Coats' Disease

*What is Coats' Disease?*

Coats' Disease, also known as Exudative Retinitis, is a rare condition that occurs in children and young adults, usually males. It is a progressive condition of abnormal development of the retinal capillaries that usually occurs in the first 10 years of life. It is a gradual process that affects central vision, usually just in 1 eye. The retinal capillaries nourish the retina, which is where light is converted into electrical signals. These signals pass along the optic nerve and to the brain where they are converted into sight. There are no known causes for Coats' Disease at this time.

*Symptoms*

The main symptom of Coats' Disease is loss of central or peripheral vision. Other symptoms include sensitivity to light, cloudy vision, and a white patch on the pupil. This is a picture of a young boy with Coats' Disease. Notice the yellowish color of his right pupil. This is caused by a build up of cholesterol in the retinal capillaries leaking into his eye.

http://www.daisyseyecancerfund.org/Photos/Symptoms/coats1.gif

*Treatment*

If it is caught early enough, vision to the affected eye may be saved. Many times the children are not able to communicate their problems or may not notice them soon enough. Their are 5 different stages that Coats' Disease is broken into.

~Stage 1: There is a high probably that vision can be saved if caught in this stage, although as mentioned earlier, the child typically will not be able to understand what is going on at the time. Although catching Coats' Disease in this stage is unlikely, it can be treated with Laser Therapy if it is caught.

~Stage 2: There is a good chance that vision can be restored in this stage, depending on if the fovea has been affected or not. The treatment during this stage is Laser Therapy or Cryotherapy. Cryotherapy uses extreme cold to freeze and destroy dead tissue.

~Stage 3: In this stage the retina has detached and Laser Therapy or Cryotherapy can still be used. Cryotherapy may be more effective here if the detachment is shallow. If the detachment is more advanced surgery may be required.

~Stage 4: There is not really any chance of vision recovery in this stage, but laser therapy would be the only attempt to stop the leakage. Many times if the pain is severe enough, removal of the eye may be necessary.

~Stage 5: This stage results in total blindness that is irreversible. 

This is an Axial CT slice showing total retinal detachment in the left eye.

http://download.imaging.consult.com/ic/images/S1933033207752692/gr1-midi.jpg

Prolactinoma

*What is Prolactinoma?*

The pituitary is a small gland in the endocrine system located at the base of the brain and sits in the sella turcica. Don't let its small size fool you though! It influences nearly every part of your body! Some of the things that it helps regulate are growth, reproduction, and blood pressure. The pituitary also produces a hormone called Prolactin which stimulates breast milk production in females, but this type of tumor can also occur in men. Although the cause of pituitary tumors is yet unknown, Prolactinoma is the most common type of pituitary tumor. These tumors are almost always noncancerous, but they can increase in size. They tend to occur more commonly in women rather than men, and they are very rare for children. People with Prolactinomas tend to develop them under 40 years of age, but they tend to develop in younger ages for women and older ages for men.

*Symptoms*

The symptoms of Prolactinoma result from excess prolactin in the blood and from pressure on the surrounding tissues. Because the area that the pituitary is housed in is so small, when the tumor grows it can compress your optic nerve and cause visual disturbances. Other symptoms that can occur in both males and females are headaches and infertility. In women, missed or irregular menstrual cycles or  production of breast milk when not pregnant or breast feeding are also common symptoms. In men, erectile dysfunction and uncommonly enlarged breasts are key symptoms. Women usually tend to notice a problem earlier than men because of the missed or irregular menstrual cycles, whereas men don't really seem to notice until the tumor gets much larger and presses on the optic nerve causing headaches and vision problems.

*Diagnosis*

The most sensitive study available to detect a pituitary tumor is MRI, although a CT scan can also be done. Also, your doctor may test prolactin levels in your blood or he may also perform a vision test to see if the growth of the tumor has impaired your vision.

*Treatment*

The 2 main treatments for Prolactinoma are medication or surgery. Medication is usually a long-term process that reduces the levels of prolactin in the blood and may also shrink the tumor. If medication shrinks the tumor and your prolactin levels remain at a normal level, you may be able to stop taking the medication. Surgery is typically done if the medication is not successful or is not tolerated by the patient. It may also be necessary to relieve pressure on the optic nerves, in which case removal of the tumor may be necessary. The most common surgery performed is removing the tumor through the nasal cavity.

This is a sagittal T1 weighted MRI image of a Prolactinoma in a 27-year-old female who was 32 weeks pregnant. She complained of headaches and vision problems.

