Optic neuropathy and central nervous system disease associated with primary Sjögren's syndrome

doi:10.1016/0002-9343(92)90788-D

The American Journal of Medicine

Volume 92, Issue 6, June 1992, Pages 686-692

https://doi.org/10.1016/0002-9343(92)90788-D Get rights and content

Abstract

Three cases of optic neuropathy associated with primary Sjögren's syndrome are reported. All three patients had clinical manifestations of primary Sjögren's syndrome, although two of the patients did not report sicca symptoms at initial examination. Two patients had focal neurologic signs in addition to optic neuropathy. The differentiation of this syndrome of optic neuropathy, focal neurologic signs, and Sjögren's syndrome from multiple sclerosis and antiphospholipid antibody syndrome is important for reasons of treatment and prognosis. This diagnostic differentiation was facilitated by positive tests for xerophthalmia and findings of positive minor salivary gland biopsy. High titers of antinuclear antibody, anti-SSA(Ro), and antiSSB(La), and the absence of antiphospholipid antibodies provided additional help in the differential diagnosis. In 5 years of observation, none of the patients developed symptoms of multiple sclerosis or additional connective tissue disorders.

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Atypical forms of optic neuritis
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Citation Excerpt :
However, in our experience, and when a large screening of the literature is made, the frequency of these pathologies is very rare. The most frequent findings may be Sjögren syndrome (Tesar et al., 1992; Govoni et al., 2001; Delalande et al., 2004), sometimes associated with myelitis conducting to a diagnosis of NMO, this association being also observed with lupus (Pittock et al., 2008). Another possible diagnosis, in the field of systemic disease, is sarcoidosis.
Inflammatory optic neuritis (ON) represents a frequent clinical situation in neurology and ophthalmology. The most current etiology is multiple sclerosis (MS) but, when MRI and Cerebrospinal fluid (CSF) analyses are normal, ON is usually considered as “idiopathic” with a suspected viral etiology. In rare cases, a systemic disease such as sarcoidosis, lupus or Sjögren syndrome may be diagnosed. In several cases either a recurrence or a myelitis may occur without any argument for MS. In the first case, it corresponds to relapsing inflammatory optic neuritis (RION) and in the second case to neuromyelitis optica (NMO). In the present paper, the author successively presents the various clinical situations and complementary findings (infectious, vasculitis, NMO or idiopathic) that can lead to a differential diagnosis of MS in a context of ON.
La neuropathie optique (NO) est une situation fréquente en neurologie et en ophtalmologie. L’étiologie la plus courante est la sclérose en plaques (SEP) mais quand l’IRM et l’analyse du liquide céphalorachidien sont normales, la NO est habituellement considérée comme idiopathique, potentiellement d’origine virale. Dans de rares cas une maladie systémique (lupus, sarcoïdose, Sjögren) est retrouvée. Dans d’autres cas, une récidive de NO homo- ou controlatérale correspondant à une recurrent optic neuritis (RON), voire l’apparition d’une myélite peut être observée. Dans cet article, l’auteur propose d’aborder successivement ces différents cas de figure (infection, vascularite, NMO ou RON) qui constituent les principaux diagnostics différentiels de la SEP dans le domaine de la NO.
Neurological manifestations in Sjögren syndrome
2010, Revue de Medecine Interne
Optic neuritis revealing a primary Gougerot-Sjögren syndrome
2010, Pratique Neurologique - FMC
Central nervous system involvement in primary Sjögren syndrome
2009, Medicina Clinica
El síndrome de Sjögren (SS) es una enfermedad sistémica autoinmunitaria que se caracteriza fundamentalmente por la afección de las glándulas de secreción exocrina. Aunque sus principales síntomas son la xerostomía y la xeroftalmía, el SS puede afectar a cualquier órgano o sistema. La afección del sistema nervioso central (SNC) se considera una de las manifestaciones sistémicas típicas del SS primario, aunque las principales cohortes de pacientes muestran una prevalencia inferior al 5%. En la práctica clínica, el espectro de manifestaciones neurológicas en el SS es muy amplio e incluye desde formas asintomáticas (lesiones desmielinizantes en sustancia blanca) hasta formas de afectación focal o difusa cerebral, de médula espinal o epilepsia. Las situaciones que con más frecuencia inducen una mayor complejidad en el diagnóstico diferencial de la afección del SNC en el paciente con SS primario son la presencia de otras enfermedades autoinmunitarias sistémicas, la enfermedad cerebrovascular y la esclerosis múltiple.
Sjögren syndrome (SS) is a systemic autoimmune disease that affects the exocrine glands and presents with sicca symptoms of the main mucosa surfaces. The spectrum of the disease extends from sicca syndrome to systemic involvement (extraglandular manifestations). Although earlier studies described central nervous system (CNS) involvement as a frequent extraglandular manifestation of primary SS, symptomatic CNS involvement is rarely described in recent large series (<5%). A wide spectrum of neurological features has been described, including not only asymptomatic white matter lesions in MR examinations, but also severe focal or diffuse neurological processes. Differential diagnosis includes the coexistence of other systemic autoimmune diseases (lupus, antiphospholipid syndrome, vasculitis), cerebrovascular disease and multiple sclerosis.
Bilateral optic neuropathy revealing Sjögren's syndrome
2008, Revue Neurologique
The central nervous system involvement has been reported in 20% of cases of primary Sjogrën's syndrome (SS), a chronic autoimmune disease characterized by a disorder of the exocrine glands secondary to progressive lymphocyte infiltration. Classically described neurological manifestations include sensorimotor deficits, aseptic meningitis or meningoencephalitis, multiple sclerosis-like syndromes and myeolopathies.
We report here the case of a 53-year-old woman who exhibited rapidly progressive visual loss, disclosing bilateral optic neuropathy, as an uncommon initial symptom of primary SS. Examination of CSF revealed associated aseptic meningitis. Because of the lack of efficacy of the first treatment by intravenous corticosteroids, monthly intravenous cyclophosphamide was quickly introduced. After six months, significant visual recovery was observed.
Optic neuropathies have been rarely reported as the initial symptom revealing primary Sjogrën syndrome, and bilateral simultaneous lesions remain exceptional.
Le syndrome de Gougerot-Sjogrën primitif est une maladie auto-immune caractérisée par une infiltration lymphocytaire progressive des glandes exocrines. Des atteintes du système nerveux central ont été rapportées dans 20 % des cas. Les manifestations neurologiques classiquement décrites comportent des déficits sensitivomoteurs, des méningites aseptiques, des méningo-encéphalites, des myélopathies ou encore une symptomatologie mimant une sclérose en plaque.
Nous rapportons le cas d’une patiente qui présenta une baisse visuelle bilatérale rapidement progressive révélant une neuropathie optique bilatérale. Le diagnostic de syndrome de Gougerot-Sjogrën primitif fut alors posé et l’étude du LCR mit en évidence une méningite aseptique associée. Du fait de l’échec du traitement initial par corticothérapie intraveineuse, des cures mensuelles de cyclophosphamide intraveineux furent débutées. Après six mois de traitement, une récupération visuelle significative fut constatée.
Les neuropathies optiques ont été rarement rapportées comme symptôme initial d’un syndrome de Gougerot-Sjogrën et une atteinte bilatérale simultanée est exceptionnelle.
Chapter 7 Inflammatory Optic Neuropathies Not Associated with Multiple Sclerosis
2008, Blue Books of Neurology
Subacute optic neuropathies that develop with and without pain may arise in a diverse and wide-ranging series of inflammatory disorders. This chapter makes note of all those disorders in which optic neuropathy may arise. It is noted that vasculitic disorders are included. Some optic neuropathies arise as a consequence of ischemia following thrombotic occlusion of the ophthalmic artery or its branches, and it is clear that recovery of visual function under these circumstances may merely be a forlorn hope. However, other vasculitic disorders may present with symptoms and signs of an optic neuropathy indistinguishable on clinical grounds and prompt treatment of which leads often to a substantial improvement of visual function. Wegener's granulomatosis, Churg–Strauss syndrome, and lupus are examples of this circumstance. It is important, therefore, to understand that vigorous and urgent investigation of such cases may lead to instigation of treatment sufficiently early to allow a recovery to take place. The relationship between the ophthalmic and the neurologic complications of these disorders is also underlined in the chapter; the use by neurologists of an ophthalmic consultation and vice versa as a mandatory collaboration rather than a test under these circumstances is an extremely important message to understand.

