Transverse Myelitis

What is it?
Transverse myelitis (TM) is the inflammation across a segment of the spinal cord, damaging and/or destroying the myelin surrounding the nerve cell fibers. It is referred to as a neurological disorder since it affects and spinal cord and the nervous system's ability to communicate with the rest of the body. Each year in the US, approximately 1,400 new cases of transverse myelitis are reported. It is estimated that 33,000 Americans have some disorder associated with the disease. Recovery from the disorder usually occurs within 2 to 12 weeks from the onset of symptoms and can continue up to 2 years. If there is no improvement within 3 to 6 months, recovery is unlikely.






What causes it?
The exact cause of the disease is unknown, and it can occur in any age, gender, or race. It may result from abnormal immune reactions, insufficient blood flow through the vessels, or viral infections (HIV, hepatitis A, influena, etc). Transverse myelitis also may occur as a consequence of syphilis, measles, or Lyme disease. Some vaccinations, such as those for chickenpox and rabies, have been associated with the disorder. Some researchers even believe that it is an autoimmune disease because it has been shown in persons with other autoimmune disorders. Spinal AVMs and vascular diseases have also been indicative of causing transverse myelitis. Hemorrhage within the spinal cord and blockage of a blood vessel causes ischemia to the spinal cord. When the spinal cord does not get the proper oxygen and nutrients it needs, inflammation can occur, resulting in TM.


What are the symptoms?
Pain is the most common presenting symptom of transverse myelitis. The pain can be localized to the lower back or it can radiate down the legs or arms or around the torso. The other symptoms can include muscle spasms, sensory loss, and partial paralysis of the legs that can progress to paraplegia. Sudden sensations such as burning, tickling, pricking, or tingling in the legs and sometimes arms are also noted. Bladder and bowel dysfunction is commonly seen in some cases. Respiratory problems may occur depending on the segment of the spinal cord.


How is it diagnosed?
Neurological exams are performed to first indicate if the problems are neurological. Blood tests them may be given to rule out other disease. A myelogram may be requested, but most commonly a MRI will be performed to check for inflammation and location.




How is it treated?
There is no cure for transverse myelitis. Treatment focuses on alleviating pain and symptoms that result from the stages and severity of the disorder. Corticosteriod therapy is usually first used to decrease inflammation. Analgesics are oftem prescribed to reduce pain. Physical therapy is used when the stages of paralysis infest. Some patients are commonly treated in rehabilitation homes when the paralysis becomes paraplegia.


References:

http://www.ninds.nih.gov/disorders/transversemyelitis/detail_transversemyelitis.htm

http://www.uiowa.edu/~c064s01/nr187.htm

http://www.nature.com/sc/journal/v44/n11/fig_tab/3101879f1.html

Bow Hunter's Syndrome

What is it?

Bow Hunter's Syndrome, most commonly referred to as just Hunter's Syndrome, is an uncommon occlusion of one or both of the arteries supplying blood to the back of the brain. The arteries at the level of C1-C2 supplying blood to the brainstem and cerebellum can become narrowed or obstructed, resulting in reduced blood flow to the posterior brain; so it is known to be a type of ischemic stroke. It usually results when a person turns their head to one side by force and the vertebral arteries in the posterior upper neck become symptomatically blocked.

How is it diagnosed?

Physicians can determine if a person may have Bow Hunter's syndrome if the patient has problems when rotating their head in one or both directions. Problems can include vertigo and a tingling feeling in the neck and arms. Radiologic exams normally indicated for Hunter's syndrome include an angiogram, CT or MRI of the brain and/or cervical spine, or an MRA.

How is it treated?

Treatment for Hunter's syndrome focuses on relieving strain and pressure of the vertebral arteries when moving the head. Most commonly, surgery or a neck brace is indicated.

References:

http://www.revolutionhealth.com/articles/bow-hunters-stroke-what-causes-it/B3A81F0D-C0CD-4A31-898048E0C65EFD34?ipc=B00448

http://journals.lww.com/neurosurgery/Abstract/1997/12000/Bow_Hunter_s_Stroke_Caused_by_a_Nondominant.30.aspx

Sjogren's Syndrome

An estimated four million Americans are living with Sjogren's syndrome today. Half the time it can also be associated with other connective tissue disorders (rheumatoid arthritis, scleroderma, etc.) in the body, and this kind is called secondary Sjogren's sydrome. When the disease doesn't correlate with a connective tissue disorder is it referred to as primary Sjogren's syndrome.