. 

http://radiographics.rsnajnls.org/cgi/content-nw/full/27/1/95/F12B

This is the coronal T1 weighted MRI image of the same 27-year-old female.

http://radiographics.rsnajnls.org/cgi/content-nw/full/27/1/95/F12A

Ramsay Hunt Syndrome

*What is Ramsay Hunt Syndrome?*

Ramsay Hunt Syndrome is an infection of your facial nerve, which is also known as the 7th cranial nerve. It is caused from the varicella-zoster virus, which is the same virus that causes chicken pox. Once you have had the chicken pox, the virus lies dormant in your nervous system. If the virus is not destroyed after the initial infection, chicken pox, it can return and affect your facial nerve, Ramsay Hunt Syndrome, or it can cause other disorders including shingles. Anyone who has had chicken pox can have this disorder, but it is most commonly seen in people over 60. It is very rare for this to occur in children. This disorder is not contagious, although the reactivation of the varicella-zoster virus can cause chicken pox in individuals that have not previously had them.

*Symptoms*

A classic symptom of Ramsay Hunt Syndrome is a painful red rash with fluid-filled blisters. These can occur in various places including the outside of your ear, the roof of your mouth, your eardrum, your external ear canal, or on your tongue. People who experience the onset of this disorder tend to think they are having a stroke because facial weakness and paralysis is another common effect. Other symptoms are ear pain, hearing loss, vertigo, ringing in the affected ear(tinnitus), and difficulty closing one eye.

This is a picture of an individual with facial weakness from experiencing Ramsay Hunt Syndrome.

http://www.mayoclinic.com/health/ramsay-hunt-syndrome/DS00878/DSECTION=symptoms

*Diagnosis*

Ramsay Hunt Syndrome is diagnosed by a medical history evaluation and a physical exam. The doctor may also draw fluid from the blisters to confirm the diagnosis.

*Treatment*

The treatment for Ramsay Hunt Syndrome is an antiviral medication, among other medicines for the other things this disorder causes. Therapy may be prescribed to help regain control of the facial muscles. Recovery may take anywhere from several weeks to several months, but if the reaction is severe, full recovery may not be possible.

These are both MRI images with one taken before Gadolinium and one taken after Gadolinium. Notice the enhancement of the cranial nerves on the right side. This, along with the symptoms of the patient, led to the diagnosis of Ramsay Hunt Syndrome.

http://home.flash.net/~drrad/tf/052796a.jpg

Epilepsy

The reason I chose this pathology is because my boyfriend's younger brother was just recently diagnosed with this. I wanted to research it and find out more information on what this actual is. 

*What is Epilepsy?*

Epilepsy is a fairly common disease that affects the nervous system and occurs in about 2.5 million Americans. It is most commonly seen affecting children and teens with about 180,000 diagnosed every year. 

The brain is run by electrical signals. When these signals misfire, there is a temporary communication problem between nerve cells. Some of the things that can lead to epilepsy are brain injuries, brain tumors, lack of oxygen during birth, and an infection or illness the fetus acquires during pregnancy. 

 

*Symptoms* 

The most common symptom of epilepsy is seizures, but not everyone that has seizures has epilepsy. Seizures can occur in anyone under certain conditions, but epilepsy is characterized by repeated seizures. They are typically the same type of seizure each time they occur, so the affected person will probably act similar during each one. When a seizure occurs the person can lose consciousness, become unaware of what is going on around them, or have involuntary motions, among other things. This will leave the person very weak and very tired. My boyfriend's brother has a couple different strengths of seizures. Sometimes he will be going upstairs to brush his teeth and stop halfway there. His mom will go to check on him a couple minutes later and find him standing in the hallway and she will ask him what he is doing. Most times he has no idea what he was supposed to be doing, so his mom has to remind him that he was supposed to be going to brush his teeth. Other times his seizure is stronger. This usually leaves him extremely tired and causes him to miss school for the day.

*Treatment*

There are a few ways in which doctors can treat this disease. The most common way is through medication to control seizures. The medication won't make the seizures stop, but they usually fade out or even disappear as the child gets older. Another way is to implant a Vagus Nerve Stimulator underneath the skin in the chest. A wire is run up through the neck where it attaches to the vagus nerve. This device stimulates the nerve to reduce seizure frequency and intensity. If the person has partial seizures, a third treatment is surgery to remove the part of the brain where the seizures occur.

As we are all aware, this is a T2 weighted MRI image of a normal brain.

http://www.indianradiologist.com/neuroradiology24.htm

This is a T2 weighted MRI image of a 3-year-old with right sided hemimegalencephaly, enlargement of one side of the brain, which leads to seizures.

http://www.childrensmemorial.org/cme/online/article.asp?articleID=103