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 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 syndrome is due to optic nerve compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary to a mass (such as meningioma or plasmacytoma, usually an olfactory groove meningioma). There are other symptoms present in some cases such as nausea and vomiting, memory loss 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).
Because the disease appears to be inherited in an autosomal recessive pattern, detection of low-sulfur brittle hair syndrome is also important for genetic counseling. Genetics
Sabinas brittle hair syndrome is inherited as an autosomal recessive genetic trait.In a study by Howell et al. patients were located and studied by means of complete histories and physical examinations, analyses of serum trace metals, ceruloplasmin concentration, urine and serum amino acids, and routine metabolic urine screens. In addition, serum and urine luteinizing hormone (LH) and follicle-stimulating hormone (FSH) values were determined, and were interpreted in conjunction with total plasma estrogen, estradiol, and testosterone levels. Close examination demonstrated the scalp hairs were very brittle, coarse, wiry in texture, and broke off quite easily with mechanical trauma such as combing and brushing. Some hairs could be visualized in their follicles, which were broken off at the skin line. Most patients had accompanying hyperkeratosis (thickening of the skin) of moderate degree on exposed surfaces. Maxillary hypoplasia (midfacial retrusion) was significant in many patients. The brittle, short hair, reduced eyelashes, crowded teeth, and dull appearance created a characteristic facial appearance. Post-pubertal patients had development of secondary sexual characteristics consistent with their age, except for sparse pubic escutcheons. All cases studied demonstrated some degree of mental deficiency; I.Q.s ranged between 50 and 60. A deficiency in eye–hand coordination was also noted. Diagnosis
The easiest way to test for this condition is through genetic testing.