PAGET'S DISEASE OF THE BONE

Introduction

Paget’s disease is a condition which bone formation is speeded up, changing the strength and shape of the bone. The disease is named after a mid-century English surgeon, Sir James Paget, who also identified Paget’s disease of the breast. Bone is a living tissue engaged in a continual process of renewal. Paget’s disease of the bone disrupts this constant remodeling process of old bone being removed and replaced by new bone. Early in the course of the disease, old bone starts breaking down faster than new bone can be built. Over time, your body responds by generating new bone at a faster than normal rate. This rapid remodeling produces bone that’s softer and weaker than normal bone, which can lead to bone pain, deformities and fractures.
Paget’s disease of the bone becomes more common with age. It often begins between the ages 50 and 70. Also it is slightly more common in men than in women No definite evidence exits indicating that paget’s disease is hereditary. However, in some instances, it tends to run in families. The disease usually affects the skull, the spine and the bones in your arms, legs, and pelvis. It may affect only one or two areas of your body, or may be widespread.

Causes, Signs and Symptoms

The cause of Paget’s disease is unknown. Some scientists believe it is related to a viral infection in your bone cells that may be present for many years before the problems appear. They also have discovered several genes that appear to be linked to the disorder. Hereditary factors seem to influence whether you’re susceptible to the disease.
Most people with Paget’s disease have no symptoms. When symptoms do occur, they typically affect each person differently. Affected areas may include:
1. Bones --- pain may be constant, aching, deep and most severe at night. Bone deformities occur such as bowlegs, enlarged head size and fractures.
2. Joints --- wear and tear in your cartilage lining of the affected bones cause pain, swelling, stiffness in your affected joints, and warmth in your skin over those affected joints.
3. Nerves --- enlarged bones can compress your spinal cord or the nerves exiting your brain and spinal cord causing numbness, tingling, weakness, hearing loss, double vision and other neurological problems
Eventually, the rapid bone breakdown and disorganized bone formation slows down, and Paget’s disease appears to “burn out”. Symptoms may come and go but, any bone enlargement or bowing that has occurred will remain.

Diagnostic tests and Treatments

Although there is no way to prevent Paget’s disease, it can be detected before it becomes a serious problem. The following procedures may help detect Paget’s disease of the bone:
1. X-rays --- images can show bone reabsorption, enlargement, and deformities
2. Blood test --- check every 2-3 years after age 40 for elevated alkaline phosphatase level (responsible for forming new bone)
3. Bone Scan --- radioactive tracers can pick up this disease before it can be seen on an X-ray
4. Urine tests --- 24 hour urine collection can determine if an increased amount of hydroxyproline ( another product of bone breakdown ).
Often, people with Paget’s disease of the bone have no symptoms at all and may not require treatment other than regular monitoring. But if signs or symptoms are painful and troublesome, treatment for Paget’s disease of the bone include the following:
1. Occupational or Physical Therapist
2. Medications
3. Healthy Diet
4. Surgery
For more information: www.orthop.washington.edu or www.mayoclinic.com/health/

CHIARI MALFORMATION

CHIARI MALFORMATION

Definition and Description
Chiari malformation is a rare genetic disorder in which parts of the brain protrude through the opening in the base of the skull into the spinal column. This displaced tissue may obstruct the flow of cerebrospinal fluid which nourishes the brain and spinal cord. A German pathologist named Chiari was the first to describe Arnold-Chiari malformation in 1891. There are four major regions of the brain affected in Chiari malformation:
 The cerebellum --- its main purpose is to coordinate the body movements
 The brain stem --- it regulates involuntary actions the body must conduct to survive, such as breathing, swallowing, and blinking the eyes
 The ventricles --- their function is to produce and circulate cerebrospinal fluid
 The cerebrum --- it’s responsible for the higher functions of the brain, such as thought

