In most cases, the papilledema is bilateral, however can be unilateral in foster kennedy syndrome and its pseudovariants. Foster kennedy s syndrome fks is a rare neurological sign first described in 1911 by robert foster kennedy. A 52yearold woman presented to the ophthalmologist with complaints of decrease of vision in her right eye for the. The pathogenesis of the optic disc pallor was believed to be direct compression of the prechiasmatic optic nerve. Foster kennedy syndrome is a rare entity found with intracranial neoplasms. It is caused by compression of the ipsilateral optic nerve and olfactory nerve early, followed by secondarily increased intracranial pressure that. We describe a peculiar case of foster kennedy syndrome associated with an arteriovenous malformation in which neither optic nerve compression nor icht was obvious. The treatment of kallmann syndrome typically involves hormone replacement therapy to normalize the sex hormone levels, so as to induce and maintain sexual development. Agreement as to what constitutes the actual syndrome and what are the common causes of it does not exist. 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 in association with anterior cranial fossa mass lesions. These findings suggested foster kennedy syndrome, in which an anterior intracranial mass directly compresses the ipsilateral optic nerve.
First described in 1911, the foster kennedy syndrome also known as gowerspaton kennedy syndrome originates from a retrobulbar compressive optic neuropathy commonly caused by sphenoid wing meningioma, frontal lobe glioma, optic neuroglioma, olfactory glioma, chiasmal glioma, and craniopharyngioma. Robert foster kennedy dr robert foster kennedy md frse 18841952 was an irishborn neurologist largely working in america. Foster kennedy syndrome is a constellation of findings associated with tumors of the frontal. Foster kennedy syndrome definition of foster kennedy.
Foster kennedy syndrome is characterized by unilateral visual loss with a compressive optic atrophy in one eye and contralateral papilledema caused by increased intracranial pressure. Pdf on jan 23, 2019, simar rajan singh and others published foster kennedy syndrome find, read and cite all the research you need on. Pseudo foster kennedy syndrome is defined as onesided optic atrophy with papilledema in the. Neurologia foster kennedy syndrome diseases and disorders. A case of foster kennedy syndrome without frontal lobe or. Foster kennedy syndrome was first described in 1911 by the neurologist robert foster kennedy, who characterized the disorder as compression of one optic nerve by a subfrontal meningioma, resulting in optic nerve head pallor, with increased intracranial pressure causing contralateral optic nerve head edema. When the intracranial mass is large enough however to elevate intracranial pressure, contralateral papilledema results.
This syndrome is due to optic nerve compression, olfactory nerve compression, and increased intracranial pressure icp secondary to a space occupying lesion such as meningioma or plasmacytoma, usually an. Foster kennedy syndrome symptoms, causes, diagnosis, and treatment information for foster kennedy syndrome kennedy syndrome with alternative diagnoses, fulltext book chapters, misdiagnosis, research treatments, prevention, and prognosis. The classical description of this rare condition, fosterkennedy syndrome, consists of four elements. However, this entity should properly fall under the definition of pseudo foster kennedy syndrome. Foster kennedy syndrome describes the clinical syndrome of unilateral optic atrophy with contralateral papilledema caused by an ipsilateral compressive mass lesion. Please use one of the following formats to cite this article in your essay, paper or report. An aggressive sphenoid wing meningioma causing foster kennedy. Mar 18, 2008 in true foster kennedy syndrome unilateral disc swelling is caused by a tumour on the inferior surface of the frontal lobe, compressing the optic nerve on one side with papilloedema contralaterally.
Le rapport entre les 2 pathologies nest pas etabli. Although the increase in intracranial pressure correlates with the size and loca. Conditions which produce injury or dysfunction of the second cranial or optic nerve, which is generally considered a component of the central nervous system. Somewhat comically, pseudopseudopseudo foster kennedy syndrome has also been suggested as a descriptor for a mass lesion that causes indirect unilateral optic nerve compression 11. Foster kennedy syndrome represents the presence of a unilateral optic disc swelling with contralateral optic nerve atrophy, due to an intracranial mass. May 23, 2011 foster kennedy s syndrome fks is a rare neurological sign first described in 1911 by robert foster kennedy. This is the first report of the foster kennedy syndrome caused by this mechanism.
