Edit concept Question Editor Create issue ticket

Foster-Kennedy Syndrome

Foster Kennedy's Syndrome

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]:

  1. Type 1 – only one optic nerve is directly compressed and intracranial hypertension is present
  2. 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
  3. 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].

Lymphadenopathy
  • There was no significant lymphadenopathy. There was no dependent oedema. Naso-pharyngeal examination did not reveal any abnormality. Respiratory system and cardiovascular system did not reveal any abnormality. The liver and spleen were not palpable.[ijo.in]
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]
  • Foster Kennedy syndrome is a very rare syndrome which includes ipsilateral optic atrophy and central scotoma, anosmia, contralateral papilledema, and, occasionally, ipsilateral proptosis.[ncbi.nlm.nih.gov]
  • . - 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]
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]
Central Scotoma
  • 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.[symptoma.com]
  • Foster Kennedy syndrome is a very rare syndrome which includes ipsilateral optic atrophy and central scotoma, anosmia, contralateral papilledema, and, occasionally, ipsilateral proptosis.[ncbi.nlm.nih.gov]
  • 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]
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]
  • 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]
  • ., headache diplopia, nausea and vomiting) [6] Family members may report personality changes Figure 2 : Patterns of visual field loss in optic neuropathies. A. OD: Paracentral scotoma OS: Cecocentral scotoma. B. Central scotoma. C. Arcuate scotoma.[eyewiki.aao.org]
Flame-shaped Hemorrhage
  • Initial presentation of the right optic nerve head showed disc edema, peripapillary flame shaped hemorrhages, nerve fi ber layer attenuation, venular distention, and partial macular star.[mjdrdypu.org]
Papilledema
  • Clinical findings included papilledema in the right eye and optic atrophy in the left eye. A diagnosis of Foster Kennedy syndrome was made.[ncbi.nlm.nih.gov]
  • 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]
  • 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[symptoma.com]
  • ", keywords "Foster Kennedy syndrome, Meningioma, Optic atrophy, Papilledema", author "Sheu, {J. J.} and Chen, {P. N.} and Yip, {B. S.} and Ko, {M. L.[tmu.pure.elsevier.com]
  • Foster-Kennedy syndrome Syndrome (see: Disease ) Papilledema (choked disc) Parent Code: H47.1 - Papilledema[icd.codes]
Tonic-Clonic Seizure
  • 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]
Altered Mental Status
  • May also present with symptoms related to pre-frontal cortex involvment like desinhibition, emotional lability, altered mental status 14 Reply I agree with the diagnosis. 1 Reply Foster Kennedy syndrome is Ipsilateral optic nerve atrophy. ipsilateral[dailyrounds.org]

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.

Elevated Sedimentation Rate
  • The patient demonstrated a chronic, corticosteroid-sensitive, relapsing optic neuropathy associated with an elevated sedimentation rate and a monoclonal gammopathy. The diagnosis, treatment, and prognosis of such tumors are reviewed.[ncbi.nlm.nih.gov]
Central Scotoma
  • 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.[symptoma.com]
  • Foster Kennedy syndrome is a very rare syndrome which includes ipsilateral optic atrophy and central scotoma, anosmia, contralateral papilledema, and, occasionally, ipsilateral proptosis.[ncbi.nlm.nih.gov]
  • 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]

Treatment

  • Conclusions: Although there is no current generally accepted treatment for NAION, a correct diagnosis and supportive treatment may contribute to the improvement in visual acuity (VA), improvement that in this case remained stable for 6 months after the[ncbi.nlm.nih.gov]
  • 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 Show Information [Source][house.wikia.com]
  • Treatment and follow-up in 41 cases. Amer Surg 35 (1969) 510–515 Google Scholar [9] Raskind R, SR Weiss, JJ Manning, RE Wermuth : Survival after surgical excision of single metastatic brain tumors.[link.springer.com]

Prognosis

  • The diagnosis, treatment, and prognosis of such tumors are reviewed.[ncbi.nlm.nih.gov]
  • A later description was written by Wilhelm Uhthoff in 1915. [7] Treatment and prognosis The treatment, and therefore prognosis, varies depending upon the underlying tumour. [5] References[ipfs.io]
  • The treatment, and therefore prognosis, varies depending upon the underlying tumour. [7] References de:Foster Kennedy-Syndrom it:Sindrome di Foster-Kennedy[wikidoc.org]
  • The treatment, and therefore prognosis, varies depending upon the underlying tumour.[en.wikipedia.org]

Etiology

  • 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]
  • 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]
  • Etiology The most common etiology for PFKS is bilateral sequential anterior ischemic optic neuropathies (AION), either arteritic (AAION) or nonarteritic anterior ischemic optic neuropathy (NAION).[eyewiki.aao.org]

Epidemiology

  • 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]
  • 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]
  • KELLY E-Mail jkelly@slone.bu.edu Slone Epidemiology Unit, Boston University School of Public Health, 1010 Commonwealth Eve, Boston, MA 02215 - Prof. Dr.[sjsupport.org]
  • Epidemiology Frequency United States The estimated incidence is approximately 1 case in 40,000 men.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Papilledema is mostly seen as a symptom resulting from another pathophysiological process.[icd.codes]
  • 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]
  • That appears to be the pathophysiology of AION." A "disc at risk." A small, crowded optic nerve head is a risk factor for AION; if axons swell, they may compress neighboring axons, triggering a cascade. All images courtesy of Jacqueline M.S.[reviewofophthalmology.com]
  • The pathophysiology of the disorder results from the depletion of mtDNA and is influenced by multiple environmental factors, in particular viral infections and medication exposure.[dovepress.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

Article

  1. Watnick RL, Trobe JD. Bilateral optic nerve compression as a mechanism for the Foster Kennedy Syndrome. Ophthalmology. 1989;96(12):1793–1798.
  2. Bulters DO, Shenouda E, Evans BT, et al. Visual recovery following optic nerve decompression for chronic compressive neuropathy. Acta Neurochir. 2009;151:32
  3. 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.
  4. Biousse V, Bousser MG. L'hypertension intracrânienne bénigne. Rev Neurol. 2001;157:21–34.
  5. 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.
  6. Ruben S, Elston J, Hayward R. Pituitary adenoma presenting as the FosterKennedy syndrome. Br J Ophthalmol. 1992;76:117–119.
  7. Walia HS, Grumbine FL, Sawhney GK, et al. An aggressive sphenoid wing meningioma causing Foster Kennedy syndrome. Case Rep Ophthalmol Med. 2012;102365.
  8. Jarus GD, Feldon SE. Clinical and computed tomographic findings in the Foster Kennedy Syndrome. Am J Ophthal. 1982;93:317–322.
  9. 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.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2019-06-28 10:26