Neurosyphilis with Bilateral Optic Perineuritis in an Immunocompetent Patient

Niemeyer, Bruno; Muniz, Bernardo Carvalho; Makita, Lana Sayuri; Marchiori, Edson

doi:10.1159/000488065

Cited by 7 publications

(6 citation statements)

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“…Known autoimmune diseases etiologies of OPN include sarcoidosis (10,11), GAP (12, 13), giant cell arteritis (14, 15), Crohn's disease (16), IgG-4 (17), Behcet's disease (9), and SLE (2). Infectious etiologies of OPN have been associated with acute retinal necrosis (18), syphilis (19,20), herpes zoster (21), tuberculosis (22,23), and Mycoplasma pneumoniae (24). In our study we found the primary causes of OPN were Graves' diseases (12), IgG-4 (10), GPA (4), SLE (2), sarcoidosis (1), Rheumatoid arthritis(1), scleroderma (1), Behcet's disease (1), gout (1), tuberculosis (5), sparganosis (1), ocular Toxoplasmosis (1), bacterial meningitis (1), fungal meningitis (1), and Cytomegalovirus (1).…”

Section: Discussionmentioning

confidence: 99%

Optic Perineuritis and Its Association With Autoimmune Diseases

Zhou

Sun

et al. 2021

Front. Neurol.

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Background: Optic perineuritis (OPN) is a special optic neuropathy that has a distinct etiology from neuromyelitis optica spectrum disorders (NMOSDs) or multiple sclerosis (MS)-related optic neuritis (ON). The mechanisms of how this inflammation developed and invaded the nerve sheath remain unknown. This study is aimed to analyze the etiology and different clinical characteristics of OPN in a Chinese patient population.Methods: Neuro-ophthalmological examination, orbit magnetic resonance imaging (MRI) and a series of blood samples were used in this retrospective observational cohort study to compare characteristics of OPN with idiopathic demyelination optic neuritis (IDON).Results: Forty-four OPN cases (74 eyes) and 61 IDON cases (78 eyes) were analyzed. OPN cases included 33 cases (59 eyes) were associated with specific autoimmune diseases, 10 cases (13 eyes) were associated with infection diseases, 1 case was idiopathic disease. The causes of OPN with CTD were Graves' disease, Immunoglobulin G4-related disease (IgG-4 RD), granulomatosis with polyangiitis (GAP), systemic lupus erythematosus (SLE), Sarcoidosis, Rheumatoid arthritis, scleroderma, Behcet's disease, and gout. All patients received orbital MRI. Overall, 33 cases showed orbit fat infiltration. Specifically, nine cases with IgG-4 RD showed trigeminal nerve branch involvement, 12 cases with Graves' disease showed extraocular muscle belly enlargement, and 4 cases with GAP showed pterygopalatine fossa pseudotumor. Compared to IDON patients, OPN patients were older (p = 0.004) and more likely bilateral involvement 26 (78.79%) patients had bilateral involvement in OPN group vs. 17 (27.87%) in the IDON group (p < 0.001). Visual acuity scores using LogMAR testing was better in OPN patients compared to those with IDON, 0.55 ± 0.91 vs. 1.19 ± 1.24 (p < 0.001). Other ophthalmologic findings unique to the OPN group include 11 (33.33%) cases of ptosis, nine (27.27%) cases of diplopia, and 10 (30.30%) cases of exophthalmos, compared to zero cases of these conditions in the IDON group. Eight (13.11%) IDON patients also had multiple sclerosis (MS) and 7 (11.48%) patients had neuromyelitis which was significantly more than the zero patients in OPN group (p = 0.04).Conclusions: OPN had distinct etiologies and clinical characteristics from IDON and is more often associated with autoimmune diseases. Using OPN characteristics to diagnose autoimmune diseases should prove useful for clinicians when presented with patients that have multiorgan dysfunction that include ophthalmologic findings.

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Section: Discussionmentioning

confidence: 99%

Optic Perineuritis and Its Association With Autoimmune Diseases

Zhou

Sun

et al. 2021

Front. Neurol.

