The classical description of this rare condition, Foster-Kennedy syndrome, consists of four elements: ipsilateral optic nerve atrophy, contralateral papilledema, central scotoma, and anosmia while ipsilateral proptosis has also been described in some patients.
Presentation
Based on the pathogenic mechanism, three types of Foster-Kennedy syndromes have been described [1]:
- Type 1 – only one optic nerve is directly compressed and intracranial hypertension is present
- Type 2 – compression of bilateral, optic nerves asymmetrically; the nerve which is more severely compressed becomes atrophied, while the other, less compressed nerve leads to papilledema
- Type 3 – the nerves are not directly compressed by the tumor, their damage is due to chronic intracranial hypertension.
The most prominent symptoms are, as expected, visual: unilateral or bilateral vision loss that, if left untreated, becomes permanent in time, visual field defects, reduced color vision, and central scotoma. Visual obscurations may also be temporary when intracranial or systemic blood pressure is transiently decreased. Symptoms regress if early treatment is applied [2] [3]. Associated symptoms include nausea and vomiting as signs of intracranial hypertension [4], memory loss and emotional lability as an expression of frontal lobe involvement. Headaches and weakness may complete the clinical picture, which is slightly variable depending on the location of the pathological process [5] and pathogenic mechanism [6] [7].
Entire Body System
- Swelling
The appearance of unilateral optic disc swelling with contralateral optic disc atrophy has been described as the Foster Kennedy Syndrome. [jmedicalcasereports.biomedcentral.com]
It is characterized by the triad of unilateral optic disc swelling, contralateral optic atrophy and ipsilateral anosmia. [sciencedomain.org]
- Pallor
The original description of the Foster Kennedy syndrome included the clinical triad of optic disc pallor in one eye, optic disc edema in the other eye, and reduced olfaction caused by space-occupying anterior fossa masses. [ncbi.nlm.nih.gov]
The presence of pallor in one eye and disc edema in the contralateral eye in the absence of an intracranial mass is called pseudo-Foster Kennedy syndrome. [eyerounds.org]
DISCUSSION We have described a patient who developed the ophthalmic manifestations of the Foster Kennedy syndrome-optic disc pallor in one eye and optic disc edema in the other eye-from a meningioma compressing one optic nerve (to produce optic disc pallor [journals.lww.com]
Because of her well-documented history of unilateral optic nerve head edema replaced by pallor in four weeks, neuroimaging was not performed. [aao.org]
The right optic nerve head showed less disc edema but the optic disc pallor became evident at this time [Figure 2]a. The picture may not do justice to the superior differentiation of the pallor. Loss of the superior nerve fiber layer was noted. [mjdrdypu.org]
- Inflammation
[…] there is unilateral optic disc swelling with contralateral optic atrophy in the absence of an intracranial mass.This occurs typically due to bilateral sequential optic neuritis or ischaemic optic neuropathy. [ 3 ] A case secondary to pachymeningitis (inflammation [patient.co.uk]
His main clinical and laboratory interest is intra-ocular inflammation (uveitis) and he has published over 150 papers on this subject. He is also an author of three ophthalmology textbooks and has contributed chapters to others. [books.google.com]
Other symptoms that need quick attention are pain, double vision, fluid coming from the eye, and inflammation. [icdlist.com]
IFN-γ plays a key role in macrophage activation, inflammation, and host defense against intracellular pathogens. [mdpi.com]
Foster-Kennedy syndrome Orbital apex syndrome Cavernous sinus syndrome Etiology Cavernous sinus thrombosis (e.g., due to sinusitis) Carotid-cavernous fistula Cavernous sinus tumors Carotid artery aneurysms Tolosa-Hunt syndrome (idiopathic granulomatous inflammation [amboss.com]
- Weakness
[…] leg and doubtful weakness of right arm. [ijo.in]
Other common complications depend on the area of intracranial involvement and potentially include nausea, vomiting, vertigo, hearing loss, memory loss, limb weakness, and facial paresis. [heraldopenaccess.us]
Headaches and weakness may complete the clinical picture, which is slightly variable depending on the location of the pathological process and pathogenic mechanism. [symptoma.com]
