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• W2013886352 abstract "Sarcoid manifesting as an optic nerve tumor without evidence of systemic disease is uncommon. Throughout a 2-year period, a 22-year-old white woman had progressive monocular loss of vision to the level of no light perception. Optic atrophy but no uveitis was noted in the affected eye. Magnetic resonance imaging revealed thickening and enhancement of the apical optic nerve, with “tram-tracking.” The presumptive diagnosis was optic nerve sheath meningioma; however, a biopsy specimen from the optic nerve revealed sarcoid. Extensive postoperative investigations revealed no systemic sarcoidosis. To our knowledge, 17 cases similar to ours, with the diagnosis proved by optic nerve biopsy, have been previously reported in the English-language literature. Most of these were mistaken preoperatively for optic nerve sheath meningioma. None of the patients had evidence of systemic sarcoidosis on initial postoperative testing. Neuroimaging, serum level of angiotensin-converting enzyme, and clinical characteristics such as age, race, sex, and optochoroidal collaterals do not distinguish optic nerve sheath meningioma from sarcoid of the optic nerve. In the absence of uveitis or systemic involvement, optic nerve sarcoid manifesting as an orbital tumor is virtually impossible to diagnose without results of biopsy. Sarcoid manifesting as an optic nerve tumor without evidence of systemic disease is uncommon. Throughout a 2-year period, a 22-year-old white woman had progressive monocular loss of vision to the level of no light perception. Optic atrophy but no uveitis was noted in the affected eye. Magnetic resonance imaging revealed thickening and enhancement of the apical optic nerve, with “tram-tracking.” The presumptive diagnosis was optic nerve sheath meningioma; however, a biopsy specimen from the optic nerve revealed sarcoid. Extensive postoperative investigations revealed no systemic sarcoidosis. To our knowledge, 17 cases similar to ours, with the diagnosis proved by optic nerve biopsy, have been previously reported in the English-language literature. Most of these were mistaken preoperatively for optic nerve sheath meningioma. None of the patients had evidence of systemic sarcoidosis on initial postoperative testing. Neuroimaging, serum level of angiotensin-converting enzyme, and clinical characteristics such as age, race, sex, and optochoroidal collaterals do not distinguish optic nerve sheath meningioma from sarcoid of the optic nerve. In the absence of uveitis or systemic involvement, optic nerve sarcoid manifesting as an orbital tumor is virtually impossible to diagnose without results of biopsy. Meningioma of the optic nerve sheath characteristically manifests with gradual progressive visual loss in young women. On neuroimaging, “tram-tracking” of the optic nerve sheath may be evident. We describe a patient with isolated optic nerve sarcoid who had clinical and radiologie findings similar to those of optic nerve sheath meningioma. A 22-year-old white woman who had been taking oral contraceptives for 5 years noted decreased vision in her left eye in November 1992. Medical records of an examination performed elsewhere in February 1992 documented visual acuity of 20/20 and 20/40 in the right and left eyes, respectively. Pallor of the left optic disk and inferotemporal visual field loss were noted in December 1993, but no abnormalities were reported in the right eye. At that time, computed tomography (CT) of the head and orbits revealed no abnormality; in February 1994, magnetic resonance imaging (MRI) showed normal findings. She had mild pain with eye movement but no other symptoms of demyelinating disease. Despite the progressive loss of vision, “optic neuritis” was diagnosed. No corticosteroids were administered at that time or subsequently. Vision in the left eye progressively deteriorated to faint light perception in May 1994. The patient came to Mayo Clinic Rochester in October 1994 because of subjective visual loss in the right eye. Visual acuity was 20/20 in the right eye, with no light perception in the left eye. The left pupil was amaurotic. The results of color vision testing and automated perimetry of the right eye were normal. No