Craniopharyngioma

Craniopharyngioma is a looooong word, but this is the pathology I chose to do! Whoo hoo!
Ok, so basically this is a tumor that develops most commonly in childhood and adolescence. If not then, it could also occur in adults over fifty. So hopefully we are all ok for now. These tumors make up 2-4% of all cranial tumors. Apparently they can get HUG, up to 3 cms to be a little more exact. There are not usually caught until they start to put pressure on other structures. These structures are then affected making the idea of a tumor plausible. Eyesight might be affected for example because the optic nerve lies just anterior to where the pituitary gland lies. Researches think these tumors might arise from embryonic cells from an imperfect involuted anterior pituitary gland; these cells eventually from the craniopharyngioma. They are benign, so the greatest damage is how they affect the structures around it, such as optic chiasm, arteries within the cranium and even the brain itself.

Symptons of this tumor really vary, depending on what structures it’s putting pressure on. If it is putting pressure on the pituitary gland itself, hormone deficiency would most likely follow, which in turn might affect growth depending on your stage in life. If it’s affecting the pituitary stalk diabetes could develop along with milk coming from the breast. More common symptons include vomiting, personality changes, and confusion.

Diagnoses can really only be made with a CT or MRI of the pituitary gland with and without contrast.

Treatment is almost always surgery. The goal is to get it out, while keeping the other structures fully intact. Whenever they can’t fully remove it, radiation therapy is an option to control the size of the tumor. CTs and MRIs should be done yearly after the surgery to ensure the tumor has not grown back.

Below is a coronal CT post contrast. Notice the bright appearance of the tumor.
https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjIt-_8_gOqP77872PywczgP_BN3W1QgMpuQzosLQ60FK89Ko61_YRvcKhd2ihvzT8J2g8ef649OIxxBIBbXKxJ5SfG8gJV4PZk_TLsMQJON7XjfSF9Q0pD3ii9eDV9BSCfU4qabQqQz2kN/s320/cranio.jpg


Below is an MRI T1 sagittal post contrast view of a childs brain.
bp0.blogger.com/.../ZVpdGq_3FeU/s320/cranio.jpg

Ramsey Hunt Syndrome!!!

Soo, Ramsey Hunt Syndrome involves the facial nerve, which we know is also known as the 7th cranial nerve, located just anterior and inferior to the pons. This nerve controls facial muscles and taste sensation. Basically you get Ramsey Hunt Syndrome by the chicken pox virus. After the symptons of chicken pox of subsided the virus itself, the varicella-zoster virus lies dormant within the nerves. There is no certain amount of years that triggers the virus, but it is most common in people over 60. In time the virus reactivates itself and infects the nerve, which causes the pathology. This virus is actually related to the herpes family of viruses, which cause sores on the mouth and genital herpes. It is also the same virus that causes shingles.

Symptoms of this pathology are fairly visible. A couple that stand out in my mind are painful red rash on your tympanic membrane, your external acoustic canal, and the roof of your mouth. You might also experience muscle weakness in your face. The palsy will be on the affected side of the ear. A constant feeling of unbalance (vertigo), change in taste, or tinnitus might also be symptons. The earlier you recognize these symptons as a problem the better. The earlier you catch it, the lesser chance you have of long-term conditions. Within seven days ensures the highest probability of a healthy recovery.

Doctors can most of the time be able to diagnose the pathology with the history of the patient. They may also may take fluid samples of blisters, or a blood test, though the fluid from the blisters is a more accurate diagnosis.

If the pathology is caught early enough, medications are given to fight the infection of the virus. Antiviral medications, corticosteroids to fight swelling and pain, diazepam for vertigo, or pain relievers are given. If a person suffers from facial palsy, physical therapy would be offered to regain strength in those facial muscles. The success rate of treatment depends on how far Ramsey Hunt Syndrome is progressed. There’s really nothing one can do to prevent the disease from coming on, but there are ways to try and lessen the discomfort. Make sure to keep the rash clean, apply cold compress to the rash, and keep good oral hygiene.


Below is a picture from a sagittal view of where the facial nerve is located in the head. It also give you an idea of how extentsive the nerve branches out into the face.
http://www.mayoclinic.com/health/ramsay-hunt-syndrome/DS00878

Below is a T1 axial MRI view of an enlarged right facial nerve. Notice the difference from the right and the left nerves. The right is clearly visible over the left.

http://images.google.com/imgres?imgurl=http://www.medscape.com/content/2001/00/40/58/405875/art-fp1405.10.fig1.jpg&imgrefurl=http://medgenmed.medscape.com/viewarticle/405875_print&usg=__yPd_TCX458RjFh54tCAGfeuI66c=&h=334&w=400&sz=28&hl=en&start=1&tbnid=fsrLF3ZujstMeM:&tbnh=104&tbnw=124&prev=/images%3Fq%3Dmri%2Bof%2Bramsey%2Bhunt%2Bsymdrome%26gbv%3D2%26hl%3Den%26sa%3DG

Leigh's Disease

Ok so I researched a pathology by the name of Leigh's disease. To be quite honest, the reason why this pathology caught my interest is because my middle name is Leigh! Anyway, from what I understand Leighs disease affects the nerves in your brain, it affects how they function, which obviously makes this a fatal disease. MOST the time the disease will show itself within toddler stages; 6 months to two years old. Rarely the disease can begin in teenage to young adult years, but it is possible.

What happens is the nerve cells die because the mitochondria (powerhouse of each cell) is affected. Some symptons that I think are unique to this disease are a childs sucking inablity, constant crying, and lack of motor skills.More obvious symptons are seizures and vomitting. Lacitic acidosis, which is lactic acid built up in the brain is a sympton as well. This leads to kidney failure.

Unforunately there is no cure for Leigh's disease. The best medicine can do right now is the use of vitamin B-1. Vitamin B-1 is what nerve cells require to maintain function.
It is quickly degenerative. Since mitochondrian ablilities inherited from the mother,Leigh's disease is also inherited from the mothers side by a DNA mutation. Most do not live to their teens, although it is possible. Though the appearance of Leigh's disease is not strikingly obvious, it is pictured below.




http://www.ispub.com/xml/journals/ijra/vol3n1/mri-fig2.jpg
Ok here is a normal T1 weighted image of a brain. Notice the size of the gyrus, ventricles, and cerebellum.

http://rad.usuhs.mil/medpix/tachy_pics/thumb/synpic39123.jpg

I apolgize about the blurriness but this is a T1 weighted image of a child with Leigh's disease. Although the difference is not dramatic, there is volume loss in the cerebellum and gyri. The ventricles are also abnormally larger.

http://healthlink.mcw.edu/article/921774134.html

http://www.bbc.co.uk/health/conditions/leigh1.shtml