Falx Meningeoma Induced Optic Neuropathy—Clinical Manifestation and Differential Diagnosis: A Case Report

doi:10.4236/ojoph.2013.33020

Paper Menu >>

Journal Menu >>

Open Journal of Ophthalmology, 2013, 3, 87-89

http://dx.doi.org/10.4236/ojoph.2013.33020 Published Online August 2013 (http://www.scirp.org/journal/ojoph)

Falx Meningeoma Induced Optic Neuropathy—Clinical

Manifestation and Differential Diagnosis: A Case Report*

Skevas Christos#, Görsch Isabel Carolin, Richard Gisbert, Linke Johannes Stephan

Universitätsklinikum Hamburg-Eppendorf, Klinik und Poliklinik für Augenheilkunde, Hamburg, Germany.

Email: #[email protected]

Received March 25th, 2013; revised April 26th, 2013; accepted June 20th, 2013

which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Purpose: Meningiomas are the most frequently diagnosed neoplasms. Ocular symptoms vary depending on size and

localisation of the tumor, which is capable of compressing critical anatomic structures. Patients and Methods: A

44-year-old immunocompetent male was admitted to our hospital for further investigation of his bilateral optic disc

swelling. A complete ophthalmological examination was performed including visual field assessment (VF), visual

evoked potentials (VEP), fluorescein angiography (FLA) and magnetic resonance imaging (MRI). The patient had ex-

perienced only minor visual disturbances. Serological testing was performed. Results: Serological testing showed posi-

tive IgM and IgGtiters for cytomegalovirus (CMV) and subsequent imaging (MRI) revealed a frontal falxcerebri men-

ingioma. Conclusions: We present a case of falx meningioma with overlapping serologically active CMV in an other-

wise healthy and immunocompetent individual. Detailed differential diagnostic workup in immunocompetent individu-

als presenting with clinically unremarkable bilateral disc swelling is recommended.

Keywords: Falx Meningeoma; Optic Neuropathy; Cytomegalovirus (CMV); Visual Disturbances; Fluorescein

Angiography (FLA); Magnetic Resonance Imaging (MRI); Visual Evoked Potentials (VEP)

1. Introduction

Meningiomas are the most frequently diagnosed neo-

plasms accounting for 33.8% of all brain and central

nervous system tumors reported in the USA between

2002 and 2006 [1]. Cushing defined falx meningioma as

a tumor arising from the falxcerebri and concealed by the

overlying cortex [2].

Ocular symptoms vary depending on size and localisa-

tion of the tumor, which is capable of compressing criti-

cal anatomic structures. Visual field abnormalities, optic

atrophy, optic disc edema, diplopia and proptosis can be

presented. Intracranial hypertension is defined as an in-

crease in the intracranial pressure >20 mmHg. Typical

manifestations are headaches, vomiting and visual symp-

toms (transient visual obscurations, loss of visual acuity)

[3]. We report on a 44-year-old immunocompetent male

with bilateral swelling of the optic discs due to falxmen-

ingeoma. The diagnosis was overlapped by positive IgM

and IgGtiters for CMV.

2. Materials and Methods

A 44-year-old male with unremarkable medical history

presented in our clinic with a bilateral optic disc swelling,

which was diagnosed during a routine ophthalmological

examination. Patient was not taking any medication and

denied having any reduced visual acuity, transient visual

disturbances or headaches. A complete ophthalmological

examination was performed including corrected distance

visual acuity (CDVA), visual field (VF) assessment,

fundoscopy, visual evoked potentials (VEP), fluorescein

angiography (FLA), serological examinations and mag-

netic resonance imaging (MRI).

3. Results

Corrected distance visual acuity (CDVA) was 20/20 for

both eyes (R −4.5 sph −0.75 cyl A 83 and L −4.5 sph

−1.25 cyl A 177) but visual field (VF) assessment (Hum-

phrey Visual Field Analyzer, protocol 24-2; Carl Zeiss

Ophthalmic Systems Inc., Dublin, CA) showed bilateral

(R > L) inferior altitudinal field defects. No afferent pu-

pillary defect or colour desaturation was evident. Ante-

rior chamber assessment showed no pathology, and fun-

*Disclosure declaration: There are no proprietary interests and no fi-

nancial support was received. No conflicts of interest regarding this

article exist.

#Corresponding author.

Falx Meningeoma Induced Optic Neuropathy—Clinical Manifestation and Differential Diagnosis: A Case Report

doscopy confirmed a bilateral (R > L) optic disc edema

without any signs of retinal haemorrhages and no cells in

the hyaloidbody. Visual evoked potentials (VEP) were

within normal limits and fluorescein angiography (FLA)

showed a late phase disc staining. Ultrasonography of the

optic nerve sheath, Doppler ultrasonography and color-

doppler imaging (CDI) elicited no abnormalities. Routine

and differential blood counts, C-reactive protein (CRP),

protein C and S deficiency, APC resistance, Factor V

Leiden, anti-cardiolipin antibodies were all within nor-

mal range. Further infectious serologic blood tests (Her-

pes Simplex virus, Varizella Zoster virus, Cytomega-

lovirus, Ebstein-Barr virus, Lues, Toxoplasmosis, Bor-

reliosis, HIV) were performed and revealed positive IgG

and IgMtiters for Cytomegalovirus (CMV) indicative for

an active CMV infection. The ensuing physical, neuro-

logical as well as chest X-ray examinations were unre-

markable with no signs of splenomegaly. A 2-week

course of intravenous ganciclovir (2 × 5 mg pro Kg i.v)

treatment was commenced and additionally a cerebral

imaging (MR) was performed. A 4.0 cm × 4.5 cm × 4.0

cm solitary, well defined, parasagittal frontal meningio-

ma (Figures 1 and 2) originating from falxcerebri was

diagnosed. A surgical resection of the tumor had been

scheduled after consultation with the department of neu-

rosurgery.

