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. Kennedy. Pseudo-Foster Kennedy syndrome is defined as one-sided optic atrophy with papilledema in the other eye but with the absence of a mass.

Presentation

The syndrome is defined as the following changes: *

optic atrophy Optic neuropathy is damage to the optic nerve from any cause. The optic nerve is a bundle of millions of fibers in the retina that sends visual signals to the brain. Damage and death of these nerve cells, or neurons, leads to characteristic featu ...

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) ipsilaterally This syndrome is due to optic nerve compression, olfactory nerve compression, and increased

intracranial pressure Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury (mmHg) and at rest, is normally 7–15 Millimeter of mercury, mmHg ...

(ICP) secondary to a mass (such as

meningioma Meningioma, also known as meningeal tumor, is typically a slow-growing tumor that forms from the meninges, the membranous layers surrounding the brain and spinal cord. Symptoms depend on the location and occur as a result of the tumor pressing o ...

or plasmacytoma, usually an olfactory groove meningioma). There are other symptoms present in some cases such as

nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. While not painful, it can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the ...

and

vomiting Vomiting (also known as emesis and throwing up) is the involuntary, forceful expulsion of the contents of one's stomach through the mouth and sometimes the Human nose, nose. Vomiting can be the result of ailments like Food-poisoning, foo ...

memory loss Amnesia is a deficit in memory caused by brain damage or disease,Gazzaniga, M., Ivry, R., & Mangun, G. (2009) Cognitive Neuroscience: The biology of the mind. New York: W.W. Norton & Company. but it can also be caused temporarily by the use o ...

and emotional lability (i.e., frontal lobe signs).

Diagnosis

Brain tumor can be visualized very well on CT scan, but MRI gives better detail and is the preferred study. Clinical localization of brain tumors may be possible by virtue of specific neurologic deficits or symptom patterns. 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.

Treatment

The treatment, and therefore prognosis, varies depending upon the underlying tumour. While awaiting surgical removal, treat any increased intracranial pressure with high-dose steroids (i.e., dexamethasone).

History

The syndrome was first extensively noted by

Robert Foster Kennedy Dr Robert Foster Kennedy MD FRSE (7 February 1884 – 1952) was an Irish-born neurologist largely working in America. He gives his name to Foster-Kennedy syndrome, the Kaplan-Kennedy test and Kennedy's Syndrome. He was one of the first medical d ...

in 1911, an Irish neurologist, who spent most of his career working in the

United States of America The United States of America (U.S.A. or USA), commonly known as the United States (U.S. or US) or America, is a country primarily located in North America. It consists of 50 states, a federal district, five major unincorporated territo ...

. However, the first mention of the syndrome came from a William Gowers in 1893. Schultz–Zehden described the symptoms again in 1905. A later description was written by Wilhelm Uhthoff in 1915.

References

External links

{{DEFAULTSORT:Foster Kennedy Syndrome Eye diseases Neurological disorders Syndromes affecting the nervous system Syndromes affecting the eye Syndromes with tumors