Recently, a BSMA phenotype with distal predominance of limb weakness and wasting has been reported, caused by mutations in a subunit of the dynactin 1 DCTN1 gene. [orpha.net]
- Fatigue
Initial clinical manifestations include tremor, muscle cramps, muscle twitching, fatigue and slurred speech. [orpha.net]
Depression was reported in 2.5%–15.4% of patients with primary brain tumors[5] and was more commonly found in patients with frontal lobe tumors.[6] Patients may also present with apathy, which is a state of motivation and chronic fatigue due to functional [tnoajosr.com]
He experienced daily temporal headaches, dizziness, and fatigue. In addition, he had erectile dysfunction and a lack of facial hair. He did not report galactorrhoea. [edm.bioscientifica.com]
Interestingly, some authors have recently advanced the hypothesis of a viral involvement in the pathogenesis of Fibromyalgia and Chronic Fatigue Syndrome, which share many pathognomonic features with MCS [37]. [mdpi.com]
Respiratoric
- Anosmia
Tumor at the base of the frontal lobe produces inappropriate behavior, optic nerve atrophy on the side of the tumor, papilledema on the other side, and anosmia (Foster- Kennedy syndrome). [en.wikipedia.org]
Type your tag names separated by a space and hit enter Abstract Tumors of the olfactory groove may cause unilateral optic atrophy with contralateral papilledema and anosmia (Foster Kennedy syndrome). [unboundmedicine.com]
Foster Kennedy syndrome is a rare neurological entity that includes ipsilateral optic atrophy, contralateral papilledema, and sometimes anosmia. [heraldopenaccess.us]
- ipsilateral optic atrophy - ipsilateral anosmia - raised icp (papilledema in the contralateral eye, nausea, vomiting,...) due to frontal mass. typically olphatory groove meningioma 26 8 Reply optic atrophy and anosmia due to compression of optic and [dailyrounds.org]
The patient may present with anosmia and frontal lobe signs alongside the visual symptoms (central scotoma). In the absence of intracranial mass, the above mentioned findings are named pseudo- Foster Kennedy Syndrome. [score99.blogspot.com]
- Hyposmia
Vitamin A deficiency is associated with hyposmia and dysosmia (odors are unpleasant), possibly the result of nasal mucosal abnormalities. c. Zinc deficiency has been associated with hyposmia and dysosmia. d. Diabetes mellitus. [dartmouth.edu]
L'Hospitalet, Spain; E-mail: [email protected] doi: 10.1097/WNO.0b013e3181a63583 Free Abstract The original 1911 description of the Foster Kennedy syndrome included the triad of optic disc pallor in one eye, optic disc edema in the fellow eye, and anosmia or hyposmia [journals.lww.com]
Gastrointestinal
- Vomiting
Other common complications depend on the area of intracranial involvement and potentially include nausea, vomiting, vertigo, hearing loss, memory loss, limb weakness, and facial paresis. [heraldopenaccess.us]
There are other symptoms present in some cases such as nausea and vomiting, memory loss and emotional lability (i.e., frontal lobe signs). Brain tumor can be visualized very well on CT scan, but MRI gives better detail and is the preferred study. [en.wikipedia.org]
Presentation of Case: A 21-year-old female presented with bilateral blurring of vision with preceded by generalized headache, nausea and vomiting. [sciencedomain.org]
[…] cardinal features, in relation to a mass lesion 1,2: ipsilateral optic nerve atrophy presenting with central scotoma contralateral optic disc swelling due to papilledema Other common clinical features include 1,2: ipsilateral anosmia headache nausea and vomiting [radiopaedia.org]
[…] compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary to a mass (such as meningioma or plasmacytoma, usually an olfactory groove meningioma).[6][7] There are other symptoms present in some cases such as nausea and vomiting [wikidoc.org]
- Nausea
Other common complications depend on the area of intracranial involvement and potentially include nausea, vomiting, vertigo, hearing loss, memory loss, limb weakness, and facial paresis. [heraldopenaccess.us]
There are other symptoms present in some cases such as nausea and vomiting, memory loss and emotional lability (i.e., frontal lobe signs). Brain tumor can be visualized very well on CT scan, but MRI gives better detail and is the preferred study. [en.wikipedia.org]
