Foster Kennedy syndrome is a constellation of findings associated with tumors of the

frontal lobe The frontal lobe is the largest of the four major lobes of the brain in mammals, and is located at the front of each cerebral hemisphere (in front of the parietal lobe and the temporal lobe). It is parted from the parietal lobe by a Sulcus (neur ...

. Although Foster Kennedy syndrome is sometimes called "Kennedy syndrome", it should not be confused with Kennedy disease, or

spinal and bulbar muscular atrophy Spinal and bulbar muscular atrophy (SBMA), popularly known as Kennedy's disease, is a rare, adult-onset, X-linked recessive lower motor neuron disease caused by trinucleotide CAG repeat expansions in exon 1 of the androgen receptor (AR) gene, whi ...

, which is named after William R. Kennedy. Pseudo-Foster Kennedy syndrome is defined as one-sided optic atrophy with

papilledema Papilledema or papilloedema is optic disc swelling that is caused by increased intracranial pressure due to any cause. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare. In ...

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 A scotoma is an area of partial alteration in the field of vision consisting of a partially diminished or entirely degenerated visual acuity that is surrounded by a field of normal – or relatively well-preserved – vision. Every normal mamm ...

(loss of vision in the middle of the visual fields) in the ipsilateral eye The presence of

anosmia Anosmia, also known as smell blindness, is the lack of ability to detect one or more smells. Anosmia may be temporary or permanent. It differs from hyposmia, which is a decreased sensitivity to some or all smells. Anosmia can be categorized int ...

(loss of smell) ipsilateral to the eye demonstrating optic atrophy was historically associated with this syndrome, but is now understood to not strictly be associated with all cases. 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 mmHg for a supine adu ...

(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 ...

plasmacytoma Plasmacytoma is a plasma cell dyscrasia in which a plasma cell tumour grows within soft tissue or within the axial skeleton. The International Myeloma Working Group lists three types: Plasma cell dyscrasia#Solitary plasmacytoma, solitary plasma ...

, 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. It can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the throat. Over 30 d ...

and

vomiting Vomiting (also known as emesis, puking and throwing up) is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis, pre ...

memory loss Amnesia is a deficit in memory caused by brain damage or brain diseases,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 temporarily caused by t ...

and

emotional lability In medicine and psychology, emotional lability is a Medical sign, sign or symptom typified by exaggerated changes in mood or affect (psychology), affect in quick succession. Sometimes the emotions expressed outwardly are very different from how th ...

(i.e.,

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, and papilledema of the contralateral eye;

on the side of the tumor may be found in certain cases of progressive disease.

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 Neurology (from , "string, nerve" and the suffix -logia, "study of") is the branch of medicine dealing with the diagnosis and treatment of all categories of conditions and disease involving the nervous system, which comprises the brain, the ...

, who spent most of his career working in the

United States of America The United States of America (USA), also known as the United States (U.S.) or America, is a country primarily located in North America. It is a federal republic of 50 states and a federal capital district, Washington, D.C. The 48 contiguo ...

. 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