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^☆: This work was supported by funds of the Department of Clinical Investigation, Walter Reed Army Medical Center, Washington, D.C.

^☆☆: The opinions and assertions contained herein are solely the views of the authors and should not be taken as representing the views of the U.S. Army or Department of Defense.

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Case reportOptic neuropathy and central nervous system disease associated with primary Sjögren's syndrome☆,☆☆

Abstract

Am J Ophthalmol

Surv Ophthalmol

Ophthalmology

Ophthalmology

Am J Ophthalmol

Lancet

Am J Med

J Autoimmun

Am J Med

Am J Ophthalmol

Anterior ischemic optic neuropathy

Inflammatory optic neuropathies

Optic neuropathy in systemic lupus erythematosus

Arch Ophthalmol

Optic neuritis in systemic lupus erythematosus

J Rheumatol

Systemic lupus erythematosus presenting as optic neuropathy

Ann Ophthalmol

Polyarteritis nodosa presenting as posterior ischaemic optic neuropathy

JR Soc Med

Sarcoidosis and its ophthalmic manifestations

Am J Ophthal

Optic neuritis in primary Sjögren's syndrome

Clin Neurol Neurosurg

Optic neuropathy as an initial manifestation of Sjögren's syndrome

J Rheumatol

Sjögren's syndrome: central nervous system manifestations

Neurology

Neurologic complications of primary Sjögren's syndrome

Medicine (Baltimore)

Magnetic resonance imaging of cerebral lesions in patients with Sjögren's syndrome

Ann Intern Med

The incidence of optic neuritis and its prognosis for multiple sclerosis

Acta Neurol Scand

Sjögren's syndrome

Sjögren's syndrome 2. Clinical associations and immunologic phenomena

QJ Med

Sjögren's syndrome

Bull Rheum Dis

Case report
Optic neuropathy and central nervous system disease associated with primary Sjögren's syndrome☆,☆☆