
Editorial
Erratum
Guest Editorial
Images
Images in Neurology
Images in Neuroscience
Images in Neurosciences
Letter to Editor
Letter to the Editor
Letters to Editor
Letters to the Editor
Media and News
None
Notice of Retraction
Obituary
Original Article
Point of View
Position Paper
Review Article
Short Communication
Systematic Review
Systematic Review Article
Technical Note
Techniques in Neurosurgery
View/Download PDF

Translate this page into:

Commentary
5 (
Suppl 1
); S77-S77

Commentary

Department of Pediatrics, Assiut University, Assiut, Egypt
Department of Neurology and Psychiatry, Assiut University, Assiut, Egypt
Address for correspondence: Dr. Khaled Saad, Department of Pediatrics, Faculty of Medicine, University of Assiut, Assiut 71516, Egypt. E-mail: khaled.ali@med.au.edu.eg

Read COMMENTARY-ARTICLE associated with this -

Licence

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Disclaimer:
This article was originally published by Medknow Publications & Media Pvt Ltd and was migrated to Scientific Scholar after the change of Publisher.

Neuropsychiatric manifestation of systemic lupus erythematosus (SLE) is one of the chief and most detrimental presentations. It involves a wide range of neurological manifestations affecting the central, peripheral and autonomic nervous systems, as well as psychiatric syndromes. The clinical features are characterized by some researchers as either diffuse (e.g. encephalopathy, coma, depression, and psychosis) or complex (e.g. encephalopathy with stroke or seizure and psychiatric presentation with stroke or seizures). Neuropsychiatric manifestations are present in ≈ 50-70% of patients with SLE. However, cerebellar ataxia is rarely reported (<2%).[123] The cause of the cerebellar ataxia in SLE case report in the current issue {Ghosh K, Chatterjee A, Ghosh S, Chakraborty S. Cerebellar ataxia in a young patient: A rare path to lupus. Journal of Neurosciences in Rural Practice}[4] was cerebellar atrophy, other reported causes, including cerebral infarction, antineuronal antibody and vasculopathy.[13]

The diverse nature of CNS syndromes in SLE proposes that different disease mechanisms may be involved. An association between neuropsychiatric symptoms and specific antibodies has been demonstrated in a few studies.[567] Deposition of immune complexes in the choroid plexus, the effects of antibodies; anticardiolipin and anti-neuronal antibodies cause neural cell dysfunction and immune-mediated vascular disease.[567] Lupus anticoagulant and anticardiolipin antibody have been found to be associated with various neurological manifestations in SLE patients. In addition to thrombotic events, antiphospholipid antibodies have been shown to cross-react with epitopes on CNS phospholipids like sphingomyelin, causing neuronal dysfunction.[578] Embolic disease secondary to Libman-Sachs endocarditis and large-vessel vasculitis have also been described.[6]

Although it would be necessary to have diagnostic tests to establish the diagnosis of neuropsychiatric lupus, such tests do not exist. Thus, the approach to lupus patients with neuropsychiatric manifestations, like the lupus case report with cerebellar ataxia[4] consists of confirmation of the diagnosis of SLE. Serologic tests are used to establish the diagnosis of SLE. The antinuclear antibody titer is positive in almost all patients. Serum levels of anti-DNA and anti-Sm antibodies and low levels of complement are useful in the diagnosis of SLE.[12] Other serologic tests include estimation of anticardiolipin, anti-neuronal and antiphospholipid antibodies. The next step is to distinguish between organic and functional etiologies of ataxia and exclude other disorders involving the cerebellum e.g. acute disseminated encephalomyelitis, diffusely infiltrating glioma or lymphoma and drug-related inflammatory processes.[59] MRI is the most useful neuroimaging study in patients with SLE, particularly in those with focal neurologic deficits. It is the modality of choice to demonstrate cerebellar pathology, which may be undetected on CT brain. MRI is highly useful in the diagnosis of acute cerebellar ataxia.[9]

The management SLE patients with CNS involvement depends on immunosuppressive therapies with azathioprine, cyclophosphamide, mycophenoate mofetil and rituximab in association with corticosteroids (oral or intravenous pulse therapy).[5]

References

  1. , , , . Clinical implications of neuropsychiatric systemic lupus erythematosus. Adv Psychiatr Treat. 2009;15:451-8.
    [Google Scholar]
  2. , , , , . Neuropsychiatric lupus erythematosus: A 10-year prospective study on the value of diagnostic tests. Am J Med. 1995;99:153-63.
    [Google Scholar]
  3. , , , , . Acute cerebellar ataxia of a patient with SLE. Clin Neurol Neurosurg. 2000;102:37-9.
    [Google Scholar]
  4. , , , , . Cerebellar ataxia in a young patient: A rare path to lupus. J Neurosci Rural Pract. 2014;5:S75-6.
    [Google Scholar]
  5. , , , . Cerebellar ataxia in systemic lupus erythematosus. Lupus. 2008;17:1122-6.
    [Google Scholar]
  6. , , , , , . Cerebellum and brainstem vasculopathy in systemic lupus erythematosus: Two clinico-pathological cases. Ann Rheum Dis. 1994;53:327-30.
    [Google Scholar]
  7. , , , . Autoantibodies involved in neuropsychiatric SLE and antiphospholipid syndrome. Semin Arthritis Rheum. 2007;36:297-315.
    [Google Scholar]
  8. , , , , , , . Cerebellar ataxia in systemic lupus erythematosus: Three case reports. Ann Rheum Dis. 1988;47:954-6.
    [Google Scholar]
  9. , , , . Neuroimaging in neuropsychiatric systemic lupus erythematosus. Arthritis Rheum. 1999;42:2026-38.
    [Google Scholar]

    Fulltext Views
    182

    PDF downloads
    318
    View/Download PDF
    Download Citations
    BibTeX
    RIS
    Show Sections