ptosis or proptosis was noted. A sensory exotropia of the left eye was present, but ocular versions were intact. Results of the anterior segment examination were normal, as was intraocular pressure. The left optic disk was pale and atrophie, with no optochoroidal collaterals (optociliary shunt vessels). The right fundus was normal. A repeated MRI scan with use of gadolinium and fat suppression revealed an intensely enhanced 8-mm mass in the superomedial apex of the left orbit, with intracranial extension. Tram-tracking of the optic nerve was evident (Fig. 1). No lesions were noted in the periventricular white matter. Optic nerve sheath meningioma was the presumptive diagnosis. Because of the patient's age and history of quickly progressive visual loss, an “aggressive” meningioma was a concern. We thought that surgical treatment would preclude further intracranial extension of the lesion and establish a tissue diagnosis in the already blind eye. A left frontal crani-otomy and superior orbitotomy approach was used to excise the optic nerve. The apical lesion was tethered to all the surrounding tissues, with a medial exophytic component. The small fibers of extraocular muscle noted in this exophytic component suggested that the tumor had invaded muscle. Pathologic examination revealed noncaseating granulo-mas with giant cells involving the dura mater, extraocular muscle,1Stannard K Spalton DJ Sarcoidosis with infiltration of the external ocular muscles.Br J Ophthalmol. 1985; 69: 562-566Crossref PubMed Scopus (26) Google Scholar and optic nerve (Fig. 2). The results of acid-fast and fungal stains were normal. No foreign bodies were evident. Subsequent cultures and stains for tuberculosis and fungi were negative. A repeated ocular examination showed no evidence of ocular sarcoid. Results of serologie testing for syphilis were normal, as were findings on examination of the cerebrospinal fluid. No oligoclonal banding was seen. Review of MRI findings suggested no lacrimal gland involvement. Results of the tuberculin skin test, chest roentgenogra-phy, and pulmonary function tests were unremarkable. The diffusion capacity of carbon monoxide was normal. The serum levels were as follows: angiotensin-converting enzyme (ACE), 6.1 U/L (normal, 6.1 to 21.1); calcium, 9.8 mg/dL (normal, 8.9 to 10.1); and albumin, 4.5 g/dL (normal, 3.5 to 5.0). No cutaneous lesions were evident. Approximately 5 to 10% of all patients with sarcoidosis have involvement of the central nervous system.2Delaney P Neurologic manifestations in sarcoidosis: review of the literature, with a report of 23 cases.Ann Intern Med. 1977; 87: 336-345Crossref PubMed Scopus (549) Google Scholar, 3Crystal RG Sarcoidosis.in: Wilson JD Braunwald E Isselbacher KJ Petersdorf RG Martin JB Fauci AS Harrison's Principles of Internal Medicine. 12th ed. McGraw-Hill, New York1991: 1463-1469Google Scholar The most frequently involved cranial nerve in sarcoidosis is the facial nerve,4Obenauf CD Shaw HE Sydnor CF Klintworth GK Sarcoidosis and its ophthalmic manifestations.Am J Ophthalmol. 1978; 86: 648-655Abstract Full Text PDF PubMed Scopus (356) Google Scholar followed by the optic nerve.5Beck AD Newman NJ Grossniklaus HE Galetta SL Kramer TR Optic nerve enlargement and chronic visual loss.Surv Ophthalmol. 1994; 38: 555-566Abstract Full Text PDF PubMed Scopus (25) Google Scholar In about 1 to 5% of patients, sarcoidosis affects the optic nerve in some manner.6Engelken JD Yuh WT Carter KD Nerad JA Optic nerve sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1992; 13: 228-230PubMed Google Scholar The optic chiasm is involved more frequently than is the intraorbital optic nerve.7Dubois PJ Beardsley T Klintworth G Sydnor C Cook W Osborne D et al.Computed tomography of sarcoidosis of the optic nerve.Neuroradiology. 1983; 24: 179-182PubMed Google Scholar Sarcoid manifesting as an isolated tumor of the optic nerve is uncommon, and to our knowledge, only 17 other cases with results of optic nerve biopsies have been published in the English-language literature during the past 3 decades (hereafter, these 17 patients and our patient are referred to as the “review series”) (Table 1).5Beck AD Newman NJ Grossniklaus HE Galetta SL Kramer TR Optic nerve enlargement and chronic visual loss.Surv Ophthalmol. 