What is it?
Sjogren's syndrome is a widespread autoimmune disorder in which a person's moisture-producing glands are attacked by white blood cells. Such glands are the lacrimal glands that produce tears and salivary and parotid glands that produce saliva for the mouth. The cause of the disorder is unknown, but it is commonly found in people with other connective tissue disorders. Another factor that can increase the chances of having it are if another family member has it, as it is suggested that it may be inherited. Also, approximately nine out of ten people with the disease are female.

What are the symptoms?
The symptoms of Sjogren's can vary from person to person. Common signs are difficulty talking/chewing/swallowing, a sore or cracked tongue, and dry throat. A change in taste or smell, increased dental decay, vaginal and skin dryness, digestive problems, and dry nose are other signs. Reduction in the body's ability to produce liquids leads to dry eyes, not enough water for tears, dry mouth, and dry lips. It is also known to cause organ dysfunction such as the kidneys, GI system, lungs, liver, pancreas, and the CNS. It can lead to extreme fatigue and joint pain and a person who has Sjogren's has a higher risk of developing lymphoma. Inflammation of the vessels is less common but a more serious symptom.

How is it diagnosed?
There is no one test that will verify if a person has the disorder. Symptoms can mimic other diseases so a series of tests are needed. Diagnosis is often difficult and it is often after 6.5 years from the onset of symptoms that it might be diagnosed. A visit to the doctor's office where lab work is taken is most common for determining Sjogren's. There they will do tests such as those for ANA (antinuclear antibody), RF (rheumatoid factor), ESR (erythrocyte sedimentation rate), and immunoglobulin counts. Tests at the opthalmogist office include the Schirmer test (for tear production) and Rose Bengal and Lissamine Green test (eye drops used to see dry spots on eye). Dental tests include those measuring saliva production, salivary scintigraphy (a nuclear medicine test measuring gland function), and biopsy of the salivary gland.

How is it treated?

Currently there is no treatment that will cure Sjogren's syndrome. Treatment focuses on relieving the symptoms of the disorder to make the person more comfortable and able to live life fully, and also to prevent complications that may arise or symptoms that may worsen. OTC or prescription drugs and drops are available for the eyes and mouth. Immunosuppressive medications are sometimes prescribed to treat internal organ manifestations for people having organ problems. The vasculitis that sometimes arises is treated with cortisone or prednisone.

References:

http://www.medicinenet.com/sjogrens_syndrome/article.htm

http://www.sjogrens.org/home/about-sjogrens-syndrome/diagnosis

http://imaging.consult.com/imageSearch?query=found&qyType=AND&global_search=Search&modality=Computed%20Tomography(CT)&thes=false&normalVariantImage=false&groupByNode=none&anatomicRegion=

http://dmfr.birjournals.org/cgi/figsearch?FIRSTINDEX=425&SEARCHID=1&hits=25&RESULTFORMAT=&resourcetype=HWFIG

Moyamoya Disease

What is it?
Moyamoya disease is a cerebrovascular disorder in which the arteries of the base of the brain, in an area referred to as the basal ganglia, are blocked. The disease was first discovered in the 1960s in Japan, but cases have now been found in many places in the world including America. The term "moyamoya" means "puff of smoke" in Japanese, which refers to how the tiny vessels formed look as they try to balance the blockage. It is most commonly found in children, but cases have been found in adults also. Moyamoya disease has been found to run in families, so researchers think that it may be a result of abnormalites in genes.

What are the symptoms?
The beginning symptoms of Moyamoya disease are stroke, recurrent TIAs (mini-strokes) commonly accompanied by muscular weakness/paralysis affecting one side of the body, or seizures. Older victims of the disease often experience a hemorrhagic stroke from recurring blood clots in the brain vessels. Other symptoms include problems with consciousness and vision, speech deficits (aphasia), sensory and cognitive impairments, and involuntary movements.

