Brown's Syndrome

Brown's Syndrome is characterized by defects and errors in eye movement.  This disease may be congential or secondary from inflammation.  Malfunction of the superior orbital tendon causes these defects in eye movement, especially when adducting the eye upward.  It is thought that about 35% of patients with Brown's Syndrome has a family member with Brown's Syndrome.  It is thought then, that Brown's Syndrome could be a genetic trait.  This syndrome is mostly found in women and then only in the right eye. 

Some medical treatments for this syndrome are an antinflammatory such as ibuprofen.  Another form of treatment is a steriod injection.  However, surgical treatment is the best way to treat this syndrome.  A tenotomy is performed and medical grade silicone 240 retinal band, this is the most effective form of treatment.  Other surgical procedures include:  superior oblique split tendon lengthening, tenotomy, and superior oblique recession.  Facial reconstruction is also an option but it is not recommended for its low success rate.  However, in some cases the best way to treat this syndrome is to do nothing at all!

Temporal Bone Disease

What do you notice that is different about these images??  If you look closely at the arrows you will notice calcifications that should not be there.  This is known as tympanosclerosis and by definition it is the formation of dense connective tissue in the middle ear, often resulting in hearing loss when the ossicles are involved.  Tympanosclerosis is also known as myrinosclerosis or intratympanic tympanosclerosis.  Myringosclerosis is classified by calcificaton only withing the tympanic membrane and and intratympanic tympanosclerosis is classified by calcification of any other structure in the middle ear, namely the ossicular chain, middle ear mucosa, or  mastoid cavity.  These diseases rarely result in any symptoms however, tympanoscerlosis can result in hearing loss or chalky white patches in the middle ear or temporal membrane.  The exact cause of tympanosclersis is not completely understood but some probable factors are:  long term otitis media, insertion of tympanostomy tubes, and atherosclerosis.  Treatment for this disease can be hearing aids to treat the associated hearing loss or surgery.  This involves removal of the affected areas and repair to the ossicular chain. Results are variable and can sometimes result in damage to the inner ear.

Creutzfeldt Jakob Disease

The above image is an actual brain with Creutzfeldt Jakob Diseae.  Notice the large holes throughout the tissues!!

Creutzfeldt Jakob disease is a degenerative brain disorder that can lead to dementia and eventually death.  This disease can resemble some dementia like brain disorder.  However this disease will progress more rapidly than any type of dementia.  In the 1990's this disease was on the rise in the United Kingdom.  Individuals developed a form of this disease callled variant CJD.  It was discovered that cattle had also contracted a form of this disease and the individuals most like contracted CJD from eating diseased meat.  Fortunatly this disease is very rare with only one out of 1 million people being diagnosed each year. 

CJD has numerous symptoms and is therefore hard to diagnose; symptoms include:  personality changes, anxiety, depression, memory loss, impaired thinking, blurred vision, insomnia, difficulty speaking and swallowing, and sudden jerky movements.  As the disease progresses the mental symptoms may progress.  CJD will usually cause the patient to go into a coma and heart failure, respitory failure, pneumonia, or other infections will likely be the actual cause of death.  The estimated survival time is about 7 months. 

CJD is caused by a varing group of both human and animal diseases known as transmissible spongiform encepholopathies.  The disease name comes from the multiple holes that form in brain tissue during the progression of the disease.  CJD is transmitted three ways.  The majority of cases occur spontaneously with no clear reason for its development; this accounts for most of the diagnosed cases.  Genetic mutation can also cause this disease as well as family history.  This accounts for 5-10% of the cases.  Lastly, individuals can be exposed by contamination of other human body tissue, or by eating contaminated meat from cattle with "mad cow disease".