1994; 38: 555-566Abstract Full Text PDF PubMed Scopus (25) Google Scholar, 6Engelken JD Yuh WT Carter KD Nerad JA Optic nerve sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1992; 13: 228-230PubMed Google Scholar, 8Statten R Blodi FC Hanigan J Sarcoidosis of the optic nerve.Arch Ophthalmol. 1964; 71: 834-836Crossref PubMed Scopus (33) Google Scholar, 9Anderson WB Parker JJ Sondheimer FK Optic foramen enlargement caused by sarcoid granuloma.Radiology. 1966; 86: 319-322PubMed Google Scholar, 10Frisén L Lindgren S MacGregor BJL Stattin S Sarcoid-like disorder of the intracranial optic nerve: clinicopathological report of two cases.J Neurol Neurosurg Psychiatry. 1977; 40: 702-707Crossref PubMed Scopus (25) Google Scholar, 11Papo I Beltrami CA Salvolini U Caruselli G Sarcoidosis simulating a glioma of the optic nerve.Surg Neurol. 1977; 8: 353-355PubMed Google Scholar, 12Krohel GB Charles H Smith RS Granulomatous optic neuropathy.Arch Ophthalmol. 1981; 99: 1053-1055Crossref PubMed Scopus (18) Google Scholar, 13Gudeman SK Selhorst JB Susac JO Waybright EA Sarcoid optic neuropathy.Neurology. 1982; 32: 597-603Crossref PubMed Google Scholar, 14Lustgarten JS Mindel JS Yablonski ME Friedman AH An unusual presentation of isolated optic nerve sarcoidosis.J Clin Neuroophthalmol. 1983; 3: 13-18PubMed Google Scholar, 15Beardsley TL Brown SV Sydnor CF Grimson BS Klintworth GK Eleven cases of sarcoidosis of the optic nerve.Am J Ophthalmol. 1984; 97: 62-77Abstract Full Text PDF PubMed Scopus (86) Google Scholar, 16Graham EM Ellis CJ Sanders MD McDonald WI Optic neuropathy in sarcoidosis.J Neurol Neurosurg Psychiatry. 1986; 49: 756-763Crossref PubMed Scopus (49) Google Scholar, 17Jordan DR Anderson RL Nerad JA Patrinely JR Scrafford DB Optic nerve involvement as the initial manifestation of sarcoidosis.Can J Ophthalmol. 1988; 23: 232-237PubMed Google Scholar, 18Carmody RF Mafee MF Goodwin JA Small K Haery C Orbital and optic pathway sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1994; 15: 775-783PubMed Google ScholarTable 1Summary of 18 Cases of Biopsy-Proven Sarcoid Manifesting as an Isolated Optic Nerve Tumor*ACE = angiotensin-converfing enzyme; CF = count fingers (vision); CN = cranial nerve; CRVO = central retinal vein occlusion; CSF = cerebrospinal fluid; CT = computed tomogram; Gl = glioma; HM = hand motion; MG = meningioma; NLP = no light perception; NVG = neovascular glaucoma; OD = oculus dexter (right eye); OS = oculus sinister (left eye); SAR = sarcoidosis; SOF = superior orbital fissure.CaseReferencePreoperative diagnosisAge (yr) and sex (race)Visual acuity (side), time courseOcular findingsSystemic sarcoidosis and other features1Statton et al,8Statten R Blodi FC Hanigan J Sarcoidosis of the optic nerve.Arch Ophthalmol. 1964; 71: 834-836Crossref PubMed Scopus (33) Google Scholar 1964Gl37 F (white)NLP (OS), 4 moDisk edema, proptosis (OS), exotropia (OS), optochoroidal collateralsNot initially; hilar adenopathy 3 yr after diagnosis; enlarged left optic foramen2Anderson et al,9Anderson WB Parker JJ Sondheimer FK Optic foramen enlargement caused by sarcoid granuloma.Radiology. 1966; 86: 319-322PubMed Google Scholar1966MG/G118F (black)20/200 (OD), 5 moOptic atrophy, proptosis (OD)Not found; enlarged right optic foramen; CSF, normal3Frisénet al,'° 1977MG70 M (?)20/400 (OD), >2moCN V (ophthalmic) deficits, Homer's syndrome (OD), eye painNot found (initial complaint, orbital pain); hyperostosis of ala minor; angiogram, “tumor blush” of SOF; no response to oral corticosteroids; CSF, increased protein and cells4Frisén et al,10Frisén L Lindgren S MacGregor BJL Stattin S Sarcoid-like disorder of the intracranial optic nerve: clinicopathological report of two cases.J Neurol Neurosurg Psychiatry. 1977; 40: 702-707Crossref PubMed Scopus (25) Google Scholar 1977MG45 M (?)CF (OS), 3 moDisk edema, sudden loss of vision, eye painNot found (abnormal phalanx on x-ray film); enlarged left optic foramen; CSF, normal5Papoet al,11Papo I Beltrami CA Salvolini U Caruselli G Sarcoidosis simulating a glioma of the optic nerve.Surg Neurol. 1977; 8: 353-355PubMed Google Scholar 1977Gl53 F (?)“Blind Decreased” (OS) (OS), 3.5 moDisk edema, proptosis (OS), eye painNot found; no response to corticosteroids; enlarged left optic foramen6Krohel et al,12Krohel GB Charles H Smith RS Granulomatous optic neuropathy.Arch Ophthalmol. 