Presentation of Case: A 21-year-old female presented with bilateral blurring of vision with preceded by generalized headache, nausea and vomiting. [sciencedomain.org]
[…] of two cardinal features, in relation to a mass lesion 1,2: ipsilateral optic nerve atrophy presenting with central scotoma contralateral optic disc swelling due to papilledema Other common clinical features include 1,2: ipsilateral anosmia headache nausea [radiopaedia.org]
[…] nerve compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary to a mass (such as meningioma or plasmacytoma, usually an olfactory groove meningioma).[6][7] There are other symptoms present in some cases such as nausea [wikidoc.org]
Cardiovascular
- Hypertension
We discuss the different pathogenic mechanisms to explain this case, for which a chronic venous hypertension was the most probable etiology. [ncbi.nlm.nih.gov]
There was no past history suggestive of hypertension, diabetes, or any other major systemic illness. Patient's mother was a known case of hypertension and diabetes. [mjdrdypu.org]
Update on idiopathic intracranial hypertension. Am J Ophthalmol. 2011; 152: 163-169 Wall M. White W.N. Asymmetric papilledema in idiopathic intracranial hypertension: prospective interocular comparison of sensory visual function. [canadianjournalofophthalmology.ca]
Musculoskeletal
- Muscle Cramp
Initial clinical manifestations include tremor, muscle cramps, muscle twitching, fatigue and slurred speech. [orpha.net]
A study of 8 heterozygous female patients with proven tandem CAG repeats showed that 50% had subclinical phenotypic expression. [26] Their clinical findings were normal, except for muscle cramps and finger tremors. [emedicine.medscape.com]
Eyes
- Central Scotoma
Symptoms include Visual loss in 70% of cases, visual field defects, usually a central scotoma, in 9%, and extraocular motility limitation in 6% [8]. Headache and anosmia are also often present. [heraldopenaccess.us]
Ken·ne·dy syn·drome ( ken'ĕ-dē ), ipsilateral optic atrophy with central scotoma and contralateral choked disc or papilledema, caused by a meningioma of the ipsilateral optic nerve. [medical-dictionary.thefreedictionary.com]
The syndrome is defined as the following changes: optic atrophy in the ipsilateral eye disc edema in the contralateral eye central scotoma (loss of vision in the middle of the visual fields) in the ipsilateral eye anosmia (loss of smell) ipsilaterallyThis [en.wikipedia.org]
The patient may present with anosmia and frontal lobe signs alongside the visual symptoms (central scotoma). In the absence of intracranial mass, the above mentioned findings are named pseudo- Foster Kennedy Syndrome. [score99.blogspot.com]
- Diplopia
A 44-year-old man was examined for headache and diplopia. Left eye showed limitation of abduction. Ocular fundus, computed tomography (CT), and magnetic resonance imaging (MRI) of the head and orbits were normal. The diplopia subsided spontaneously. [ncbi.nlm.nih.gov]
Enlarged blind spots Symptoms Progressive visual loss Headache Nausea/vomiting Anosmia Diplopia Emotional lability Physical examination Visual acuity is variably affected in the ipsilateral eye with optic atrophy A relative afferent pupillary defect [eyewiki.org]
The diplopia subsided spontaneously. Sixmonths later, he noticed sudden visual loss in the left eye.The left eye showed optic disk atrophy and episcleritis,and the right eye showed papilloedema. [docslide.com.br]
Psychiatrical
- Aggressive Behavior
Conflicts related to food may lead to other behavioral problems. Aggressive behaviors or acting out can also be problematic. Co-management with psychology and/or psychiatry may be important if simple measures are ineffective. [medicalhomeportal.org]
Urogenital
- Urinary Incontinence
When symptomatic, they can produce Foster Kennedy syndrome (anosmia, ipsilateral optic atrophy, contralateral papilledema), metal status change, urinary incontinence Histological variables follow WHO grading I to III (from low recurrence to high rates [radiologyinthai.blogspot.com]
The typical presentation of the frontal lobe tumors includes difficulty in attention and concentration, urinary incontinence, problems in memory and learning, and difficulties in impulse control, reasoning, planning, decision-making, control, and coordination [tnoajosr.com]
Neurologic
- Foster-Kennedy Syndrome