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“…OPN is an uncommon orbit inflammatory disease 7 (18), syphilis (19,20), herpes zoster (21), tuberculosis (22,23), and Mycoplasma pneumoniae (24). In our study we found the primary causes of OPN were Graves' diseases (12), IgG-4(10), GPA (4), SLE (2), sarcoidosis (1), rheumatoid disease (1), scleroderma (1), Behcet's disease (1), gout (1), tuberculosis (5), sparganosis (1), ocular Toxoplasmosis (1), bacterial meningitis (1), fungal meningitis (1), and Cytomegalovirus (1).…”

Section: Discussionmentioning

confidence: 99%

Optic Perineuritis and its Association with Rheumatic Connective Tissue Disease

Zhou

Sun

et al. 2020

Preprint

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Objective Analyze the etiology and clinical characteristics of optic perineuritis (OPN) in a Chinese patient population Methods Neuro-ophthalmological examination and a series of blood samples were used in this retrospective observational cohort study to compare characteristics of OPN with idiopathic demyelination optic neuritis (IDON). Results 44 OPN cases (74 eyes) and 61 IDON cases (78 eyes) were analyzed. The causes of OPN with CTD were Graves’ disease, Immunoglobulin G4-related disease (IgG-4 RD), granulomatosis with polyangiitis (GAP), systemic lupus erythematosus (SLE), Sarcoidosis, Rheumatoid, scleroderma, Behcet's disease, and gout. Overall, 33 cases showed orbit fat infiltration. Specifically, 9 cases with IgG-4 RD showed trigeminal nerve branch involvement, 12 cases with Graves’ showed extraocular muscle belly enlargement, and 4 cases with GAP showed pterygopalatine fossa pseudotumor through orbital magnetic resonance imaging (MRI). Compared to IDON patients, OPN patients were older (p=0.004) and more likely bilateral involvement 26 (78.79%) patients had bilateral involvement in OPN group versus 17 (27.87%) in the IDON group (p<0.001). Visual acuity scores was better in OPN patients compared to those with IDON, 0.55±0.91versus 1.19±1.24 (p<0.001). 8 (13.11%) IDON patients also had multiple sclerosis (MS) and 7(11.48%) patients had neuromyelitis which was significantly more than the 0 patients in OPN group (p=0.04). Conclusions OPN had distinct etiologies and clinical characteristics from IDON and is more often associated with rheumatic CTD. Using OPN characteristics to diagnose CTD should prove useful for clinicians when presented with patients that have multiorgan dysfunction that include ophthalmologic findings.

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“…In addition to predominantly lymphocytic infiltration, there are reports of foci of degenerating collagen and vasculitic changes, perivascular lymphocytic infiltration, and granulomatous inflammation [8 ▪ ]. Moreover, some cases of immune-mediated inflammation within the nerve septa along with adjacent axons of optic nerve being affected have been shown [8 ▪ ,10].…”

Section: Pathogenesismentioning

confidence: 99%

Optic perineuritis

Saitakis

Chwalisz

2022

Current Opinion in Ophthalmology

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Purpose of reviewThis review paper aims at discussing pathogenesis, etiology, clinical features, management, and prognosis of OPN.Recent findingsOptic perineuritis (OPN) is an inflammatory process primarily involving the optic nerve sheath. Clinically, OPN usually presents with unilateral, gradual decline of visual function, eye pain, and/or pain on eye movements, disc edema and various features of optic nerve dysfunction, including visual field defects. It can mimic typical optic neuritis. In most cases of OPN, the disease is isolated with no specific etiology being identified, however, it can also occur secondary to a wide range of underlying systemic diseases. OPN is clinically diagnosed and radiologically confirmed based on the finding of circumferential perineural enhancement of the optic nerve sheath on magnetic resonance imaging (MRI).SummaryUnlike optic nerve, OPN is not typically self-limited without treatment. High-dose oral corticosteroids are the mainstay of treatment in OPN. The initiation of therapy usually causes rapid and dramatic improvement in signs and symptoms. In general, OPN usually has a relatively good visual prognosis, which is influenced by delays between the onset of visual loss and the initiation of steroid therapy as well as the presence of underlying systemic diseases.

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Neurosyphilis with Bilateral Optic Perineuritis in an Immunocompetent Patient

Cited by 7 publications

References 1 publication

Optic Perineuritis and Its Association With Autoimmune Diseases

Optic Perineuritis and Its Association With Autoimmune Diseases

Optic Perineuritis and its Association with Rheumatic Connective Tissue Disease

Optic perineuritis

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