1981; 99: 1053-1055Crossref PubMed Scopus (18) Google Scholar 1981SAR24 F (?)HM (OS), 8 moDisk edema, optochoroidal collaterals, periphlebitis, vitreous “snowballs”Not found; mild improvement with corticosteroids preoperatively; ACE, 31 IU/L (normal, 12-30); visual acuity improved to 20/400 (OS) after biopsy and corticosteroid therapy7Gudeman et al,13Gudeman SK Selhorst JB Susac JO Waybright EA Sarcoid optic neuropathy.Neurology. 1982; 32: 597-603Crossref PubMed Google Scholar 1982MG23 F (black)NLP (OS), 11 moDisk edema, optochoroidal collateralsNot found; hyperostosis of left anterior clinoid; abnormal results of pulmonary function tests; corticosteroid-responsive improvement in visual acuity8Gudeman et al,13Gudeman SK Selhorst JB Susac JO Waybright EA Sarcoid optic neuropathy.Neurology. 1982; 32: 597-603Crossref PubMed Google Scholar 1982MG47 F (black)20/400 (OD), 1 moNormal fundi, bilateral tonic pupilsNot found; hyperostosis of planum sphenoidale; angiogram and CT suggested MG; spontaneous recovery of vision to acuity of 20/40 (OD) before biopsy; postoperative visual acuity, 20/20 (OD) with corticosteroid therapy9Lustgarten et al,14Lustgarten JS Mindel JS Yablonski ME Friedman AH An unusual presentation of isolated optic nerve sarcoidosis.J Clin Neuroophthalmol. 1983; 3: 13-18PubMed Google Scholar 1983MG/G132 F (black)NLP (OS), 3 moDisk edema, vitritis, prior “diabetic” CN VI palsy, mild improvement in visual acuity after corticosteroid therapyNot found; no response to oral corticosteroids (60 mg); optic canal enlarged on CT; ACE, normal; CSF, normal10Beardsley et al,15Beardsley TL Brown SV Sydnor CF Grimson BS Klintworth GK Eleven cases of sarcoidosis of the optic nerve.Am J Ophthalmol. 1984; 97: 62-77Abstract Full Text PDF PubMed Scopus (86) Google Scholar 1984MG/G145 F (?)HM (OD) and 20/20 (OS), monthsBilateral disk pallor, visual acuity initially improved after corticosteroid therapyNot found; enlarged right optic foramen11Beardsley étal,15Beardsley TL Brown SV Sydnor CF Grimson BS Klintworth GK Eleven cases of sarcoidosis of the optic nerve.Am J Ophthalmol. 1984; 97: 62-77Abstract Full Text PDF PubMed Scopus (86) Google Scholar 1984MG36 F (?)HM (OS), 12 moDisk edema, retinal exudatesNot found; enlarged left optic foramen12Graham et al,16Graham EM Ellis CJ Sanders MD McDonald WI Optic neuropathy in sarcoidosis.J Neurol Neurosurg Psychiatry. 1986; 49: 756-763Crossref PubMed Scopus (49) Google Scholar 1986?43 F (black)CF (OD), 6 wkOptic atrophy (OD), aneurysmal dilatation of disk vessels, proptosis (OD) 9 yr after initial visual lossNot found13Jordan et al,17Jordan DR Anderson RL Nerad JA Patrinely JR Scrafford DB Optic nerve involvement as the initial manifestation of sarcoidosis.Can J Ophthalmol. 1988; 23: 232-237PubMed Google Scholar 1988MG26 F (†One patient was black and one was white (Jordan DR. Personal communication).)NLP (OD), 4 moDisk pallor, optochoroidal collaterals, normal visual acuity, field defect (OS)Not found; ACE, normal14Jordan et al,'7Dubois PJ Beardsley T Klintworth G Sydnor C Cook W Osborne D et al.Computed tomography of sarcoidosis of the optic nerve.Neuroradiology. 1983; 24: 179-182PubMed Google Scholar 1988MG44 F (†One patient was black and one was white (Jordan DR. Personal communication).)CF 1 ft (OD),5 moDisk pallor, right exotropiaNot initially; hilar adenopathy 5 mo after diagnosis; episode of loss of visual acuity 6 yr earlier; ACE, normal15Engelken et al,6Engelken JD Yuh WT Carter KD Nerad JA Optic nerve sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1992; 13: 228-230PubMed Google Scholar1992?52 F (white)NLP (OD), 3 wkNot found; CRVO with NVG after biopsy16Beck et al,5Beck AD Newman NJ Grossniklaus HE Galetta SL Kramer TR Optic nerve enlargement and chronic visual loss.Surv Ophthalmol. 