| Article Access Statistics|
| Viewed||290 |
| Printed||14 |
| Emailed||0 |
| PDF Downloaded||7 |
| Comments ||[Add] |
Click on image for details.
|Year : 2022 | Volume
| Issue : 4 | Page : 1655-1657
Reverse Foster Kennedy Syndrome Caused by an Intra-axial Tumor
Raghvendra Ramdasi1, Smita Thorve1, Mayank Vekariya1, Chandresh Karnavat2
1 Department of Neurosurgery, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India
2 Department of Radiology, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India
|Date of Submission||18-Jun-2019|
|Date of Decision||14-Nov-2019|
|Date of Acceptance||29-Sep-2021|
|Date of Web Publication||30-Aug-2022|
Consultant, Department of Neurosurgery, Jaslok Hospital and Research Centre, 15 G Deshmukh Marg, Pedder Road, Mumbai - 400 026, Maharashtra
Source of Support: None, Conflict of Interest: None
We present a case of a 23-year-old male complaining of painless loss of vision in right eye for 1 year and rapidly progressing vision loss in left eye with bilateral lateral rectus palsy. His fundoscopy revealed optic atrophy on the right side and papilledema on the left. Magnetic resonance imaging (MRI) showed intra-axial contrast-enhancing left frontal tumor. We discuss this first case of reverse Foster–Kennedy syndrome in the English literature with emphasis on the clinico-radiological and clinico-histopathological correlation.
Keywords: Foster–Kennedy, intra-axial, reverse, tumor
Key Message: Amaurosis fugax can be a presentation of a patient presenting with radiation-induced carotid artery stenosis, and such patients can be safely managed by carotid angioplasty and stenting.
|How to cite this article:|
Ramdasi R, Thorve S, Vekariya M, Karnavat C. Reverse Foster Kennedy Syndrome Caused by an Intra-axial Tumor. Neurol India 2022;70:1655-7
After the first description of the Foster–Kennedy syndrome in 1911, it has been found to be associated with a variety of tumors and nontumorous conditions., Various types and subtypes have been described., We herewith introduce Reverse Foster–Kennedy in the English literature and describe the first case of intra-axial tumor causing this sign.
| » Case Report|| |
A 23-year-old male was brought to our hospital with complaints of painless progressive loss of vision in right eye over last 1 year. Over the period of last one month, he also started getting severe holocranial continuous headache with projectile morning vomiting. Along with this, the vision in the left eye diminished progressively so much so that at the time of presentation he as blind on both sides. He also had urge incontinence for last 1 week.
Neurological examination revealed loss of olfaction on the right side. There was no perception of light in both eyes. We found the relative afferent pupillary defect (RAPD) on the right side but the left pupil was sluggishly reacting. The fundus examination showed descending primary optic atrophy on the right side [Figure 1]a and papilledema with secondary optic atrophy on the left [Figure 1b]. He also had bilateral lateral rectus palsy. The rest neurological examination was unremarkable. His magnetic resonance imaging (MRI) showed left frontal intra-axial lesion heterogeneous on T1, T2, and SWI-weighted sequences, enhancing heterogeneously on gadolinium injection and causing significant subfalcine herniation [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d. SWI hypointense and T1 hyperintense areas seen within the tumor represent hemorrhage within [Figure 2]b and [Figure 2]c. The right optic nerve was compressed by the gyrus rectus and the left optic nerve was free [Figure 2]d and [Figure 2]e. There was a difference in the size of the optic nerves, the right being smaller in caliber than the left. This is due to the primary optic atrophy on the right side [Figure 2]e. The patient underwent left frontal craniotomy and near-total excision of the lesion. His left-sided vision improved after surgery to finger counting at 2 feet and right side was blind as before. The postoperative MRI showed free right optic nerve [Figure 2]f. His histopathological examination revealed a highly cellular neoplastic lesion with tumor cells scant cytoplasm, raised NC ratio, hyperchromasia, irregular nuclear contour, with brisk mitotic activity. There was evidence of focal areas necrosis and microvascular proliferation [Figure 3]a and [Figure 3]b suggestive of glioblastoma multiforme (GBM grade IV). There were foci of bleeding within the tumor [Figure 3]c. The cytoplasm showed brisk positivity to glial fibrillary acid protein (GFAP) [Figure 3]d. More than 50% of the nuclei showed p53 mutation [Figure 3]e. More than 80% of the tumor cells were mutant for the IDH1 gene [Figure 3]f, confirming that it was secondary GBM.
