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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].

Anosmia
  • […] of a mass. [3] Presentation [ edit ] 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[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]
  • The patient had right-sided anosmia. Computed tomography and magnetic resonance imaging showed a lesion in the frontal lobe that was deviated to the right and attached to the olfactory groove.[ncbi.nlm.nih.gov]
  • A 52-year-old man had progressive loss of visual acuity in his left eye associated with anosmia of five years duration. Clinical findings included papilledema in the right eye and optic atrophy in the left eye.[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]
  • scotoma (loss of vision in the middle of the visual fields) in the ipsilateral eye anosmia (loss of smell) ipsilaterally This syndrome is due to optic nerve compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary[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]
  • 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.org]
  • Refractive error : Hyperopia / Myopia - Astigmatism - Anisometropia / Aniseikonia - Presbyopia Visual disturbances and blindness Amblyopia - Leber's congenital amaurosis - Subjective ( Asthenopia , Hemeralopia , Photophobia , Scintillating scotoma ) - Diplopia[wikidoc.org]
  • Kelumpuhan lesi N VI Lesi N VI melumpuhkan otot rektus lateralis, jadi melirik kearah luar (lateral, temporal) terganggu pada mata yang terlibat, yang mengakibatkan diplopia horizontal.[lellyanggrainy.blogspot.com]
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. [3] Presentation [ edit ] The syndrome is defined as the following changes: optic atrophy in the ipsilateral eye disc[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 is a very rare syndrome which includes ipsilateral optic atrophy and central scotoma, anosmia, contralateral papilledema, and, occasionally, ipsilateral proptosis.[ncbi.nlm.nih.gov]

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.

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]
  • scotoma (loss of vision in the middle of the visual fields) in the ipsilateral eye anosmia (loss of smell) ipsilaterally This syndrome is due to optic nerve compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary[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

  • 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]
  • 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). [6] Treatment and prognosis [ edit ] The treatment, and therefore[en.wikipedia.org]
  • The diagnosis, treatment, and prognosis of such tumors are reviewed.[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]
  • Early detection and initial surgical treatment with adjunct radiotherapy could have prevented visual loss on this boy.[ncbi.nlm.nih.gov]

Prognosis

  • prognosis, varies depending upon the underlying tumour. [5] History [ edit ] The syndrome was first extensively noted by Robert Foster Kennedy in 1911, a British neurologist , who spent most of his career working in the United States of America . [7][en.wikipedia.org]
  • 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 v t e Eye disease - pathology of the eye ( H00-H59 , 360-379 ) Adnexa eyelid : inflammation ( Stye , Chalazion , Blepharitis ) - Entropion - Ectropion[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]

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]
  • 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.[ncbi.nlm.nih.gov]
  • 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]
  • This combination of signs may be considered a form of FKS because of its common etiology.[journals.lww.com]
  • 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). NAION is more commonly seen [15].[eyewiki.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]
  • Bukti tmbahan etiologi familial berasal dari studi epidemiologi yang membandingkan keluarga dengan riwayat tumor otak dan dengan kontrol.[electricore18.blogspot.com]
  • KELLY E-Mail [email protected] Slone Epidemiology Unit, Boston University School of Public Health, 1010 Commonwealth Eve, Boston, MA 02215 - Prof. Dr.[sjsupport.org]
Sex distribution
Age distribution

Pathophysiology

  • Papilledema is mostly seen as a symptom resulting from another pathophysiological process.[icd.codes]
  • Intracranial pressure measurements would help elucidate the pathophysiology of this particular case, but they were not performed because they were not deemed necessary for the diagnosis.[journals.lww.com]
  • 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]

Prevention

  • Early detection and initial surgical treatment with adjunct radiotherapy could have prevented visual loss on this boy.[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.[ncbi.nlm.nih.gov]
  • Early detection and treatment could prevent vision loss. See an eye care professional right away if you have a sudden change in vision, if everything looks dim, or if you see flashes of light.[icdlist.com]
  • Risk Factors Risk Factors for NAION: Hypertension Diabetes Mellitus High cholesterol History of smoking Sleep Apnea Syndrome [12] Primary prevention Primary prevention of NAION includes control of vasculopathic risk factors (e.g., blood pressure, blood[eyewiki.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.

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Last updated: 2018-06-21 23:55