1994; 38: 555-566Abstract Full Text PDF PubMed Scopus (25) Google Scholar 1994SAR56 F (black)NLP (OS) and 20/25 (OD) (OS >2 yr)Optic atrophy (OS), pallor (OD), previous anterior uveitis, visual field loss (OD)Not found; diabetes mellitus; ACE, 57 IU/mL (normal, <52); vision improved (OD) after corticosteroid therapy17Carmody et al,18Carmody RF Mafee MF Goodwin JA Small K Haery C Orbital and optic pathway sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1994; 15: 775-783PubMed Google Scholar 1994MG47 M (white)“Decreased” (OS), ? timeDisk edema (OS), eye painNot noted18Current caseMG22 F (white)NLP (OS), 23 moNo ocular inflammation, pain on eye movementNot found; ACE, 6.4 U/L (normal, 6.1-21.1); CSF, normal* ACE = angiotensin-converfing enzyme; CF = count fingers (vision); CN = cranial nerve; CRVO = central retinal vein occlusion; CSF = cerebrospinal fluid; CT = computed tomogram; Gl = glioma; HM = hand motion; MG = meningioma; NLP = no light perception; NVG = neovascular glaucoma; OD = oculus dexter (right eye); OS = oculus sinister (left eye); SAR = sarcoidosis; SOF = superior orbital fissure.† One patient was black and one was white (Jordan DR. Personal communication). Open table in a new tab As with our patient, 13 of these 17 patients preoperatively were thought to have an optic nerve sheath meningioma or optic nerve glioma. On initial postoperative testing, none of the patients had systemic sarcoidosis. In the series by Gudeman and associates,13Gudeman SK Selhorst JB Susac JO Waybright EA Sarcoid optic neuropathy.Neurology. 1982; 32: 597-603Crossref PubMed Google Scholar one patient had abnormal findings on pulmonary function testing and diffuse parenchymal changes without hilar adenopathy on chest radiography but was not considered to have systemic sarcoidosis. In two patients with isolated optic nerve sarcoid, hilar adenopathy was detected on follow-up evaluation 5 months17Jordan DR Anderson RL Nerad JA Patrinely JR Scrafford DB Optic nerve involvement as the initial manifestation of sarcoidosis.Can J Ophthalmol. 1988; 23: 232-237PubMed Google Scholar and 3 years8Statten R Blodi FC Hanigan J Sarcoidosis of the optic nerve.Arch Ophthalmol. 1964; 71: 834-836Crossref PubMed Scopus (33) Google Scholar after optic nerve biopsy. Long-term follow-up results are available for only a few of the other patients with optic nerve sarcoid (Table 1). Systemic sarcoidosis was not noted at 1-year follow-up assessment of the patient described by Anderson and colleagues.9Anderson WB Parker JJ Sondheimer FK Optic foramen enlargement caused by sarcoid granuloma.Radiology. 1966; 86: 319-322PubMed Google Scholar According to Gudeman and co-workers,13Gudeman SK Selhorst JB Susac JO Waybright EA Sarcoid optic neuropathy.Neurology. 1982; 32: 597-603Crossref PubMed Google Scholar neither of Frisén's10Frisén L Lindgren S MacGregor BJL Stattin S Sarcoid-like disorder of the intracranial optic nerve: clinicopathological report of two cases.J Neurol Neurosurg Psychiatry. 1977; 40: 702-707Crossref PubMed Scopus (25) Google Scholar patients had evidence of systemic sarcoidosis at 7-year follow-up. In contrast to our patient who had no evidence of systemic sarcoidosis at the time of the initial diagnosis, 14 of the 15 patients with orbital sarcoid described by Collison and associates19Collison JM Miller NR Green WR Involvement of orbital tissues by sarcoid.Am J Ophthalmol. 1986; 102: 302-307Abstract Full Text PDF PubMed Scopus (69) Google Scholar had systemic involvement at the time of the initial examination. Neurosarcoidosis, including cranial nerve sarcoidosis, can manifest early during the course of sarcoidosis without a history or evidence of systemic involvement.2Delaney P Neurologic manifestations in sarcoidosis: review of the literature, with a report of 23 cases.Ann Intern Med. 1977; 87: 336-345Crossref PubMed Scopus (549) Google Scholar, 20Scott TF Neurosarcoidosis: progress and clinical aspects.Neurology. 1993; 43: 8-12Crossref PubMed Google Scholar In addition, patients with sarcoidosis of the central nervous system may have no clinically detectable systemic sarcoidosis despite pathologic involvement of other organs at autopsy.21Waxman JS Sher JH The spectrum of central nervous system sarcoidosis: a clinical and pathologic study.Mt Sinai J Med. 1979; 46: 309-317PubMed Google Scholar Slowly progressive loss of vision in a female patient, optic nerve atrophy or swelling, and optochoroidal collaterals are characteristic of optic nerve sheath meningioma. These features, however, were also evident in some of the patients in the review series. Although a sex predilection for systemic sarcoidosis has been questioned by some investigators,22DeRemee RA Sarcoidosis.Mayo Clin Proc. 1995; 70: 177-181Abstract Full Text Full Text PDF PubMed Scopus (65) Google Scholar 15 of the 18 patients (83%) in our review series were women. Of 89 patients with optic nerve sheath meningioma, 63 (71%) were female.23Kennerdell JS Maroon JC Maltón M Warren FA The management of optic nerve sheath meningiomas.Am J Ophthalmol. 