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