Intracranial meningiomatosis causing foster kennedy syndrome. True foster kennedy syndrome is very rare, and is typically caused by an olfactory groove meningioma. Foster kennedy syndrome radiology reference article. Read more about symptoms, diagnosis, treatment, complications, causes and. Foster kennedy syndrome was described in 1911 as an ophthalmologic manifestation of compression by a solid tumor in the frontal area with intracranial hypertension icht. Le traitement a consiste en une chimiotherapie adriamycine, decarbazine suivie d\une radiotherapie. A further note on the diagnostic value of retrobulbar neuritis in expanding lesions of the frontal lobes, with a report of this syndrome in a case of aneurysm of the right internal carotid artery. Foster kennedy syndrome, named after neurologist robert foster kennedy 18841952, describes ipsilateral optic atrophy with contralateral papilledema from an intracranial mass. Foster kennedy syndrome, a triad of optic atrophy in the ipsilateral eye, papilledema in the contralateral eye, and anosmia, is characteristically seen with these meningiomas. The foster kennedy syndrome originally was described as a meningioma of the olfactory groove may implicate the olfactory bulb and tract producing ipsiateral anosmia and then it extend posteriorly. True foster kennedy syndrome is the combination of papilledema in one eye and pallor in the other eye due to a large mass lesion causing compressive optic neuropathy and increasing intracranial pressure.
Pseudo foster kennedy syndrome due to unilateral optic nerve hypoplasia. Foster kennedy syndrome other names gowerspatonkennedy syndrome, kennedys phenomenon, kennedys syndrome frontal lobe on the right specialty neurology foster kennedy en. In the absence of an intracranial mass these findings may be labelled as pseudo foster kennedy syndrome, typically due to bilateral sequential. An atypical case of foster kennedy syndrome europe pmc. The same ophthalmoscopic features however can be seen in the pseudo foster kennedy syndrome. These findings suggested foster kennedy syn drome, in which an anterior intracranial mass directly compresses the ipsilateral optic nerve. A rare case of pseudo foster kennedy syndrome in a hiv.
Lifraumeni syndrome is a rare disorder that greatly increases the risk of developing several types of cancer, particularly in children and young adults the cancers most often associated with lifraumeni syndrome include breast cancer, a form of bone cancer called osteosarcoma, and cancers of soft tissues such as muscle called soft tissue sarcomas. Foster kennedys syndrome is a constellation of symptoms and signs associated with tumors of the frontal lobe of the brain. A large frontal lobe, olfactory groove, or medial third sphenoidal wing tumor, usually a meningioma, creates this syndrome. Foster kennedy 1911 was the first to consider the combination as a syndrome being patho gnomonic for an intracranial expanding lesion in the frontal lobes. Foster kennedy vs pseudo foster kennedy neuroophthalmology with dr. Foster kennedy syndrome is characterized by unilateral optic atrophy, central scotoma, anosmia, and contralateral disc edema kennedy, 1911. Although foster kennedy syndrome is equated with kennedy syndrome, it should not be confused with kennedy disease, which is named for w. Although robert foster kennedy noted it in 1911, william gowers had first described this constellation of symptoms 18 years before. Among the syndromes to which the student of neurology is quite early exposed is the foster kennedy syndrome or sign. The foster kennedy syndrome then became a triad consisting of ipsilateral optic atrophy, contralateral disc edema, and ipsilateral anosmia. Fosterkennedy syndrome an overview sciencedirect topics.
Dec, 2011 foster kennedy syndrome is thought to be present in 1% to 2. Pseudo foster kennedy syndrome is defined as onesided optic atrophy with papilledema in the other eye but with the absence of a mass. Damage to optic nerve fibers may occur at or near their origin in the retina, at the optic disk, or in the nerve, optic chiasm, optic tract, or lateral. Pdf pseudofoster kennedy syndrome due to unilateral. Diagnose and manage pseudofoster kennedy syndrome f. Hans, kennedy s syndrome stem cell treatment diary duration. Foster kennedy syndrome is a constellation of findings associated with tumors of the frontal lobe although foster kennedy syndrome is sometimes called kennedy syndrome, it should not be confused with kennedy disease, or spinal and bulbar muscular atrophy, which is named after william r. Neurologia foster kennedy syndrome free download as pdf file.
Foster kennedy syndrome jama neurology jama network. Foster kennedy syndrome is characterized by the compression of the ipsilateral optic nerve by an intracranial mass, often an anterior cranial fossa meningioma e. The differentiating and distinguishing features of both conditions are detailed below. Pseudofoster kennedy syndrome due to unilateral optic nerve. He gives his name to fosterkennedy syndrome, the kaplankennedy test and kennedys syndrome. Foster kennedy syndrome is thought to be present in 1% to 2.