4. Discussion

Among the differential diagnosis of bilateral optic disc

edema, intracranial hypertension and infectious papillitis

are most frequently reported. The differentiation can fur-

Figure 1. A 4.0 cm × 4.5 cm × 4.0 cm solitary, well defined,

parasagittal frontal meningioma originating from falxcere-

bri.

Figure 2. A 4.0 cm × 4.5 cm × 4.0 cm solitary, well defined,

parasagittal frontal meningioma originating from falxcere-

bri.

ther be hampered by the absence of neurological symp-

toms. Classification of meningiomas according to Cush-

ing and Eisenhardt was based on surgical findings of a

dural or bony site of origin and depending on their origin

in the falx, they can be divided into anterior, middle or

posterior types [2]. Optic disc edema which is fortui-

tously discovered in patients without complaints can in-

sidiously lead to visual handicap, due to chronic optic

disc edema and optic atrophy. One feature of optic disc

edema from raised ICH is that normal vision usually

continues without interference for many weeks or even

months. Hayreh speculated that the cause of the laminar

transport block is mechanical and the visual loss in

chronic optic disc edema results from ischaemia secon-

dary to optic disc swelling [4].

Cytomegalovirus (CMV) is a neurotropic virus with a

predilection to the retina and the central nervous system.

The natural course of CMV papillitis leads to blindness.

Grossniklaus et al. detected CMV inflammation cell in-

filtrated in the retrolaminar portion of the optic nerve.

The pathological examination shows signs of necrosis [5].

CMV retinitis usually affects individuals that have been

already diagnosed with acquired immunodeficiency syn-

drome (AIDS) (stages of advanced immunsupression-CD

4 + counts of < 50 cells/mm3) [6]. The therapy of CMV

Falx Meningeoma Induced Optic Neuropathy—Clinical Manifestation and Differential Diagnosis: A Case Report

retinitis/optic neuritis consists of intravenous ganciclovir

or intravenous foscarnet, but their use in combination is

also presented. The combination is more effective in

controlling progression of CMV retinitis in relapsed pa-

tients than monotherapy alone [7,8]. Besides patients

with immune deficiency (AIDS), there are reports of

CMV induced optic neuropathy in healthy immunocom-

petent individuals [9,10].

We present to the best of our knowledge the first case

of falx meningioma with overlapping serologically active

CMV in an otherwise healthy and immunocompetent

individual. A step by step approach for detailed differen-

tial diagnostic workup in immunocompetent individuals

presenting with clinically unremarkable bilateral disc

swelling is recommended. Treatment should involve a

harmonious cooperative effort between the ophthalmolo-

gist and physicians of other specialities such as microbi-

ology, neurology and neurosurgery.

REFERENCES

[1] CBTRUS, “Statistical Report: Primary Brain and Central

Nervous System Tumors Diagnosed in Eighteen States in

2002-2006,” Central Brain Tumor Registry of the United

States, Hinsdale, 2009-2010.

[2] H. Cushing and L. Eisenhardt, “Meningiomas. Their Clas-

sification, Regional Behavior, Life History and Surgical

End Results,” Bulletin of the Medical Library Association,

Vol. 27, No. 2, 1938, pp. 224-249.

[3] D. I. Friedman and D. M. Jacobson, “Diagnostic Criteria

for Idiopathic Intracranial Hypertension,” Neurology, Vol.

59, No. 10, 2002, pp. 1492-1495.

[4] S. S. Hayreh, “Optic Disc Edema in Raised Intracranial

Pressure. VI. Associated Visual Disturbances and Their

Pathogenesis,” Archives of Ophthalmology, Vol. 95, No.

9, 1977, pp. 1566-1579.

doi:10.1001/archopht.1977.04450090088007

[5] H. E. Grossniklaus, K. E. Frank and R. L. Tomsak, “Cy-

tomegalovirus Retinitis and Optic Neuritis in Acquired

Immune Deficiency Syndrome: Report of a Case,” Oph-

thalmology, Vol. 94, No. 12, 1987, pp. 1601-1604.

[6] J. D. Roarty, E. J. Fisher and J. J. Nussbaum, “Long-Term

Visual Morbidity of Cytomegalovirus Retinitis in Patients

with Acquired Immune Deficiency Syndrome,” Ophthal-

mology, Vol. 100, No. 11, 1993, pp. 1685-1688.

[7] D. N. Friedberg, “Cytomegalovirus Retinitis: Diagnosis

and Status of Systemic Therapy,” Journal of Acquired

Immune Deficiency Syndromes & Human Retrovirology,

Vol. 14, No. 1, 1997, pp. S1-S6.

doi:10.1097/00042560-199700001-00002

[8] A. M. Mansor and H. K. Li, “Cytomegalovirus Optic

Neuritis: Characteristics, Therapy and Survival,” Oph-

thalmologica, Vol. 209, No. 5, 1995, pp. 260-266.

doi:10.1159/000310627

[9] E. Baglivo, P. M. Leuenberger and K. H. Krause, “Pre-

sumed Bilateral Cytomegalovirus-Induced Optic Neu-

ropathy in an Immunocompetent Person: A Case Report,”

Journal of Neuro-Ophthalmology, Vol. 16, No. 1, 1996,

pp. 14-17. doi:10.1097/00041327-199603000-00004

[10] S. R. De Silva, G. Chohan, D. Jones and M. Hu, “Cy-

tomegalovirus Papillitis in an Immunocompetent Patient,”

Journal of Neuro-Ophthalmology, Vol. 28, No. 2, 2008,

pp. 126-127. doi:10.1097/WNO.0b013e3181782fed