What are some treatments?
Surgery involving revascularization (opening narrowed blood vessels or bypassing blocked vessels in the brain) to restore blood flow is the most common treatment. Surgery is usually more successful in children than adults, but after surgery the symptoms tend to go away. However, without surgery, the arteries continue to progressively narrow and the majority of individuals will experience mental decline and multiple strokes; some can experience results as fatal as an intracerebral hemorrhage.


References:
http://www.ninds.nih.gov/disorders/moyamoya/moyamoya.htm

Sinusitis

Sinusitis is the inflammation of any of the sinus cavities, commonly as a result of bacterial infection. It can also be caused by allergies, pollutants in the air, and nasal problems. It is estimated that one in three people will develop sinusitis at one point in their lives. In 2003, studies showed that approximately thirty-five million people in the US had sinusitis. There are two kinds of sinusitis which differ greatly in symptoms and treatment: acute, lasting for less than three or four weeks, or chronic, which lasts much longer. Ethmoid and maxillary sinusitis usually occur more often than sphenoid and frontal sinusitis, which usually aren't seen in children because those sinuses develop later in life.

Acute sinusitis symptoms include those of a cold, such as headache, cough, fatigue, and weakness. Fever, nasal congestion, and pus-like fluid from the nose can also accompany those symptoms. Chronic sinusitis has symptoms that vary and are more difficult to distinguish and can include excessive mucus discharge from the nose (rhinorrhea), mucus draining to the back of the throat (postnasal drip) varying in color, dizziness, and earaches. Physicians usually determine sinusitis if the nasal mucosa is swollen, the posterior pharynx is inflamed (due to post nasal drip), and if pus is draining from the middle meatus.

Diagnosis relies on a physical examination by the physician. It can involve the doctor taking a culture to determine which bacteria is present in order to figure out how the infection can be fought. A procedure known as a fiberoptic nasal endoscopy is sometimes used. X-rays can be used to make the diagnosis of acute sinusitis in the maxillary and sphenoid sinuses, but CT scans are most often used because they can visualize all of the sinuses.

Treatments of sinusitis include antibiotics (amoxicillin and erythromycin) that are taken until the symptoms are gone for at least one week. Decongestants, pain relievers, saline nasal sprays and vaporizers are also sometimes used to relieve pressure. It is estimated that each year 300,000 people will refer to surgery to get rid of their sinusitis when all other treatments have failed. Functional endoscopic sinus surgery (FESS) is a minimal suregery where the sinuses can sometimes be removed. The Calwell-Luc procedure is done by stripping the maxillary sinuc mucosa and creating new ostium in the inferior meatus by entering the maxillary sinus through the palate or below the upper lip.

https://ssl.search.live.com/health/article.aspx?id=articles%2fmlp%2fpages%2f2%2fSinusitis.htm&q=Sinusitis

http://www.ent-consult.com/sinus_lay.html

http://medgenmed.medscape.com/viewarticle/408707_print

Optic Nerve Meningioma

What is it?

Optic meningioma (also optic nerve meningioma, optic nerve sheath meningioma, and orbital meningioma) is a slow-growing tumor of the optic nerve covering, or meninge. It is a benign neoplasm that can be located in the brain behind the orbit. It normally is identified in adults, most frequently in middle-aged women, and can cause severe, progressive visual loss.

What are the symptoms?

A person with this type of tumor normally has proptosis (the tumor pushes the eye forward so it bulges). When the optic nerve is compressed it can cause optociliary shunt vessels to form, leading to loss of vision. The effects of the tumor depend on the size, location, and degree to which it interferes with the optic nerve and other brain functions. Symptoms can also be double vision and drooping of the eyelid (ptosis).

What is the treatment?

Diagnosis of optic meningioma involves viewing the optic nerve blood vessels, which appears raised if disrupted, and for dilated retinal veins. Ultrasonography is sometimes used to measure the diameter of the optic nerve sheath, and CT imaging of the brain shows the eye is pushed forward by the meningioma and can determine if and how far the meningioma continues into the brain. Treatment involves seeing if and how fast the tumor is spreading. Treatment usually consists of surgery to try to remove the entire meningioma, but sometimes the optic nerve cannot be spared and must also be removed. Therefore, many physicians recommend radiation therapy.

