Bilateral Tonic Pupils as the Initial Manifestation of Sjögren’s Syndrome

Bilateral internuclear ophthalmoplegia has been linked with various pathological conditions of the central nervous system (CNS), such as multiple sclerosis, stroke, tumours, and brainstem inflammatory processes. Herein the authors report a case of a 45-year-old female patient who presented with diplopia due to bilateral internuclear ophthalmoplegia, with no evidence of brainstem lesion in brain magnetic resonance imaging (MRI) and was diagnosed with primary Sjögren's syndrome. ARTICLE HISTORY

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“…14 Bilateral Adie tonic pupil due to autonomic failure 15,16 and opsoclonus 17 are rare neuroophthalmological features of pSS.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Internuclear Ophthalmoplegia as a Presenting Manifestation of Primary Sjögren’s Syndrome

Natsis

Boura

Kyriazis

et al. 2016

Neuro-Ophthalmology

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“…Re tais at vejais ðià pa to lo gi jà ga li su kel ti au to imu ni nës li gos (Sjog reno sin dro mas, sis te mi në skle ro zë, sar koi do zë, Gul lain-Bar re sin dro mas, Vogt-Ko y a na gi-Ha ra da li ga). Sjog re no sin dro mas ga li su kel ti Adie vyz dá dël au to imu ni nio krumply no maz go uþ de gi mo [10]. Ap ra ðy ti at ve jai, kai sis te minë skle ro zë su ke lia au to imu ni nae krum ply no maz go disfunk ci jà ir le mia Adie vyz dá, kaip pa grin di ná ðios au to imuni nës li gos simp to mà [7].…”

Section: Etiologijaunclassified

“…To ni nis vyz dys taip pat sie ja mas su pa ra ne op las ti niais sin dro mais (Hodþ ki no lim fo ma, krû tø vë þiu, tar pup lau èio smul kiø jø làs te liø kar ci no ma, ade noi di ne cis ti ne kar ci noma), anes te zi ja, vais tø var to ji mu ir ki to mis bûk lë mis [7,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28].…”

Section: Etiologijaunclassified

Adie pupil. Literature review

Judickaitė,

Zizas,

Varoniukaitė

et al. 2023

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Anisocoria (unequal pupil size) is a condition described as various lacerations in iris sphincter or dilator muscles, innervation abnormalities, or conditions caused by external pharmacological agents. Adie pupil is one of the causes of anisocoria. Adie pupil is a rare neurological disorder which represents itself with unilateral or bilateral mydriasis, unresponsiveness to bright or dim light, and constriction on accommodation. This condition is idiopathic, affecting 25-45-year-old adults, more frequently women. The symptoms are a unilateral or bilateral dilated pupil, photophobia, and blurred vision. The diagnosis is based on patients’ symptoms, clinical examination findings, and a dilute pilocarpine test can also be performed. Most patients do not need treatment. In this article, we review the epidemiology, pathogenesis, etiology, clinical evaluation, diagnostic features, treatment of Adie pupil, its relationship with COVID-19, and other conditions that cause anisocoria.

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“…Autonomic neuropathy characterized by various autonomic features including orthostatic intolerance (OI), and gastrointestinal (GI), sudomotor, pupillary and genitourinal impairment in patients with SS may be the initial manifestation and before or after the appearance of the "sicca" symptoms, accompanied by symptoms other than dysautonomia including sensory disturbance [36][37][38][39][40][41][42][43][44][45][46][47][48][49][50][51][52][53][54][55].…”

Section: Autonomic Dysfunction In Sjögren's Syndromementioning

confidence: 99%

“…It could be localized [41,47,53] or systemic in distribution. Although autonomic neuropathy is potentially immunoresponsive, immunotherapy with intravenous immunoglobulin (IVIg) may require repetitive, continuous, or adjunctive therapy with rituximab for sustained improvement [56].…”

Section: Autonomic Dysfunction In Sjögren's Syndromementioning

confidence: 99%

Ganglionic Acetylcholine Receptor Antibodies and Autonomic Dysfunction in Autoimmune Rheumatic Diseases

Imamura

Mukaino

Takamatsu

et al. 2020

IJMS

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Autonomic neuropathy has been reported in autoimmune rheumatic diseases (ARD) including Sjögren’s syndrome, systemic sclerosis, rheumatoid arthritis, and systemic lupus erythematosus. However, the pathophysiological mechanism underlying autonomic dysfunction remains unknown to researchers. On the other hand, autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder, which causes dysautonomia that is mediated by autoantibodies against ganglionic acetylcholine receptors (gAChRs). The purpose of this review was to describe the characteristics of autonomic disturbance through previous case reports and the functional tests used in these studies and address the importance of anti-gAChR antibodies. We have established luciferase immunoprecipitation systems to detect antibodies against gAChR in the past and determined the prevalence of gAChR antibodies in various autoimmune diseases including AAG and rheumatic diseases. Autonomic dysfunction, which affects lower parasympathetic and higher sympathetic activity, is usually observed in ARD. The anti-gAChR antibodies may play a crucial role in autonomic dysfunction observed in ARD. Further studies are necessary to determine whether anti-gAChR antibody levels are correlated with the severity of autonomic dysfunction in ARD.

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Bilateral Tonic Pupils as the Initial Manifestation of Sjögren’s Syndrome

Cited by 10 publications

References 11 publications

Bilateral Internuclear Ophthalmoplegia as a Presenting Manifestation of Primary Sjögren’s Syndrome

Bilateral Internuclear Ophthalmoplegia as a Presenting Manifestation of Primary Sjögren’s Syndrome

Adie pupil. Literature review

Ganglionic Acetylcholine Receptor Antibodies and Autonomic Dysfunction in Autoimmune Rheumatic Diseases

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