Inflammatory optic neuropathy in granulomatosis with polyangiitis can mimick isolated idiopathic optic neuritis

2019 ◽  
pp. 112067211988900
Author(s):  
Manon Clément ◽  
Antoine Néel ◽  
Frédérique Toulgoat ◽  
Michel Weber ◽  
Pascal Godmer ◽  
...  

Objective: We describe a clinico-radiological presentation of inflammatory optic neuropathy that mimicked optic neuritis. Methods: Retrospective single-center case series and literature review of optic neuropathy without orbital pseudotumor. Results: Five local patients fulfilled the inclusion criteria. Clinical presentation revealed rapidly progressive severe unilateral visual loss, retrobulbar pain (n = 4), and paralytic strabismus (simultaneous = 2, protracted = 2) without proptosis. Optic nerve abnormality was not appreciated on initial scan review. Patients did not have any general activity of the granulomatosis with polyangiitis. Upon follow-up magnetic resonance imaging and initial imaging review, all patients revealed orbital apex anomalies. Visual acuity improved in three patients who received high-dose intravenous glucocorticosteroids immediately. Relapse was frequent and visual outcome was poor (final vision > 20/40 in two patients only). Literature review identified 16 well-documented cases of granulomatosis with polyangiitis–related isolated optic neuropathies. Magnetic resonance imaging revealed no abnormality (n = 6), optic nerve and/or sheath involvement (n = 9), apex infiltration (n = 3), and/or pachymeningitis (n = 7). Conclusion: Granulomatosis with polyangiitis is a rare yet potentially blinding cause of inflammatory optic neuropathy. Optic neuropathy in granulomatosis with polyangiitis may occur in the absence of systemic symptoms of disease activity and is challenging to distinguish from other inflammatory and non-inflammatory disorders affecting visual acuity. Several clinical and imaging clues suggest that optic neuropathy results from the development of an extravascular granulomatous process within the optic nerve sheath in the orbital apex, a place that is difficult to image. In a granulomatosis with polyangiitis patient with unexplained visual loss and a seemingly normal workup (fundoscopy, biology, and imaging), clinician should keep a high index of suspicion.

Author(s):  
Samantha D. Sagaser ◽  
John C. Benson ◽  
Laurence J. Eckel ◽  
Sasha A. Mansukhani ◽  
Launia White ◽  
...  

Neurosurgery ◽  
2001 ◽  
Vol 48 (1) ◽  
pp. 214-218 ◽  
Author(s):  
Takeshi Nakajima ◽  
Toshihiro Kumabe ◽  
Hidefumi Jokura ◽  
Takashi Yoshimoto

Abstract OBJECTIVE AND IMPORTANCE Intracranial germinomas often disseminate via the ventricular and subarachnoid pathways, but seeding to the perioptic arachnoid space is extremely unusual. We report two cases of recurrent germinoma seeding in the optic nerve. CLINICAL PRESENTATION Two men with pure germinoma were initially treated with three cycles of a three-drug regimen of bleomycin, etoposide, and cisplatin, and a complete response was achieved. Patient 1 experienced ventricle wall dissemination 10 months after undergoing the initial treatment and was successfully treated with three cycles of carboplatin and etoposide and then by 24-Gy whole-ventricle radiation. Twelve months later, he complained of progressive visual acuity loss, and magnetic resonance imaging demonstrated bilateral enhancement of the optic nerves. Patient 2 also experienced ventricle wall dissemination 3 months after undergoing the initial chemotherapy, but he exhibited a complete response after undergoing 24-Gy whole-ventricle radiation. Two years later, he complained of progressive visual acuity loss. Magnetic resonance imaging demonstrated bilateral enhancement of the optic nerves and cerebellar hemispheres. INTERVENTION None of the locations of recurrence were included in the irradiation field, whereas there was no recurrence within the radiation field. Complete responses were obtained with three cycles of a three-drug regimen of ifosfamide, cisplatin, and etoposide and then by 24-Gy whole-brain radiation that included the bilateral optic nerves. The visual acuity of each patient improved slightly. CONCLUSION Delayed seeding in the optic nerve may result from germinoma cells that remain dormant, so they cannot be destroyed by chemotherapy regimens alone.


Neurosurgery ◽  
2005 ◽  
Vol 57 (1) ◽  
pp. E190-E190 ◽  
Author(s):  
Luis M. Tumialán ◽  
Sanjay S. Dhall ◽  
Valérie Biousse ◽  
Nancy J. Newman

Abstract OBJECTIVE AND IMPORTANCE: The clinical and radiographic presentations of optic nerve gliomas and optic neuritis are for the most part distinct and their diagnoses straightforward. We present two cases illustrating the occasional difficulty one can encounter in distinguishing neoplastic from inflammatory optic neuropathies. CLINICAL PRESENTATION: Patient 1 is a 17-year-old girl who presented with acute onset of pain and rapidly progressive visual loss in the right eye. Patient 2 is a 38-year-old man who presented with painless progressive visual loss in the left eye. INTERVENTION: Patient 1 was initially diagnosed with idiopathic retrobulbar optic neuritis. Interval increase of the optic nerve on magnetic resonance imaging prompted a biopsy of the optic nerve, which revealed a pilocytic astrocytoma. Patient 2 was found to have left optic nerve enhancement most consistent with an optic nerve glioma. Before a biopsy, the patient spontaneously improved without treatment, indicating an inflammatory process. CONCLUSION: Differentiating between optic nerve neoplasm and inflammation may be difficult. On occasion, the classic clinical finding of pain with eye movement and the radiographic finding of enlargement and enhancement of the optic nerve may be misleading. Open biopsy of the optic nerve is indicated only after a completely negative metabolic, infectious, and inflammatory workup; interval increase of the optic nerve on magnetic resonance imaging; and failure of the patient to recover vision.


2015 ◽  
Vol 6 (03) ◽  
pp. 403-404 ◽  
Author(s):  
Mandeep Singh Ghuman ◽  
Shabdeep Kaur ◽  
Samarjit Kaur Bhandal ◽  
Archana Ahluwalia ◽  
Kavita Saggar

ABSTRACTMucormycosis is an emerging disease in diabetes and immunocompromised patients. Rhino-orbito-cerebral mucormycosis is one of the common forms of the disease. Mucormycosis leading to ischemic optic neuropathy is a rare complication. The role of magnetic resonance imaging (MRI) in the diagnosis of ischemic optic neuropathy is limited and uncommonly reported. We report an unusual case of mucormycosis in which MRI revealed bilateral optic nerve infarction, in addition to perineural extension of the fungus along the trigeminal nerve, another uncommon imaging finding.


1999 ◽  
Vol 19 (4) ◽  
pp. 235???237 ◽  
Author(s):  
Andrew G. Lee ◽  
Eric R. Eggenberger ◽  
David I. Kaufman ◽  
Carlos Manrique

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Grigorios T. Sakellariou ◽  
Nicoleta Kefala

Central nervous involvement, mainly with symptoms of cranial neuropathies, occurs in 2–8% of patients with granulomatosis with polyangiitis (GPA). Meningeal involvement, with persistent and severe headache as main manifestation and abnormal thickening and enhancement of the dural mater on postcontrast magnetic resonance imaging, is extremely rare. We present a case of pachymeningitis due to limited GPA, providing simultaneously a literature review.


Sign in / Sign up

Export Citation Format

Share Document