Neurology -- Correspondence for Joseph et al., 69 (7) 644-654

Correspondence published:

CNS lupus: A study of 41 patients: Edgar Avalos Herrera, Silvia Y. Alvarez, Carlos E. Pacay (18 October 2007)

Reply from the authors: Neil Scolding, Fady Joseph (18 October 2007)

CNS lupus: A study of 41 patients 18 October 2007

Edgar Avalos Herrera,
Hospital General San Juan de Dios
Guatemala,
Silvia Y. Alvarez, Carlos E. Pacay
Send Correspondence to journal:
Re: CNS lupus: A study of 41 patients

eavalosh{at}yahoo.com Edgar Avalos Herrera, et al.

We read the article by Joseph et al with interest. [1] The authors did not mention that roughly 30% to 90% of CNS-SLE is associated with serum antineuronal antibodies compared to 5% to 20% in non-CNS-SLE. In addition, they do not mention that CSF IgG antineuronal antibodies (with specificity against lymphocytic, neuronal membrane, and neuronal intracellular antigens) can be found in up to 90% in CNS-SLE compared with 10% in non-CNS-SLE.[3]
These antibodies can be used as markers of CNS-SLE but only if found in CSF with direct binding to brain tissue. The amount of antibodies can be correlated with diffuse CNS manifestations like the 7/11 patients with normal scans who had headaches, meningism, memory impairment, and confusion. [1, 2, 3] There is a report where one patient followed serially with CSF antineuronal antibodies had a decrease in activity of these antibodies when CNS-SLE was treated. During an exacerbation of CNS- SLE, the activity of these markers increased. [3]
Joseph et al [1] also mentioned that movement disorders are not part of American College of Rheumatology (ACR) criteria for CNS-SLE but, in more recent reviews, movement disorders are part of this criteria. [3, 4, 5] The presence of myoclonus wasn't previously reported in ACR reviews. Chorea is not considered a "hallmark of disease" by ACR, but in a patient with ANA antibodies and chorea the suspected diagnosis of SLE is very strong. [3]
It is interesting that ten of the reported patients (24%) had "CNS manifestations as the initial presenting feature of SLE." Current data on this are lacking, and we agree with the authors that ACR criteria do not suggest how to handle these diagnostic challenges.
We recommend making a proposal to ACR for an exception in patients with CNS-SLE that does not fill 4 of 11 criteria. SLE should be suspected in a patient meeting these criteria: High suspicion with CNS manifestations alone together with CSF antineuronal antibodies. It should be noted that making the diagnosis of CNS-SLE is different than other non-CNS-SLE cases.
References
1. Joseph FG, Lammie GA, Scolding NJ. CNS lupus: A study of 41 patients. Neurology 2007; 69: 644-654.
2.Greenwood DLV, Gitlits VM, Alderuccio F et al. Autoantibodies in neuropsychiatric lupus. Autoimmunity 2002;35:79-85.
3. Wallace DJ, Hahn BH. In: Dubois' Lupus Erythematosus, Seventh Edition, Lippincott Williams & Wilkins, 2006.
4. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999;42:599-608.
5. Hanly JG, ACR classification criteria for systemic lupus erythematosus: limitations and revisions to neuropsychiatric variables Lupus 2004;13:861�864.
Disclosure: The authors report no conflicts of interest.

Reply from the authors 18 October 2007

Neil Scolding,
University of Bristol Institute of Clinical Neurosciences
as per manuscript,
Fady Joseph
Send Correspondence to journal:
Re: Reply from the authors

n.j.scolding{at}bristol.ac.uk Neil Scolding, et al.

We thank Avalos et al for their interest and their comments. Our study was not intended to be a comprehensive literature review of neurological lupus, but a clinical practical study.
Anti-neuronal antibodies are not routinely sought in lupus patients in the UK and so we had nothing to report on this aspect. However, as we previously noted, there is not a consensus on their diagnostic or monitoring value. [6] In one study we reviewed in this article, only 19% of patients with clinical CNS lupus exacerbations exhibited an accompanying rise in anti-CNS antibodies. Other studies found no association between brain pathology and autoantibodies, no definite correlation with CNS symptoms, or CNS antibody positive patients without cerebral involvement.
Other reviews and studies specifically looking at this question have concluded that �no specificity was encountered among brain-specific or systemic Abs for any single NP manifestation.� [7] In addition, �the presence of antineuronal antibodies adds little if any information beyond that obtained by clinical examination, neuropsychological testing, and MRI� [8] and finally �the search for specific antibody marker(s) that can be applied for the routine laboratory diagnosis for neuropsychiatric lupus remains elusive�. [2]
We accept that movement disorders are included in the ACR criteria [4], and we are grateful to Avalos et al for pointing out this error in our Discussion. Our original intention was to point out that other than chorea, specific movement disorders were not detailed in these criteria. On this point, Avalos et al assert that chorea is not considered a "hallmark of disease" by ACR. This may now be valid, but the 1999 criteria names chorea as the only specified movement disorder, and indicate that �Chorea is the most common movement disorder observed in SLE�. [4]
References
6. Scolding NJ, Joseph FG. The neuropathology and pathogenesis of systemic lupus erythematosus. Neuropathol Appl Neurobiol 2002;28:173-189.
7. Zandman-Goddard G, Chapman J, Shoenfeld Y. Autoantibodies involved in neuropsychiatric SLE and antiphospholipid syndrome. Semin Arthritis Rheum 2007;36:297-315.
8. Sailer M, Burchert W, Ehrenheim C, et al. Positron emission tomography and magnetic resonance imaging for cerebral involvement in patients with systemic lupus erythematosus. J Neurol 1997;244:186-193.
Disclosure: The authors report no conflicts of interest.