Sequential NAION presenting as pseudo Foster Kennedy syndrome

Accepted Manuscript Sequential NAION presenting as pseudo Foster Kennedy syndrome

Anuja Patil, Aastha Takkar, Manoj Goyal, Ramandeep Singh, Vivek Lal PII: DOI: Reference:

S0022-510X(17)30097-7 doi: 10.1016/j.jns.2017.02.002 JNS 15137

To appear in:

Journal of the Neurological Sciences

Received date: Revised date: Accepted date:

25 November 2016 30 January 2017 1 February 2017

Please cite this article as: Anuja Patil, Aastha Takkar, Manoj Goyal, Ramandeep Singh, Vivek Lal , Sequential NAION presenting as pseudo Foster Kennedy syndrome. The address for the corresponding author was captured as affiliation for all authors. Please check if appropriate. Jns(2017), doi: 10.1016/j.jns.2017.02.002

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ACCEPTED MANUSCRIPT Sequential NAION presenting as Pseudo Foster Kennedy syndrome Anuja Patil*, Aastha Takkar**, Manoj Goyal**, Ramandeep Singh***, Vivek Lal****

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*Senior resident, Department of neurology, PGIMER; ** Assistant

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Professor, Department of Neurology, PGIMER; *** Additional

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Professor, Department of Ophthalmology, PGIMER; ****Professor

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and Head of Department of neurology, PGIMER.

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Corresponding author:

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Dr. Vivek Lal

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Professor & Head

Department of Neurology

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INDIA

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PGIMER, Chandigarh-160 012

Tel : 0172-2756691, 2756690 Fax : 0172-274440 Word count: for Abstract: 146. Main text (manuscript): 1035 No. of references: 11

ACCEPTED MANUSCRIPT No. of figures: 1

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No. of tables: 1

ACCEPTED MANUSCRIPT Keywords: Ischemic optic neuropathy, bilateral NAION, Pseudo-Foster Kennedy

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syndrome, optic atrophy

ACCEPTED MANUSCRIPT ABSTRAT Purpose: To review recurrent NAION as a cause for PFK syndrome.

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METHODS:

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In an observational study patients presenting with sudden loss of vision were evaluated. We reviewed patients presenting with disc

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edema on one side and optic atrophy in contralateral eye on fundus

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angiography was assessed.

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examination. Their visual field defects and fundus fluorescein

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RESULTS:

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Of the 7 patients evaluated 4 (57.1%) were females. Mean age at

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presentation was 53.7±11.9years. Mean duration between the two episodes was 12.7 months (range: 2-30). The visual acuity of presenting eye ranged from 6/9 to worse counting fingers close to face. CONCLUSIONS:

ACCEPTED MANUSCRIPT The diagnosis in a PFK presentation is essentially one of exclusion. Patients with NAION are at risk for recurrence in fellow eye, thereby presenting as PFK syndrome. NAION should be considered as a

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differential especially when imaging and other laboratory

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investigations are not suggestive of any compressive lesion.

ACCEPTED MANUSCRIPT Sequential NAION presenting as Pseudo-Foster Kennedy syndrome. 1. Introduction:

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Pseudo-Foster Kennedy (PFK) syndrome refers to a constellation of

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unilateral optic disc edema and contralateral optic atrophy in the

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absence of any compressive optic nerve lesion. Classically it has been

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described in ischemic optic neuropathy particularly Non-arteritic

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anterior ischemic optic neuropathy (NAION) or bilateral sequential optic neuritis and chronic unilateral optic atrophy due to traumatic or

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compressive optic neuropathies. Literature also describes other rare

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conditions with presentation similar to PFK like congenital optic

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nerve hypoplasia [1], pachymeningitis [2] and idiopathic intracranial

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hypertension.

NAION is the most common form of non-glaucomatous optic neuropathy in elderly individuals. The mean age at onset of NAION ranges from 57 to 65 years [3,4]. It presents with sudden painless vision loss associated with disc edema and visual field defects consistent with optic disc involvement on examination. The

ACCEPTED MANUSCRIPT probability of involvement of fellow eye varies among different studies. While Beri et al [5], reported 25% risk of involvement of fellow eye over 3 years, the risk was 17% over 5 years as per Beck et

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al [6] and 14.7% patients in the ischemic optic neuropathy decompression trial ( IONDT) developed sequential involvement of

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fellow eye.