References:

http://www.eyecancer.com/Patient/Condition.aspx?nID=66&Category=Optic+Nerve+Tumors&Condition=Optic+Nerve+Sheath+Meningioma

http://webeye.ophth.uiowa.edu/TONI/FAQ.htm#5

http://webeye.ophth.uiowa.edu/TONI/general.htm

http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ija/vol16n1/meningioma.xml

Rathke's Cleft Cyst

What is it?

A Rathke's cleft cyst (RCC) is a large cyst from a cleft in the pituitary gland that remains as the Rathke's pouch, a normal part of embryological development that forms the pituitary gland, doesn't close during early fetal stages. They are non-cancerous growths that can interfere with normal pituitary actions and are often mistaken as tumors on an MRI. They are most commonly recognized in adults, but are also seen in childhood. These cysts are rare lesions that make up less than 1% of all primary brain masses.

What are some symptoms?

Rathke's cleft cysts are usually occur without symptoms and are incidentally discovered by an autopsy or MRI scan. When symptoms of these cysts do occur, they are usually ones of headaches and visual disturbances. Symptoms that the pituitary is not functioning right include: growth problems, delayed puberty, loss of libido, menstrual irregularities, fatigue, personality changes, and irregular blood pressure. Some other signs are dry skin, consitpation, nausea, and excessive thirst.

How is it treated?

Rathke's cleft cysts are diagnosed usually by MRI imaging and sometimes CT scanning. People with symptomatic cysts primarily opt for surgical removal. The surgeon tries to remove the cyst completely without impairing normal pituitary gland and brain function. One way to remove the cyst is through the nasal sinuses, also called transsphenoidal. If the cyst is located above the pituitary gland, the surgeon will probably have to go in through the skull. Once surgical treatment has been given the likelihood of recurrence is very low, but if the cyst cannot be completely removed physicians sometimes advise radiation treatment.

References:

http://neurosurgery.ucla.edu/body.cfm?id=215

http://www.pituitary.org/disorders/rathkes_cleft_cysts.aspx

http://www.hormone.org/Pituitary/non-secretory.cfm

Otosclerosis

What is it?

Otosclerosis is the abnormal growth of spongy bone in the middle ear that causes structures to not properly work, resulting in conductive hearing loss. Depending on where in the ear the growth is occuring, different kinds of hearing loss can occur. Usually, otosclerosis affects the stapes that is located in the entrance to the inner ear, also called the oval window. The abnormal growth causes the stapes to become attached in the oval window, and sound waves are obstructed from entering the inner ear. Otosclerosis may sometimes impair sensory cells or nerve fibers in the inner ear resulting in sensorineural hearing loss, making the level of hearing loss higher.

What causes it?

Some studies have shown that otosclerosis may be hereditary. A person that has one parent having the disease is 25% more likely to get it, and a person with both parents having the disease is 50% more likely to have it passed on to them. White, middle aged women are more susceptible to it than others. Research proposes that there may be a connection between otosclerosis and pregnancy hormonal changes, and a link between the disease and some viral infections.

What are the symptoms?

The most frequent and obvious sign of otosclerosis is hearing loss. It may start out as an overlooked loss of hearing of low pitched sounds, but it can gradually become worse until it is very severe. Some people may also experience dizziness, issues with balance, or tinnitus.

How is it diagnosed?

People who suffer from the symptoms of otosclerosis will need to see a physician specializing in the ear, an otologist for example, to rule out other illnesses or diseases. the physician may send the patient to an audiologist to diagnose, assess, and rehabilitate impairment of hearing and balance using tests. An audiogram is a graph that shows hearing sensitivity. A tympanogram is a graph that shows the functional ability of the middle ear to carry sound.






How is it treated?

If the hearing loss is mild and the person chooses not to opt to measures such as surgery, a hearing aid may be used to amplify sound. In many cases though, surgery is the first option for the complete treatment of otosclerosis, although some hearing loss can persist post-surgery.
Sometimes a stapedectomy is performed. In this operation, a surgeon removes the old stapes and inserts a prosthetic device that transfer sound waves to the inner ear.