1988; 106: 450-457Abstract Full Text PDF PubMed Scopus (114) Google Scholar, 24Wright JE McNab AA McDonald WI Primary optic nerve sheath meningioma.Br J Ophthalmol. 1989; 73: 960-966Crossref PubMed Scopus (84) Google Scholar Optochoroidal collaterals are evident in optic nerve sheath meningioma and many other ocular disorders. Optochoroidal collaterals are also well described in sarcoid optic disk edema25Mansour AM Sarcoid optic disc edema and optociliary shunts.J Clin Neuroophthalmol. 1986; 6: 47-52PubMed Google Scholar and were noted in at least three patients with optic nerve sarcoid (cases 6, 7, and 13 in Table 1). Age is not a distinguishing feature between optic nerve sarcoid and optic nerve sheath meningioma. The median age at initial assessment of patients with optic nerve sarcoid was 43.5 years. In the series by Wright and colleagues,24Wright JE McNab AA McDonald WI Primary optic nerve sheath meningioma.Br J Ophthalmol. 1989; 73: 960-966Crossref PubMed Scopus (84) Google Scholar the median age at the onset of symptoms in patients with optic nerve sheath meningioma was 40 years. Black race; previous facial nerve palsy, intraocular inflammation, or venous sheathing; and improvement after corticosteroid treatment are suggestive but not diagnostic of optic nerve sarcoidosis in patients without known systemic sarcoidosis. Of the 12 patients in the review series whose race was reported, 7 were black. None of the patients in the review series had a previous facial nerve palsy. At least 3 of the 18 patients in the review series had previous or coexisting uveitis with the optic nerve tumor. Improvement in vision after corticosteroid therapy is consistent with optic nerve sarcoid (cases 6, 7, 8, and 16) but not pathognomonic for the condition. Three patients (cases 3, 5, and 9) had minimal or no response to corticosteroid therapy. In patients with meningiomas or other mass lesions that affect the anterior visual pathways, impaired vision is occasionally ameliorated after corticosteroid treatment.13Gudeman SK Selhorst JB Susac JO Waybright EA Sarcoid optic neuropathy.Neurology. 1982; 32: 597-603Crossref PubMed Google Scholar In the 18 patients in the review series, visual acuity was hand motions or worse in 11 (61%), with visual loss progressing to this stage during a median period of 4 months. Although one patient (case 8) had spontaneous recovery of visual acuity to 20/40, optic nerve biopsy was still performed. Spontaneous recovery of vision (cases 8 and 14) is more characteristic of optic nerve sarcoid than optic nerve sheath meningioma. Loss of vision with spontaneous recovery of vision in a young female patient with radiographie evidence of optic nerve swelling is also consistent with optic neuritis. In light of the fact that both conditions involve inflammation near the anulus of Zinn, pain would be expected with eye movement, as occurred in our patient. Diagnosis is complicated further because on MRI, periventricular white matter lesions that are indistinguishable from those of multiple sclerosis have been described in patients with orbital and optic pathway sarcoidosis.18Carmody RF Mafee MF Goodwin JA Small K Haery C Orbital and optic pathway sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1994; 15: 775-783PubMed Google Scholar Neurosarcoidosis should be included in the differential diagnosis of isolated periventricular lesions in patients younger than age 50 years.26Smith AS Meisler DM Weinstein MA Tomsak RL Hanson MR Rudick RA et al.High-signal periventricular lesions in patients with sarcoidosis: neurosarcoidosis or multiple sclerosis?.AJR Am J Roentgenol. 