Journal of the Neurological Sciences 376 (2017) 49–51

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Journal of the Neurological Sciences

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Clinical Short Communication

Sequential NAION presenting as pseudo Foster Kennedy syndrome

Anuja Patil a, Aastha Takkar a, Manoj Goyal a, Ramandeep Singh b, Vivek Lal a,⁎
a
Department of Neurology, PGIMER, India
b
Department of Ophthalmology, PGIMER, India

a r t i c l e i n f o a b s t r a c t

Article history: Purpose: To review recurrent NAION as a cause for PFK syndrome.
Received 25 November 2016 Methods: In an observational study patients presenting with sudden loss of vision were evaluated. We reviewed
Received in revised form 30 January 2017 patients presenting with disc edema on one side and optic atrophy in contralateral eye on fundus examination.
Accepted 1 February 2017 Their visual field defects and fundus fluorescein angiography was assessed.
Available online 3 February 2017
Results: Of the 7 patients evaluated 4 (57.1%) were females. Mean age at presentation was 53.7 ± 11.9 years.
Mean duration between the two episodes was 12.7 months (range: 2–30). The visual acuity of presenting eye
Keywords:
Ischemic optic neuropathy
ranged from 6/9 to worse counting fingers close to face.
Bilateral NAION Conclusions: The diagnosis in a PFK presentation is essentially one of exclusion. Patients with NAION are at risk for
Pseudo-Foster Kennedy syndrome recurrence in fellow eye, thereby presenting as PFK syndrome. NAION should be considered as a differential
Optic atrophy especially when imaging and other laboratory investigations are not suggestive of any compressive lesion.
© 2017 Elsevier B.V. All rights reserved.

1. Introduction must be considered as one of the likely causes in patients presenting

Pseudo-Foster Kennedy (PFK) syndrome refers to a constellation of
unilateral optic disc edema and contralateral optic atrophy in the
absence of any compressive optic nerve lesion. Classically it has been
described in ischemic optic neuropathy particularly Non-arteritic
anterior ischemic optic neuropathy (NAION) or bilateral sequential
optic neuritis and chronic unilateral optic atrophy due to traumatic or
compressive optic neuropathies. Literature also describes other rare
conditions with presentation similar to PFK like congenital optic nerve
hypoplasia [1], pachymeningitis [2] and idiopathic intracranial
hypertension.
NAION is the most common form of non-glaucomatous optic neu-
ropathy 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 as-
sociated with disc edema and visual field defects consistent with optic
disc involvement on examination. The probability of involvement of fel-
low 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 al. [6] and 14.7% patients in the ischemic
optic neuropathy decompression trial (IONDT) developed sequential
involvement of fellow eye.
The subsequent involvement of fellow eye gives the clinical appear-
ance with previously involved pale disc and disc edema in presenting
eye thereby presenting like Pseudo-Foster Kennedy syndrome. AION
⁎ Corresponding author at: Department of Neurology, PGIMER, Chandigarh 160012,
India.
E-mail address: vivekl44@yahoo.com (V. Lal).
with a PFK.
2. Methods
We analysed patients presenting to adult neurology and ophthal-
mology 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 examina-
tion were reviewed. A detailed clinical history was obtained.
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.
c) Visual fields with specific field defects.
d) FFA – minimal or no filling defect or delay in filling in the optic disc
and/or peripapillary choroid or choroidal watershed zone.
e) Exclusion of other causes of vision loss.
The diagnosis of AION requires that 3 out of first 4 criteria are met
while the 5th (e) criterion must essentially be fulfilled. Arteritic-AION
(A-AION) was considered in cases suspected of Giant cell arteritis or
any other vasculitic etiology while rest of the cases (NAION) were eval-
uated 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.

http://dx.doi.org/10.1016/j.jns.2017.02.002
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50 A. Patil et al. / Journal of the Neurological Sciences 376 (2017) 49–51