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The subsequent involvement of fellow eye gives the clinical appearance with previously involved pale disc and disc edema in

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presenting eye thereby presenting like Pseudo- Foster Kennedy

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syndrome. AION must be considered as one of the likely causes in

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2. Methods:

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patients presenting with a PFK.

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We analysed patients presenting to adult neurology and ophthalmology OPD over 14 years of age with bilateral sequential NAION from June 2014 to September 2015. Of these patients with disc edema on one side and optic atrophy in contralateral eye on fundus examination were reviewed. A detailed clinical history was obtained.

ACCEPTED MANUSCRIPT Diagnosis of AION based on following criteria [7] : a) Sudden/sequential loss of vision of one or both eyes b) Fundus examination suggestive of disc edema / pallor

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c) Visual fields with specific field defects

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d) FFA – minimal or no filling defect or delay in filling in the optic

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disc and/or peripapillary choroid or choroidal watershed zone.

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e) Exclusion of other causes of vision loss The diagnosis of AION requires that 3 out of first 4 criteria are met

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while the 5th (e) criterion must essentially be fulfilled. Arteritic-

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AION (A-AION) was considered in cases suspected of Giant cell

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arteritis or any other vasculitic etiology while rest of the cases

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(NAION) were evaluated for the presence of risk factors including diabetes, hypertension, coronary artery disease, stroke, smoking, etc. Patients with congenital optic disc anomalies were excluded. Visual acuity was assessed using Snellen’s charts. Automated Perimetry was performed using Humphrey field analyser. Visual field defects were described according to the respective quadrant involved.

ACCEPTED MANUSCRIPT Fundus photography was obtained for all patients at first visit and during follow ups using Visupac digital image archiving system (software release 4.4).

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An MRI brain and angiography of intracranial and neck vessels was

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done in each case to exclude other probable causes for PFK. All

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patients were evaluated for risk factors and vasculitic workup.

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The clinical details of the patients with AION with PFK presentation

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are given in table no.1

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3. Results:

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We evaluated 12 patients with sequential bilateral AION. Of these 7 patients had PFK like presentation. 4 (57.1%) out of these 7 patients

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were females. Mean age at presentation was 53.7±11.9years. Mean

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duration between the two episodes was 12.7 months (range: 2-30). The visual acuity of presenting eye ranged from 6/9 to worse counting fingers close to face. Only two out of the 7 patients had been on antiplatelet agent (aspirin 75mg/day). None of the risk factors were significantly associated with the risk of recurrence. Poor visual acuity

ACCEPTED MANUSCRIPT in the fellow eye was not associated with poorer acuity or severity of visual field defect in the presenting eye.

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4. Discussion:

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The presence of unilateral disc atrophy and contralateral disc edema

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has been classically termed as Foster Kennedy (FK) syndrome after

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the British neurologist Robert Foster Kennedy who described it in

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1911[8]. However the entity was first recognised by William Gowers in 1893 hence also known as Gowers-Paton-Kennedy syndrome. FK

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syndrome typically results from frontal lobe tumours or optic nerve meningiomas leading to ipsilateral optic atrophy. In the absence of

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mass or compressive lesions such presentation is termed Pseudo-

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Foster Kennedy syndrome. Optic atrophy from either inflammatory (ex. optic neuritis), traumatic or inherited (ex. LHON) are frequent causes for PFK. Anterior ischemic optic neuropathy presenting with sequential bilateral involvement leads to similar clinical picture. The history of sudden acute often sectoral visual loss in the fellow eye

ACCEPTED MANUSCRIPT may give the clue and poor visual acuity is not a rule. NAION may present with almost normal visual acuity[9]. NAION is the commonest cause of AION in individuals aged ≥50

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years[3]. The mechanism of NAION is proposed to be multifactorial.

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In addition to “disc at risk” (small optic nerve with small or absent

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physiologic cup), it has been associated with various vascular risk

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factors such as hypertension, diabetes mellitus, hyperlipidemia, obstructive sleep apnea, smoking, migraine, drugs and various

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coagulopathies[10].

