Neurosarcoidosis masquerading as glioma of the optic chiasm in a child

Ng, K L; McDermott, N.; Romanowski, C.A.J.; Jackson, Alan

doi:10.1136/pgmj.71.835.265

Cited by 24 publications

(8 citation statements)

References 5 publications

(1 reference statement)

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“…2 Hovda and colleagues and Ng and colleagues have reported bilateral involvement of the optic nerves, but these were associated with extensive leptomeningeal involvement. 7,12 Our case also responded dramatically to corticosteroid treatment, as Beck's case. MRI has been shown to be sensitive yet nonspecific in identifying neurosarcoidosis.…”

Section: Figure 5 (Continued)mentioning

confidence: 55%

Primary Bilateral Optic Nerve Sarcoidosis

Yilmaz

Atalay

Özen

et al. 2005

Neuro-Ophthalmology

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Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. The nervous system is involved in 5%-15% of patients. In 1%-5% of patients, sarcoidosis affects the optic nerve in some manner. Sarcoid manifesting as an isolated tumor of the optic nerve is uncommon. We report here a 50-yearold woman with primary bilateral optic nerve sarcoidosis. She had a history of progressive, painless visual loss over a 2-month period. Her cerebral magnetic resonance imaging (MRI) scan revealed diffuse thickening and enhancement of the optic chiasm affecting the pre-chiasmatic optic nerves. Surgery was performed for histopathological diagnosis and decompression of the optic nerve. Analysis of a biopsy specimen confirmed a diagnosis of sarcoidosis, and the patient was treated successfully with corticosteroids.

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Section: Figure 5 (Continued)mentioning

confidence: 55%

Primary Bilateral Optic Nerve Sarcoidosis

Yilmaz

Atalay

Özen

et al. 2005

Neuro-Ophthalmology

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“…Clinically, optic nerve involvement may manifest as papilledema, papillitis, retrobulbar neuritis, a compressive optic neuropathy associated with an extra-axial mass lesion, or an infiltrative optic neuropathy. Because of the diverse range of its clinical features, the diagnosis of neurosarcoidosis can be quite challenging, and although MRI is sensitive, it is nonspecific (9). Because of the diverse range of its clinical features, the diagnosis of neurosarcoidosis can be quite challenging, and although MRI is sensitive, it is nonspecific (9).…”

Section: Dr Vaphiadesmentioning

confidence: 99%

“…enhancement and thickening (5,9), findings that are especially useful in cases with a negative systemic workup (9). enhancement and thickening (5,9), findings that are especially useful in cases with a negative systemic workup (9).…”

Section: Figmentioning

confidence: 99%

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Progressive Bilateral Visual and Hearing Loss in an Elderly Woman

Vaphiades

Gupta

Palmer

et al. 2010

Journal of Neuro-Ophthalmology

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Dr. Vaphiades:A 63-year-old white woman with a history of previously treated tuberculosis and a 10-year history of bilateral hearing loss presented with a complaint of progressive visual loss in both eyes over the last 2 years. She denied tobacco, alcohol, or drug abuse. At the time of initial vision loss, she had been found to have a swollen right optic disc; however, 2 weeks later, she was noted to have bilateral optic disc swelling. Initial brain and orbital MRI was interpreted as showing no abnormalities. Repeat MRI 9 months later showed marked bilateral optic nerve enhancement. The patient was treated with high-dose corticosteroids without improvement. In the meantime, a variety of serologic and blood studies were performed, including complete blood count, erythrocyte sedimentation rate, C-reactive protein, Lyme, Bartonella, Brucella, Toxoplasma, rickettsial, and antinuclear antibody titers, serology for syphilis, and testing for human immunodeficiency virus. All gave negative or normal results. A lumbar puncture revealed no abnormalities other than 10 white blood cells, all monocytes. The cerebrospinal fluid (CSF) glucose was 73 mg/dL (normal: 40-80 mg/dL), and the CSF protein was 34 mg/dL (normal: 15-45 mg/dL). The fluid was negative for cryptococcal antibody, there were no oligoclonal bands, and cultures for bacteria, including acid-fast bacteria, showed no growth. Cytology was negative for malignancy but showed small mature lymphocytes. CT of the chest and a gallium scan showed no lesions. Bilateral temporal artery biopsies showed no evidence of vasculitis. The patient was referred to our institute for an assessment.On examination, the patient's vital signs were normal. Her visual acuity was light perception, right eye, and no light perception, left eye. Color vision was absent on the right. The pupils measured 7 mm with the right pupil minimally reactive to direct light stimulation, and the left was nonreactive. There was a left relative afferent pupillary defect. Extraocular movements were full, but there was a 40-prism diopter comitant right exotropia by Krimsky testing. There was no proptosis. Slit-lamp examination revealed normal corneas, anterior segments, and intraocular pressures. The ocular fundi showed bilateral optic disc pallor. There was no vascular sheathing, and there were no retinal hemorrhages or exudates. Trigeminal and facial nerve function were normal bilaterally.The patient was treated with another course of systemic steroids without improvement in vision. In the meantime, further blood tests were ordered, including tests for the common Leber hereditary optic neuropathy mitochondrial mutations and assays for collapsin response mediator protein-5, angiotensin-converting enzyme (ACE), and aquaporin-4 antibody. All gave negative or normal results. A third brain and orbital MRI was performed (Figs. 1-3).Section Editor: Neil R. Miller, MD FIG. 1. Fat-suppressed gadolinium-enhanced T1 axial (A) and coronal (B) MRI of the orbits demonstrate uniform thickening and enhancement of both optic n...

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“…11). [34][35][36][37][38][39] If diagnosed appropriately, optic neuropathy can be treated with corticosteroids and often responds well. However, some cases are refractory to corticosteroid treatment and eventually progress to complete visual loss.…”

Section: Optic Nervementioning

confidence: 99%

Neuro-ophthalmic Complications of Sarcoidosis

Constantino¹,

Digre²,

Zimmerman³

2000

Semin Neurol

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Sarcoidosis is a systemic disorder that is caused by granulomatous changes of uncertain etiology and commonly has multiorgan involvement. Ocular involvement may occur in up to 32% of persons afflicted by sarcoid. The neuro-ophthalmic manifestations of sarcoid, in particular, are varied and may affect any portion of the visual system, including neural structures. Diagnosis is often difficult due to the fact that the clinical presentation can mimic other disorders, such as Multiple Sclerosis, and therefore a systematic approach to testing must be used once the diagnosis has been considered. The importance of diagnosing neuro-ophthalmic sarcoid lies in the fact that it is a treatable disease. The mainstay of treatment is corticosteroids although other immunosuppressive agents may be used. The long-term prognosis of neuro-ophthalmic sarcoid has not been studied in large patient populations, but the data that is available suggests that remission may occur in up to 47%.

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Neurosarcoidosis masquerading as glioma of the optic chiasm in a child

Cited by 24 publications

References 5 publications

Primary Bilateral Optic Nerve Sarcoidosis

Primary Bilateral Optic Nerve Sarcoidosis

Progressive Bilateral Visual and Hearing Loss in an Elderly Woman

Neuro-ophthalmic Complications of Sarcoidosis

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