[…] on Foster Kennedy syndrome Articles on Foster Kennedy syndrome in N Eng J Med, Lancet, BMJ Media Powerpoint slides on Foster Kennedy syndrome Images of Foster Kennedy syndrome Photos of Foster Kennedy syndrome Podcasts & MP3s on Foster Kennedy syndrome [wikidoc.org]
Fundoscopy revealed optic nerve atrophy of the right eye and a swollen optic disc on the left in keeping with features of Foster Kennedy Syndrome. [sciencedomain.org]
Foster Kennedy syndrome (also known as Gowers–Paton–Kennedy syndrome, Kennedy's phenomenon or Kennedy's syndrome) is a constellation of findings associated with tumors of the frontal lobe.Although Foster Kennedy syndrome is equated with Kennedy syndrome [en.wikipedia.org]
[…] papilledema and anosmia (Foster Kennedy syndrome). [unboundmedicine.com]
- Papilledema
This mass directly compresses one optic nerve, accounting for ipsilateral optic nerve atrophy, and causes chronic raised intracranial pressure resulting in contralateral papilledema 1,2. [radiopaedia.org]
Similar Codes ICD-9 Code ICD-9 Description 377.00 Papilledema, unspecified 377.01 Papilledema associated with increased intracranial pressure 377.02 Papilledema associated with decreased ocular pressure 377.03 Papilledema associated with retinal disorder [healthprovidersdata.com]
Foster-Kennedy syndrome Syndrome (see: Disease) + Papilledema(choked disc) + Parent Code: H47.1 - Papilledema [icd.codes]
Foster Kennedy syndrome is a rare neurological entity that includes ipsilateral optic atrophy, contralateral papilledema, and sometimes anosmia. [heraldopenaccess.us]
Pseudo-Foster Kennedy syndrome is defined as one-sided optic atrophy with papilledema in the other eye but with the absence of a mass. [en.wikipedia.org]
- Headache
The vision in her right eye has also deteriorated over the preceding week together with frontal headaches but not associated with nausea and vomiting. On examination, she was generally well, afebrile, alert, and oriented. [heraldopenaccess.us]
CASE PRESENTATION: We present a 21-year-old male patient, presented with progressive visual disturbance of the left eye, associated with global headache. The headache was refractory to over-counter medications. [ethjhealths.org]
Presentation of Case: A 21-year-old female presented with bilateral blurring of vision with preceded by generalized headache, nausea and vomiting. [sciencedomain.org]
She had a long history of progressive headaches, fainting attacks, and ultimately vomiting. [journals.lww.com]
- Seizure
However, most patients with brain tumors do not have a seizure as the first symptom. [link.springer.com]
A rare occurrence of transient oculomotor nerve palsy during the seizure episode has also been reported in our patient. [tnoajosr.com]
In December 1987, after a generalized tonic-clonic seizure, she was found to have a large right frontoparietal mass without direct impingement on the optic nerves, or chiasm on neuroradiological studies. [ncbi.nlm.nih.gov]
We report a 43-year-old female patient of sphenoid ridge meningioma with not only unilateral optic atrophy, contralateral disc swelling, but also headache, seizure and unilateral anosmia. [tmu.pure.elsevier.com]
The increased use of seizure medications can compound the losses if infection also exacerbates seizures. [dovepress.com]
- Confusion
[…] syndrome (also known as Gowers–Paton–Kennedy syndrome, Kennedy's phenomenon or Kennedy's syndrome) is a constellation of findings associated with tumors of the frontal lobe.Although Foster Kennedy syndrome is equated with Kennedy syndrome, it should not be confused [en.wikipedia.org]
A later description was written by Wilhelm Uhthoff in 1915.[3] Although "Foster Kennedy syndrome" is equated with "Kennedy syndrome",[4] it should not be confused with Kennedy disease, which is named for W. R. Kennedy. [wikidoc.org]
Subsequently, because of discrepancies in the definition of this syndrome, and arguments about frontal space-occupying lesions and nonspace-occupying lesions as well as confusion among papilledema, dise swelling, and papillitis, some neurologists and [tmu.pure.elsevier.com]
Workup
Workup aims to identify the pathological process leading to the appearance of Foster-Kennedy syndrome and to evaluate the extent of optic nerve damage. Thus, computer tomography (CT) [8] and magnetic resonance imaging (MRI) of the brain are useful tools [9]. CT is optimum when trying to determine bone involvement and thus predict recurrence risk, whereas MRI better describes soft tissues.