Fundus photography was obtained for all patients at first visit and dur-
ing follow ups using Visupac digital image archiving system (software
release 4.4) (Fig. 1).
An MRI brain and angiography of intracranial and neck vessels was
done in each case to exclude other probable causes for PFK. All patients
were evaluated for risk factors and vasculitic workup.
The clinical details of the patients with AION with PFK presentation
are given in Table 1.
were females. Mean age at presentation was 53.7 ± 11.9 years. 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. Only two out of the 7 patients had been on
antiplatelet agent (aspirin 75 mg/day). None of the risk factors were
significantly associated with the risk of recurrence. Poor visual acuity
in the fellow eye was not associated with poorer acuity or severity of
visual field defect in the presenting eye.

3. Results 4. Discussion

We evaluated 12 patients with sequential bilateral AION. Of these 7 The presence of unilateral disc atrophy and contralateral disc edema
patients had PFK like presentation. 4 (57.1%) out of these 7 patients has been classically termed as Foster Kennedy (FK) syndrome after the

Fig. 1. Fundus photographs showing disc edema in the presenting eyes and optic atrophy in the fellow eyes (left two columns), visual field defects(middle two columns) and fundus
fluorescein angiography findings (right two columns).

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 A. Patil et al. / Journal of the Neurological Sciences 376 (2017) 49–51 51

Table 1
Clinical profile of patients (VA-visual acuity, VF-visual field, FE-fellow eye, RE-right eye, LE-left eye, CF-CF: counting fingers close to face , HTN-hypertension, DM-diabetes mellitus, IHD-
ischemic heart disease, SUP-superior, INF-inferior, Asp: aspirin).

Patient Age Sex Presenting eye VA VF defect Risk factors VA FE VF FE Interval (months) Treatment received

A 48 F RE CF-CF Peripheral constriction HTN 4Y 6/60 Peripheral constriction 24 None

B 48 F RE 6/24 Diffuse DM 8Y 3/60 Diffuse 6 None
C 67 F RE 6/18 Diffuse HTN 12Y, IHD 2Y 6/24 Inf altitudinal 7 Asp
D 73 M LE 6/12 Diffuse DM 20Y, Smoking 38Y, 6/18 Diffuse 2 None
E 39 F RE 6/12 Inf sectoral Migraine 8Y 6/12 Inf altitudinal 30 None
F 52 M RE 6/9 Inferior and nasal quadrant Smoking 25Y 6/60 Nasal sectoral 8 Asp
G 49 M LE 6/36 Diffuse HTN 2Y CF-CF Peripheral constriction 12 None

British neurologist Robert Foster Kennedy who described it in 1911 [8].
However the entity was first recognised by William Gowers in 1893
hence also known as Gowers-Paton-Kennedy syndrome. FK syndrome
typically results from frontal lobe tumours or optic nerve meningiomas
leading to ipsilateral optic atrophy. In the absence of mass or compres-
sive lesions such presentation is termed Pseudo-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 involve-
ment leads to similar clinical picture. The history of sudden acute often
sectoral visual loss in the fellow eye 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 years [3]. The mechanism of NAION is proposed to be multifactorial.
In addition to “disc at risk” (small optic nerve with small or absent phys-
iologic cup), it has been associated with various vascular risk factors
such as hypertension, diabetes mellitus, hyperlipidemia, obstructive
sleep apnea, smoking, migraine, drugs and various coagulopathies [10].
In the Ischemic Optic Neuropathy Decompression Trial (IONDT) ap-
proximately 15% of patients developed NAION in the fellow eye within
5 years [11]. Presumably the other eye is exposed to same risk factors
for small vessel disease and has the same structural 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 acuity of 20/200 or worse, di-
abetes, 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 association between smoking
and risk of recurrence.
5. Conclusion
The diagnosis in a PFK presentation is essentially one of exclusion.
Though tumorous causes which are invariably considered in all
cases, can be readily excluded by appropriate imaging techniques, a
low threshold of suspicion must be exercised to rule out bilateral
sequential NAION as one of the cause as has been highlighted by
our series.
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