1989; 153: 147-152Crossref PubMed Scopus (32) Google Scholar About 80% of patients with active systemic sarcoidosis have an increased serum level of ACE if they are receiving no corticosteroid therapy.27DeRemee RA Rohrbach MS Serum angiotensin-converting enzyme activity in evaluating the clinical course of sarcoidosis.Ann Intern Med. 1980; 92: 361-365Crossref PubMed Scopus (149) Google Scholar Therefore, a substantial number of patients with clinically active sarcoidosis may have normal serum levels of ACE.28DeRemee RA Rohrbach MS Normal serum angiotensin converting enzyme activity in patients with newly diagnosed sarcoidosis.Chest. 1984; 85: 45-48Crossref PubMed Scopus (18) Google Scholar The serum level of ACE was recorded in six patients in the review series, and only two (cases 6 and 16) had an abnormal value (a minimally increased level). Patients with neurosarcoidosis can have numerous abnormalities of the cerebrospinal fluid. Typically, the concentration of protein is increased, and a mild lymphocytic pleocytosis is noted. Lumbar puncture was performed in 6 of the 18 patients in the review series, and only 1 (case 3) had abnormal findings (an increase in both protein concentration and number of cells). An increased concentration of ACE in the cerebrospinal fluid may suggest neurosarcoidosis,20Scott TF Neurosarcoidosis: progress and clinical aspects.Neurology. 1993; 43: 8-12Crossref PubMed Google Scholar but testing for this is no longer performed in our laboratory. On CT with contrast enhancement, both optic nerve sheath meningioma and optic nerve sarcoidosis may appear as thickening of the optic nerve. In patients with optic nerve sheath meningioma, the optic nerve tends to be diffusely enlarged, with irregular expansion along the optic nerve. Diffuse enlargement of the optic nerve with apical expansion is the most frequent pattern. Enlargement of the optic foramen was noted in many of the patients in the review series. Calcification within the optic nerve is common with optic nerve sheath meningioma. A central linear lucency extending the length of the optic nerve associated with tram-tracking is also a typical finding in this condition. Additionally, tram-tracking was seen in many of the patients with sarcoid in the review series. Thickened contrast-enhanced optic nerves, basilar meningeal infiltration, a densely enhanced and thickened falx cerebri, and multiple enhanced solid nodules detected on CT are suggestive of but not pathognomonic for neurosarcoidosis. Diffuse uptake of contrast agent in the basal cisterns can be noted in carcinomatous meningitis as well as in fungal disease, tuberculosis, and bacterial meningitis. Multiple enhanced nodules may be demonstrated in fungal and bacterial infections, although they may have a ring configuration.29Post MJD Quencer RM Tabei SZ CT demonstration of sarcoidosis of the optic nerve, frontal lobes, and falx cerebri: case report and literature review.AJNR Am J Neuroradiol. 1982; 3: 523-526PubMed Google Scholar Fat-suppressed, contrast-enhanced MRI is sensitive for the detection of optic nerve lesions such as optic nerve sarcoid, especially at the orbital apex.6Engelken JD Yuh WT Carter KD Nerad JA Optic nerve sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1992; 13: 228-230PubMed Google Scholar Nonetheless, the MRI findings in optic nerve sarcoid may closely resemble those of diseases such as optic nerve sheath meningioma and chronic inflammatory processes, in both anatomic site and signal characteristics.18Carmody RF Mafee MF Goodwin JA Small K Haery C Orbital and optic pathway sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1994; 15: 775-783PubMed Google Scholar Prominent enhancement of the optic nerve or nerve sheath with gadolinium was noted in 4 of 15 patients with orbital and optic pathway sarcoidosis.18Carmody RF Mafee MF Goodwin JA Small K Haery C Orbital and optic pathway sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1994; 15: 775-783PubMed Google Scholar The enhancement was similar to that seen in optic nerve sheath meningioma, orbital pseudotumor, optic neuritis, and lepto-meningeal spread of tumor. On MRI, optic nerve sheath meningioma is typically isointense to cortical gray matter on T1Stannard K Spalton DJ Sarcoidosis with infiltration of the external ocular muscles.Br J Ophthalmol. 