Ocular evaluation should include fundus examination to determine the degree of papillary edema and observe disk swelling or pallor, nerve fiber layer evaluation with red-free light, color vision test, capillary number test (less than 10 capillaries on the optic disk suggest optic atrophy) and electrophysiological tests, in cases where symptoms do not match disk pallor. The assessment of the electrical activity of the visual cortex after retinal stimulation comprises two parameters, amplitude, and latency, both of which are affected due to optic nerve malfunction. Pupillary reflexes assessment should also be performed.
Visual Field Test
- Central Scotoma
Symptoms include Visual loss in 70% of cases, visual field defects, usually a central scotoma, in 9%, and extraocular motility limitation in 6% [8]. Headache and anosmia are also often present. [heraldopenaccess.us]
Ken·ne·dy syn·drome ( ken'ĕ-dē ), ipsilateral optic atrophy with central scotoma and contralateral choked disc or papilledema, caused by a meningioma of the ipsilateral optic nerve. [medical-dictionary.thefreedictionary.com]
The syndrome is defined as the following changes: optic atrophy in the ipsilateral eye disc edema in the contralateral eye central scotoma (loss of vision in the middle of the visual fields) in the ipsilateral eye anosmia (loss of smell) ipsilaterallyThis [en.wikipedia.org]
The patient may present with anosmia and frontal lobe signs alongside the visual symptoms (central scotoma). In the absence of intracranial mass, the above mentioned findings are named pseudo- Foster Kennedy Syndrome. [score99.blogspot.com]
Treatment
[…] and prognosis The treatment, and therefore prognosis, varies depending upon the underlying tumour.[7] References ↑ Template:DorlandsDict ↑ Thorofare, NJ (1911). [wikidoc.org]
Treatment and prognosis Treatment options vary depending on the exact cause but generally 'true' Foster Kennedy syndrome requires neurosurgical intervention as part of management 1. [radiopaedia.org]
Treatment / Management The treatment modality for Foster Kennedy syndrome tumors include: Surgical resection Chemotherapy Radiotherapy Medical therapy The management of FKS is usually by surgical resection, although chemotherapy and radiotherapy are also [ncbi.nlm.nih.gov]
[…] watch 01:30 The Loop (TV) Foster Kennedy syndrome Pathology Type Cancer Cause(s) Brain tumor Symptoms Damage to optic nerve, optic disc pressure, loss of vision in middle of visual field, loss of sense of smell Treatments Treatment of underlying tumor [house.fandom.com]
Prognosis
The treatment, and therefore prognosis, varies depending upon the underlying tumour.[7] References ↑ Template:DorlandsDict ↑ Thorofare, NJ (1911). [wikidoc.org]
The treatment, and therefore prognosis, varies depending upon the underlying tumour. [en.wikipedia.org]
The diagnosis, treatment, and prognosis of such tumors are reviewed. [ncbi.nlm.nih.gov]
Etiology
Potential etiologies include traumatic optic neuropathy, prior optic neuritis, compressive optic neuropathy, and a history of meningitis. Additional etiologies. There are some rare reports of PFK occurring in younger individuals. [aao.org]
We discuss the different pathogenic mechanisms to explain this case, for which a chronic venous hypertension was the most probable etiology. [ncbi.nlm.nih.gov]
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
