Optic Perineuritis in Neuromyelitis Optica Spectrum Disorder

Kamaluddin, Nur Afiah; Tai, Evelyn Li Min; Hitam, Wan Hazabbah Wan; Ibrahim, Mohtar; Samsudin, Ahmad Hadif Zaidin

doi:10.7759/cureus.4834

Cited by 3 publications

(4 citation statements)

References 13 publications

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“…However, vision impairment in OPN has subacute onset, evolving over a period of several weeks, with severity varying from none to severe, and often characterized by descriptions of blurred vision, dimness, or the perception of splotches or "spots" in vision [ 3 ]. Patients diagnosed with OPN might exhibit paracentral or arcuate scotomas instead of the central scotoma typically observed in ON [ 6 ]. They might also have orbital signs such as diplopia, ptosis, ophthalmoparesis, and chemosis [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

“…Patients diagnosed with OPN might exhibit paracentral or arcuate scotomas instead of the central scotoma typically observed in ON [ 6 ]. They might also have orbital signs such as diplopia, ptosis, ophthalmoparesis, and chemosis [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

“…Lumbar puncture cerebrospinal fluid analysis is essential to exclude malignancy and infection within the central nervous system [ 3 ]. In a recent study, some cases that had been diagnosed with idiopathic optic perineuritis were found to be associated with neuromyelitis optica spectrum disorder (NMOSD) [ 6 ]. In the present case, the investigation for anti-aquaporin-4 antibodies was done in view of presence of periventricular hyperintense white matter lesion in MRI that could be due to NMOSD.…”

Section: Discussionmentioning

confidence: 99%

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Light After Darkness: A Case Report of Isolated Optic Perineuritis

Abdul Rahman,

Mohamad,

Abdul Hamid

2024

Cureus

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This is a report on remarkable visual recovery from blindness in a case of isolated optic perineuritis (OPN). A 68-year-old Chinese lady presented with a two-week history of progressive painless bilateral vision loss. Her vision was 6/18 on the right eye and no perception of light (NPL) on the left eye with positive relative afferent pupillary defect (RAPD). Fundus showed hyperaemic and swollen optic disc bilaterally. MRI of the brain and orbit revealed hyperintense periventricular white matter lesions, possibly early changes of multiple sclerosis (MS), and perineural enhancement of optic nerve bilaterally, consistent with OPN. All other investigations were negative. Intravenous methylprednisolone 1g/day for three days was started, followed with oral prednisolone, tapered in three months. At the third month of follow-up, her vision had improved to 6/12 on the left and 6/9 on the right. The hyperaemic and swollen disc has resolved. Intravenous megadose corticosteroid treatment is an effective first-line treatment for OPN.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Light After Darkness: A Case Report of Isolated Optic Perineuritis

Abdul Rahman,

Mohamad,

Abdul Hamid

2024

Cureus

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“…The diagnosis is confirmed by obtaining a fat suppressed, gadolinium-enhanced orbital MRI, showing characteristic avid enhancement of the optic nerve sheath and adjacent orbital fat rather than thickening and enhancement of the optic nerve (11). The parallel linear arrangement of enhancement at the edge of the optic nerve on axial views has been called the “tram-track sign,” and the circular enhancement around the optic nerve on coronal views has been called the “doughnut sign.” The etiologies that cause optic perineuritis comprise a diverse spectrum of inflammatory, infectious, neoplastic, and toxic entities including idiopathic inflammation (11), granulomatosis with polyangiitis (Wegener) (15), Behcet disease (16), giant cell arteritis (17), Crohn disease (18), sarcoidosis (19), herpes viruses (20,21), mycobacteria (22), syphilis (23), toxocara (20), Lyme disease (24), HIV (25), pre–B-cell acute lymphocytic leukemia (20), relapsing polychondritis (26), colon cancer with IgG4-related disease (27), neuromyelitis optica spectrum disorder (28), myelin oligodendrocyte glycoprotein antibody–associated disease (29), and toxic effects of imatinib, methotrexate, cytarabine, and linezolid (20). Phuljhele et al reported MRI imaging findings of optic nerve sheath enhancement in a case of diffuse large B-cell gastric lymphoma but did not specifically diagnose optic perineuritis (30).…”

Section: Discussionmentioning

confidence: 99%

Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia

Parker¹,

Mays²,

Pratibhu³

et al. 2021

Journal of Neuro-Ophthalmology

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Background:To describe the first case of optic perineuritis because of meningeal involvement of early stage chronic lymphocytic leukemia (CLL).Methods:A case report and review of the literature.Results:A case of unilateral optic neuropathy associated with enhancement of the optic nerve sheath is described in a patient with a prior 2-year history of Rai Stage 0 CLL. Lumbar puncture revealed a lymphocytic pleocytosis. Cerebrospinal fluid flow cytometry revealed a monoclonal expansion of CD5+ B cells compatible with CLL, matching the flow cytometry characteristics of his peripheral blood.Conclusions:Optic perineuritis is often initially diagnosed as optic neuritis, yet the 2 have different etiologies and follow a different clinical course. Orbital MRI with contrast structurally separates the 2, revealing a characteristic pattern of peripheral optic nerve sheath rather than primary optic nerve enhancement. Etiologies of optic perineuritis are varied and include inflammatory, infectious, neoplastic, and toxic entities. Central nervous system (CNS) involvement by chronic lymphocytic leukemia is unusual, but cranial nerve and meningeal involvement have been reported. This case adds central nervous system chronic lymphocytic leukemia to the list of differential diagnostic possibilities for optic perineuritis. It also alerts clinicians to consider optic perineuritis as a potential presenting feature of CNS involvement in otherwise asymptomatic and stable CLL.

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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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Optic Perineuritis in Neuromyelitis Optica Spectrum Disorder

Cited by 3 publications

References 13 publications

Light After Darkness: A Case Report of Isolated Optic Perineuritis

Light After Darkness: A Case Report of Isolated Optic Perineuritis

Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia

Optic Perineuritis and Its Association With Autoimmune Diseases

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