1985; 69: 562-566Crossref PubMed Scopus (26) Google Scholar-weighted images and remains isointense on proton density studies. Patients with optic nerve sarcoid who have only peri-neural enhancement may have a better prognosis for visual function than those who have neural enhancement.18Carmody RF Mafee MF Goodwin JA Small K Haery C Orbital and optic pathway sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1994; 15: 775-783PubMed Google Scholar Usually, the CT or MRI appearance of optic nerve sarcoid improves after corticosteroid treatment. Slight enlargement and enhancement of the optic nerves may persist, however, despite clinical improvement.5Beck AD Newman NJ Grossniklaus HE Galetta SL Kramer TR Optic nerve enlargement and chronic visual loss.Surv Ophthalmol. 1994; 38: 555-566Abstract Full Text PDF PubMed Scopus (25) Google Scholar, 6Engelken JD Yuh WT Carter KD Nerad JA Optic nerve sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1992; 13: 228-230PubMed Google Scholar Distinguishing optic nerve sheath meningioma from optic nerve sarcoid is important because (1) corticosteroids may be effective in treating the latter, (2) optic nerve biopsy may result in blindness or ophthalmoplegia, (3) pseudo-meningiomas may be irradiated inadvertently without biopsy (in a series of 38 patients, meningioma was diagnosed in 19 on the basis of clinical findings and radiologie appearance alone23Kennerdell JS Maroon JC Maltón M Warren FA The management of optic nerve sheath meningiomas.Am J Ophthalmol. 1988; 106: 450-457Abstract Full Text PDF PubMed Scopus (114) Google Scholar), and (4) surgical excision has been recommended for patients with optic nerve sheath meningioma who are younger than age 30 years, even if vision is relatively good.24Wright JE McNab AA McDonald WI Primary optic nerve sheath meningioma.Br J Ophthalmol. 1989; 73: 960-966Crossref PubMed Scopus (84) Google Scholar Despite the advent of MRI, sarcoidosis remains the “great mimicker.” In the absence of systemic involvement or uveitis, isolated optic nerve sarcoid is virtually impossible to diagnose without performance of biopsy.18Carmody RF Mafee MF Goodwin JA Small K Haery C Orbital and optic pathway sarcoidosis: MR findings.AJNR Am J Neuroradiol. 1994; 15: 775-783PubMed Google Scholar We do not advocate an extensive work-up for sarcoidosis in a patient with findings typical for optic nerve sheath meningioma unless the patient has a history of systemic sarcoidosis, uveitis, facial nerve palsy, or spontaneous visual recovery or if the patient is black. Optic nerve sheath meningioma is the leading diagnosis for progressive blindness in a woman with unilateral optic disk pallor or edema, optic nerve enlargement, and no other sign of systemic or ocular inflammation. Optic nerve sarcoid should be considered in the differential diagnosis of optic nerve sheath meningioma, but it is a diagnosis of exclusion that necessitates biopsy. Blind con-junctival biopsy and lacrimal gland biopsy, although helpful if findings are abnormal, are usually of limited value.30Karcioglu ZA Brear R Conjunctival biopsy in sarcoidosis.Am J Ophthalmol. 1985; 99: 68-73Abstract Full Text PDF PubMed Scopus (41) Google Scholar, 31Copeland RA The classics: tuberculosis, syphilis and sarcoidosis.Ophthalmol Clin North Am. 1993; 6: 69-80Google Scholar, 32Weinreb RN Tessler H Laboratory diagnosis of ophthalmic sarcoidosis.Surv Ophthalmol. 1984; 28: 653-664Abstract Full Text PDF PubMed Scopus (66) Google Scholar Gudeman and associates13Gudeman SK Selhorst JB Susac JO Waybright EA Sarcoid optic neuropathy.Neurology. 1982; 32: 597-603Crossref PubMed Google Scholar believe that known systemic sarcoidosis with granulomatous involvement of the optic nerve does not necessarily obviate performance of biopsy of the optic nerve. In patients with such features, empiric corticosteroid treatment and thorough clinical follow-up with serial imaging studies to monitor the response to corticosteroid therapy may be helpful.13Gudeman SK Selhorst JB Susac JO Waybright EA Sarcoid optic neuropathy.Neurology. 1982; 32: 597-603Crossref PubMed Google Scholar The management of optic nerve sheath meningioma in patients who do not undergo biopsy is complicated because the lesions of this disease mimic those of optic nerve sarcoid. We acknowledge the helpful assistance of Douglas A. Nichols, M.D., Department of Diagnostic Radiology." @default.
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