Compressive Optic Neuropathy From Neurosarcoidosis

Lally, Erin; Murchison, Ann P.; Moster, Mark L.; Bilyk, Jurij R.

doi:10.1097/iop.0000000000000342

Cited by 4 publications

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“…Although the treatment is very effective, but it might cover up the systemic symptoms of sarcoidosis. MRI features can be helpful to differentiate neurosarcoidosis from other diseases, such as meningioma, schwannomas, lymphomas, or metastatic conditions [ 12 – 14 ]. In our experience, the discrete features (like the optic and cranial nerves, spinal cord, leptomeninges, and dura mater), rather than a contiguous enhancement of the lesions, will be helpful for differential diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Neurosarcoidosis presenting as CRVO combined CRAO: a biopsy-proven case report of a Chinese patient

et al. 2020

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Background: Neurosarcoidosis is a rare systemic disorder that can affect the eye and other organs, including the central nervous system. Neurosarcoidosis infiltrating the optic nerve presenting as central retinal vein occlusion combined with artery ischaemia has not been reported in the literature previously. We describe a Chinese patient presenting with acute monocular vision loss, in whom an optic nerve biopsy confirmed the diagnosis of neurosarcoidosis. Case presentation: A 47-year-old woman complained of acute decreased vision in her left eye over the course of 1 month. She reported that her vision deteriorated quickly within first 3 days of consulting an ophthalmologist at a local hospital. She was diagnosed with central retinal vein occlusion after funduscopic examination and fundus fluorescein angiography, and the vision in her left eye further deteriorated to no light perception. An orbital magnetic resonance imaging showed an abnormal T1-weighted image of the optic nerve after contrast enhancement. She was referred to a neuro-ophthalmologist for further evaluation. After routine blood tests ruled out infectious and metastatic diseases, she was prescribed 500 mg/d methylprednisolone for 5 days, but her vision did not improve. As she could still not perceive light, an optic nerve biopsy was performed, and the histopathology revealed non-necrotising granuloma that was consistent with neurosarcoidosis. Conclusions: Isolated optic nerve infiltration by neurosarcoidosis without the involvement of the central nervous system or other systemic organs is challenging to diagnose. Biopsy of the optic nerve sheath is crucial for the final diagnosis of neurosarcoidosis. Therefore, a comprehensive ophthalmologic and systemic examination and work-up for inflammation of the eye, chest, and central nervous system should be conducted for atypical cases.

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Section: Discussionmentioning

confidence: 99%

Neurosarcoidosis presenting as CRVO combined CRAO: a biopsy-proven case report of a Chinese patient

et al. 2020

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“…Corticosteroids remain the mainstay of acute treatment: initial dosing of prednisone 1 mg/Kg/die is suggested in presence of CNS abnormalities, whilst prednisone 0.5 mg/kg/die may be sufficient in case of cranial and or peripheral neuropathy. Nonresponders or patients affected by severe disease at presentation may benefit from course of a 3–5-day intravenous methylprednisolone 1 g/die [ 8 , 9 ]. Patients with NS require in all cases a prolonged course of glucorticoids therapy (at least 6–12 weeks) and progressive tapering.…”

Section: Introductionmentioning

confidence: 99%

Corticosteroid treatment for acute hydrocephalus in neurosarcoidosis: a case report

Schilke,

Remoli,

Cutellé

et al. 2024

J Med Case Reports

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Background Neurosarcoidosis occurs symptomatically in 5–10% of patients with sarcoidosis, and hydrocephalus is a rare complication of neurosarcoidosis, with either acute or subacute onset and presenting symptoms related to increased intracranial pressure. It represents a potentially fatal manifestation with a mortality rate of 22% (increased to 75% in case of coexistence of seizures) that requires a prompt initiation of treatment. High-dose intravenous corticosteroid treatment and neurosurgical treatment must be considered in all cases of neurosarcoidosis hydrocephalus. Case presentation Here we present a case of hydrocephalus in neurosarcoidosis, complicated by generalized seizures, in a 29-year-old Caucasian male patient treated with medical treatment only, with optimal response. Conclusion Since neurosurgery treatment can lead to severe complications, this case report underlines the possibility to undergo only medical treatment in selected cases. Further studies are needed to stratify patients and better identify those eligible for only medical approach.

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Neurosarcoidosis

Ungprasert¹,

Matteson²

2017

Rheumatic Disease Clinics of North America

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Neurosarcoidosis occurs in 3% to 10% of patients with sarcoidosis. Cranial neuropathy and meningeal involvement are the most common manifestations, but any part of the nervous system can be affected. Definite diagnosis requires the presence of noncaseating granuloma in the nervous system, although histopathologic confirmation is often not obtainable. Moderate to high dose of glucocorticoids is the main therapy for neurosarcoidosis. Relapse often occurs after the dose of glucocorticoids is tapered down, often necessitating the use of steroid-sparing immunosuppressive agents.

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Compressive Optic Neuropathy From Neurosarcoidosis

Cited by 4 publications

References 0 publications

Neurosarcoidosis presenting as CRVO combined CRAO: a biopsy-proven case report of a Chinese patient

Neurosarcoidosis presenting as CRVO combined CRAO: a biopsy-proven case report of a Chinese patient

Corticosteroid treatment for acute hydrocephalus in neurosarcoidosis: a case report

Neurosarcoidosis

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