In optic atrophy that does not show characteristic visual field alterations, typical symptoms, or the classic evolution of a given disease, diagnostic imaging may reveal the etiology. [journals.lww.com]
Epidemiology
Assessing Exposures to Magnetic Resonance Imaging's Complex Mixture of Magnetic Fields for In Vivo, In Vitro, and Epidemiologic Studies of Health Effects for Staff and Patients. Front Public Health. 2018;6:66. [ncbi.nlm.nih.gov]
Summary Epidemiology The prevalence of BSMA is 1/30,000 male births. The incidence is 1/526,315 males/year. Clinical description Disease onset occurs between 30-60 years of age. [orpha.net]
Journal of medical case reports. 2008 Mar 18; [PubMed PMID: 18348732] [9] Ogasawara C,Philbrick BD,Adamson DC, Meningioma: A Review of Epidemiology, Pathology, Diagnosis, Treatment, and Future Directions. [statpearls.com]
Brain tumors and hormonal factors: review of the epidemiological literature. Cancer Causes Control (2011) 22(5):697–714. doi:10.1007/s10552-011-9742-7 CrossRef Full Text 10. Bickerstaff ER, Small JM, Guest IA. [frontiersin.org]
Pathophysiology
Papilledema is mostly seen as a symptom resulting from another pathophysiological process. [icd.codes]
Objectives: Describe the pathophysiology of patients with Foster Kennedy syndrome. Review the etiology of Foster Kennedy syndrome. [ncbi.nlm.nih.gov]
Other investigators have also reviewed CAG repeats in KD. [31, 32] A number of molecular pathophysiologic studies of the androgen receptor have been conducted to clarify its role in the pathogenesis of KD. [33, 18, 34, 35, 36, 37, 38, 39] Androgen-receptor [emedicine.medscape.com]
Prevention
We describe a case of pseudo-Foster Kennedy Syndrome in a two year old boy with unilateral papilloedema due to a congenital optic disc anomaly in one eye preventing transmission of raised intracranial pressure to the optic nerve. [ncbi.nlm.nih.gov]
Conclusion In this case the finding of unilateral papilloedema was due to a congenital abnormality of the left optic disc, preventing transmission of the raised intracranial pressure to the optic nerve head. [doi.org]
References
- Watnick RL, Trobe JD. Bilateral optic nerve compression as a mechanism for the Foster Kennedy Syndrome. Ophthalmology. 1989;96(12):1793–1798.
- Bulters DO, Shenouda E, Evans BT, et al. Visual recovery following optic nerve decompression for chronic compressive neuropathy. Acta Neurochir. 2009;151:32
- Fogarty-Mack P, Pile-Spellman J, Hacein-Bey L, et al. The effect of arteriovenous malformations on the distribution of intracerebral arterial pressures. Am J Neuroradiol. 1996;17:1443–1449.
- Biousse V, Bousser MG. L'hypertension intracrânienne bénigne. Rev Neurol. 2001;157:21–34.
- Lotfipour S, Chiles K, Kahn JA, et al. An unusual presentation of subfrontal meningioma: a case report and literature review for Foster Kennedy syndrome. Intern Emerg Med. 2011;6:267–269.
- Ruben S, Elston J, Hayward R. Pituitary adenoma presenting as the FosterKennedy syndrome. Br J Ophthalmol. 1992;76:117–119.
- Walia HS, Grumbine FL, Sawhney GK, et al. An aggressive sphenoid wing meningioma causing Foster Kennedy syndrome. Case Rep Ophthalmol Med. 2012;102365.
- Jarus GD, Feldon SE. Clinical and computed tomographic findings in the Foster Kennedy Syndrome. Am J Ophthal. 1982;93:317–322.
- Fogarty-Mack P, Pile-Spellman J, Hacein-Bey L, et al. The effect of arteriovenous malformations on the distribution of intracerebral arterial pressures. Am J Neuroradiol. 1996;17:1443–1449.