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In the Ischemic Optic Neuropathy Decompression Trial (IONDT)

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approximately 15% of patients developed NAION in the fellow eye

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within 5 years[11]. Presumably the other eye is exposed to same risk factors for small vessel disease and has the same structural

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vulnerability i.e. a small cup to disc ratio. In IONDT, risk of NAION in fellow eye related positively with poor baseline visual acuity in the first eye, younger age at onset and presence of diabetes, but not with sex, smoking, or use of aspirin. In younger patients, the risk of fellow eye involvement seems to be 35% within 7 months. Baseline visual

ACCEPTED MANUSCRIPT acuity of 20/200 or worse, diabetes, coronary artery disease (CAD), hypertension, stroke or transient ischemic attacks (TIAs) were weakly associated with occurrence of NAION in the fellow eye. There was no

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association between smoking and risk of recurrence.

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5. Conclusion:

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The diagnosis in a PFK presentation is essentially one of exclusion.

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Though tumorous causes which are invariably considered in all cases,

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can be readily excluded by appropriate imaging techniques, a low threshold of suspicion must be exercised to rule out bilateral

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sequential NAION as one of the cause as has been highlighted by our

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series.

ACCEPTED MANUSCRIPT 6. References:

1. Bansal S, Dabbs T, Long V. Pseudo-Foster Kennedy Syndrome

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due to unilateral optic nerve hypoplasia: a case report. Journal of

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Medical Case Reports. 2008;2:86. doi:10.1186/1752-1947-2-86.

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2. Tamai H, Tamai K, Yuasa H. Pachymeningitis with pseudo-

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Foster Kennedy syndrome. Am J Ophthalmol. 2000

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Oct;130(4):535-7.

3. Hattenhauer, M. G., Leavitt, J. A., Hodge, D. O., et al. Incidence

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of nonarteritic anterior ischemic optic neuropathy. Am. J.

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Ophthalmol. 123, 103–107 (1997).

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4. L N Johnson, A. A. Johnson, L. N. & Arnold, A. C. Incidence of nonarteritic and arteritic anterior ischemic optic

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neuropathy. Population-based study in the state of Missouri and Los Angeles County, California. J. Neuroophthalmol. 14, 38-44. J. Neuro-Ophthalmol. Off. J. North Am. Neuro-Ophthalmol. Soc. 14, 38–44 (1994).

ACCEPTED MANUSCRIPT 5. Beri, M., Klugman, M. R., Kohler J. A. et al. Anterior ischemic optic neuropathy. VII. Incidence of bilaterality and various influencing factors. Ophthalmology 94, 1020–1028 (1987).

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6. Beck, R. W., Servais, G. E. & Hayreh, S. S. Anterior ischemic optic neuropathy. IX. Cup-to-disc ratio and its role in

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pathogenesis. Ophthalmology 94, 1503–1508 (1987). 7. Optic nerve decompression surgery for nonarteritic anterior

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ischemic optic neuropathy (NAION) is not effective and may be

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harmful. The Ischemic Optic Neuropathy Decompression Trial Research Group. JAMA 273, 625–632 (1995).

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8. Kennedy R.F, Thorofare N.J. Retrobulbar neuritis as an exact diagnostic sign of certain tumors and abscesses in the frontal

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368.

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lobe. American Journal of the medical sciences., 1911; 142:355-

9. Hayreh, S. S. & Zimmerman, M. B. Nonarteritic anterior ischemic optic neuropathy: natural history of visual outcome. Ophthalmology 115, 298–305.e2 (2008). 10. Hayreh, S. S. Ischemic optic neuropathy. Int. Ophthalmol. 1, 9– 18 (1978).

ACCEPTED MANUSCRIPT 11. Newman, N. J. et al. The fellow eye in NAION: report from the ischemic optic neuropathy decompression trial follow-up study.

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Am. J. Ophthalmol. 134, 317–328 (2002).

ACCEPTED MANUSCRIPT Figure 1: fundus photographs showing disc edema in the presenting eyes and optic atrophy in the fellow eyes (left two columns), visual field defects(middle

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two columns) and fundus fluorescein angiography findings (right two columns)