|Figure 1: Fundoscopy: (a) Fundus examination showing optic atrophy on the right side and (b) showing papilledema on the left with secondary optic atrophy.|
Click here to view
|Figure 2: Radiology of the tumor: (a) T2-weighted axial scan heterogeneous left frontal lesion with subfalcine herniation (blue arrow). (b and c): Susceptibility-weighted imaging (SWI) hypointense and T1 hyperintense areas in the tumor representing hemorrhage (yellow arrow) (d): shows heterogeneously enhancing lesion in the left frontal lesion with compression of the right optic nerve (blue and yellow arrows) by opposite gyrus rectus. The left optic nerve is free (yellow arrow). (e) Optic atrophy on right with prominence of optic nerve on left. (f) Release of the right optic nerve compression (yellow arrow).|
Click here to view
|Figure 3: Histopathology and immunohistochemistry of the tumor. (a) shows highly cellular neoplastic lesion with tumor cells scant cytoplasm, hyperchromasia, and brisk mitotic activity. There is evidence of necrosis in the same (black arrow). (b) Microvascular proliferation (black arrows). (c) Hemorrhage in the tumor (black arrows). (d) Brilliant cytoplasmic positivity to glial fibrillary acid protein (GFAP). (e) Nuclear p53 reactivity. (f) Brisk IDH 1 mutation.|
Click here to view
| » Discussion|| |
In the first description of this unique syndrome in 1911, Kennedy included only ipsilateral optic atrophy, ipsilateral central scotoma, and contralateral papilledema. However, in 1916, he added ipsilateral anosmia to the original syndrome. This syndrome is rare, constituting less than 1% of cases in the largest cranial neoplasm series. Foster–Kennedy syndrome can be of three types: classical, pseudo, and atypical.
Classical Foster–Kennedy syndrome is associated with either intracranial mass causing compression of optic nerve or raised intracranial pressure or both. Watnick et al. classified it into three types.
Type 1: Here, there is ipsilateral compression of optic nerve, the tumor causing atrophy, and raised intracranial pressure causes contralateral papilledema. This was originally described by Kennedy. The majority are caused by a meningioma of sphenoidal wing or subfrontal regions, frontal abscess, plasmacytoma, nasopharyngeal angiofibroma, neuroblastoma, and aneurysms.
Type 2: Here, the compression is bilateral but asymmetric. Thus, the nerve compressed for a longer time will atrophy and the other will swell. The meningiomas of the olfactory groove, craniopharyngioma, and pituitary adenoma can cause this.,
Type 3: Here, the tumor does not compress the nerve. Instead, it causes chronic raised intracranial pressure. The asymmetrical chronic papilledema ends in Foster–Kennedy syndrome. Pineal tumor can cause this kind of presentation.
Pseudo Foster–Kennedy syndrome: Here, there is neither tumor nor raised intracranial pressure. It commonly results from sequential optic neuritis and sequential ischemic optic neuropathy.
Atypical Foster–Kennedy: It does not fit into either of the above. Desai et al. reported a case of compression of the optic nerve by gyrus rectus displaced by central neurocytoma (indirect compression).
We introduce a new category “Reverse Foster–Kennedy syndrome,” where an ipsilateral tumor causes contralateral optic atrophy and papilledema on the same side. To the best of our knowledge, it is the first case in English literature. It is also the first case of intra-axial tumor presenting with Foster–Kennedy variant.
In our case, we propose that there was chronic displacement of the ipsilateral medial frontal structures by the slowly expanding ipsilateral intra-axial frontal mass. This might have caused the impingement of the contralateral optic nerve by the opposite gyrus rectus, leading to optic atrophy [Figure 2]d and [Figure 2]e. It also might have caused the compression of the contralateral olfactory nerve causing opposite side anosmia. There was conversion of the glioma into a high-grade glioblastoma [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d, [Figure 3]e, [Figure 3]f with resultant edema and raised intracranial pressure. There was also bleeding inside the tumor [Figure 2]b, [Figure 2]c, and [Figure 3]c, further increasing the mass effect. This caused the bilateral sixth nerve palsy and ipsilateral papilledema as the opposite side atrophic nonviable optic neurons might have failed to swell.
| » Conclusion|| |
Reverse Foster–Kennedy syndrome is an extremely rare presentation of intra-axial frontal tumor and does carry localization value.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| » References|| |
Kennedy F. Retrobulbar neuritis as an exact diagnostic sign of tumours and abscesses in the frontal lobes. Am J Med Sci 1911;142:355-68.
Kennedy F. A further note on the diagnostic valuc of rctrobulbar neuritis in expanding lcsions of the frontal lobes, with a report of this syndrome in a case of aneurysm of the right internal earotld artery. J Amer Med Assoc 1916;67:1361-3.
Lotfipour S, Chiles K, Kahn JA, Bey T, Rudkin S. An unusual presentation of subfrontal meningioma: A case report and literature review for Foster Kennedy syndrome. Intern Emerg Med 2011;6:267-9.
Watnick RL, Trobe JD. Bilateral optic nerve compression as a mechanism for the Foster Kennedy syndrome. Ophthalmology 1989;96:1793-8.
Mbekeani JN, Ahmed M, Hassounah MI, Abdulshafi K, Al Hazzaa SA, Al Hindi H. Papillary tumor of the pineal region presenting with Foster Kennedy sign. Hematol Oncol Stem Cell Ther 2015;8:140-2.
Liang F, Ozanne A, Offret H, Ducreux D, Labetoulle M. An atypical case of Foster Kennedy syndrome. Interv Neuroradiol 2010;16:429-32.
Desai N, Yong RL, Doshi A, Rucker JC. Pseudo-Foster-Kennedy syndrome with optic nerve compression by the gyrus rectus. Neurology 2015;85:385.
[Figure 1], [Figure 2], [Figure 3]