References:

http://www.nidcd.nih.gov/health/hearing/otosclerosis.asp

https://www.audiologyonline.com/articles/article_detail.asp?article_id=288

http://www.radswiki.net/main/index.php?title=Otosclerosis

http://deafness.about.com/cs/etiology/a/otosclerosis.htm

ALS

The reason I chose to research this is once a year usually my work raises money and awareness for research of this disease. So I figured I could learn some information about it.

What is it?

Amyotrophic lateral sclerosis (ALS) is a progressive neurological disease that attacks motor neurons (nerve cells in the lateral brain and spinal cord that reach muscles all over body and are responsible for voluntary movement). The neurons are affected and degenerated until they eventually die. Basically, the brain cannot send messages to muscles in order for them to move. ALS is also referred to as Lou Gehrig's disease, named after a famous baseball player who died of the disease in 1941.

There are an estimated number of 30,000 cases of ALS in the U.S. at any given time. It does not affect some races and ethnicities over others, but it is much more common in males than females. People from the ages of 40 to 60 are most commonly affected, but there have been cases in people much younger than that. Most of the time there are no risk factors that can conclude whether a person will have ALS; it is very random. ALS can be inherited from parents in about 5 to 10 percent of cases. The familial cases usually are a result of one parent carrying the gene encoded for ALS.

Detection of Corticospinal Tract Compromise in Amyotrophic Lateral Sclerosis with Brain MR Imaging: Relevance of the T1-Weighted Spin-Echo Magnetization Transfer Contrast Sequence

What are some symptoms?

Symptoms include weakness of the limbs such as the arms, hands, legs, and feet. Twitching and cramping of the muscles is usually seen. Difficulty speaking (slurred speech) and swallowing can occur. In more advanced stages, difficulty breathing can result and a ventilator is needed to help keep the person alive. Symptoms can progressively get worse, leading to paralysis, and spread to other parts of the body with time.

How is it diagnosed?

There is no one exact test that can verify a person has ALS. A series of tests are given and the diagnosis results as other diseases are ruled out. Exams that the physician usually orders along with reviewing symptoms and signs are:

• EMG (electromyography)- detects electrical activity in muscles using fine wire electrodes

• NCV (nerve conduction velocity)- electrodes send a small shock to muscles to determine function; usually rules out other diseases

• MRI (magnetic resonance imaging)- uses radio waves and a strong magnetic field to visualize brain and spinal cord; used for detection of tumors and other abnormalities

• blood and urine tests
  
• muscle biopsy- removal of piece of muscle if muscle disease is suspected
  
• spinal tap- test the fluid surrounding brain and spinal cord

Detection of Corticospinal Tract Compromise in Amyotrophic Lateral Sclerosis with Brain MR Imaging: Relevance of the T1-Weighted Spin-Echo Magnetization Transfer Contrast Sequence

How is ALS treated?


Today, nothing has been found to reverse or cure ALS. Treatment focuses more on slowing down the stages of ALS and making patients more comfortable as they have to continue to deal with the disease. Physicians can prescribe drugs to reducepain, fatigue, twitching and cramping of muscles, and also for the relief of depression that can sometimes go along with the disease. Physical therapy and speech therapy are offered to help the person try to stay mobile and learn to adapt. Many times, ventilators are needed to inflate and deflate the lungs after advanced stages of ALS have set in and the muscles of the diaphragm are no longer to do so on their own.

There is one FDA approved drug that slows the disease's progression by reducing damage to the motor neurons. Riluzole (Rilutek) reduces the level of glutamate (a chemical messenger in the brain) in the body. The drug does not reverse the stages of ALS, but studies have shown that people who take the drug have longer survival rates by several months. It also prolongs the time before which a person would need to be placed on a ventilator.





References:

http://www.alsa.org/als/what.cfm?CFID=2685063&CFTOKEN=e844aa327d6427ab-87842C7C-188B-2E62-8090905205824399

http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.htm#126484842

http://www.mayoclinic.com/health/amyotrophic-lateral-sclerosis/DS00359/DSECTION=causes