Causes and Symptoms
Although this malformation is present at birth, there may not be any symptoms of a problem until adulthood. Women have a higher incidence of this disorder than men. One of the most common symptoms of Chiari malformation is a headache. Coughing, sneezing, or bending forward may bring on these headaches which can last minutes or hours and can be linked with nausea. There may be neck or upper arm pain. Often pain is more on one side, rather than equal on both sides. There also may be weakness in the arm or hand, tingling, burning, numbness, difficulty swallowing, hoarseness, visual problems, and balance can be affected.
The exact cause is unknown; however, it is suspected that an increased pressure in the brain during embryonic development may cause brain structures to be displaced into the spinal canal. Other possible causes include exposure to harmful substances during fetal development or genetic factors. There has not yet been found a gene responsible for Chiari malformation or a study that shows whether or not this disorder is inherited, but there are reports of several families where more than one family member has this malformation.

Diagnosis and Treatment
To diagnosis Chiari malformation a complete medical history, physical exam, and a thorough neurological exam will be conducted by physician. The following procedures are also commonly used for diagnosis and follow up care:
 MRI --- the most helpful diagnostic tool which allows doctors to see the brain and spinal cord from several different angles
 CT --- produces cross-section images of the body that are helpful in assessing abnormalities of the brain and backbone
 Ultrasound --- a diagnostic procedure used to visualize internal organs
The recommended treatment for Chiari malformation is surgery

For more information: http://www.healthline.com/galecontent/chiari-malformation or
http://www.healthline.com/galecontent/arnold-chiari-malformation

CLAY-SHOVELER'S FRACTURE

Definition and Causes

The clay-shoveler’s fracture is an oblique fracture of the lower cervical or upper thoracic spinous processes, commonly C6 >C7 >T1. This uncommon breakage of the spine is a result of stress. It results from hyper flexion, a type of avulsion injury at the base of the spinous process. If the avulsion fracture is not limited to the spinous process but extends into the lamina, there is greater potential for spinal cord injury. This is a stable injury that was originally named for those who were mining clay, although it usually occurs in laborers who perform activities involving lifting weights rapidly with the arms extended. Examples of these activities include shoveling snow up and over the head backwards. The shear force of the muscles ( trapezius and rhomboid muscles ) pulling on the spine at the base of the neck actually tears off the bone of the spine.

Symptoms and Treatment

Symptoms of clay-shoveler’s fracture include burning, “knife-like” pain at the level of the fractured spine between the upper shoulder blades. The pain can sharply increase with the repeated activity that strains the muscles of the upper back. While the intense pain gradually subsides in days to weeks, the area can intermittently develop burning pain with certain activities that involve prolonged extending of the arms ( like computer work ).
The clay-shoveler’s fracture is diagnosed by an x-ray exam of the spine. Most patients require no treatment. Pain medications, physical therapy, and massage can be of help. Once the presence of segmental instability has been ruled out, clay-shoveler’s fractures can be treated non operatively. Occasionally, surgical removal of the tip of the broken spine is performed for those with long standing pain.

For more information: http://www.gentili.net/fracture.asp , http://www.wheelessonline.com/ or
http://www.medterms.com/script/main/art.asp

CERVICAL VERTIGO

OVERVIEW

Cervical vertigo is sometimes called Bow Hunter’s Syndrome, an uncommon condition in which the vertebral artery is symptomatically occluded during neck rotation. Vertigo or dizziness is provoked by a neck posture no matter what the orientation of the head is to gravity. It occasionally accompanies a neck injury. Also it is estimated that 20—58% of patients who sustain closed-head injuries or whiplash experience late onset symptoms of dizziness, disequilibrium, hearing loss, ear pain and vertigo.

CAUSES

There are many potential causes of cervical vertigo. Some causes are as follows:
§ Vascular compression --- the vertebral arteries in the neck can be compressed by the vertebrae or other structures (Bow Hunter’s Syndrome)
§ Sensory information from the neck may be absent or unreliable.
§ Cervical cord compression --- this is the most common mechanism of cervical vertigo
§ Cerebrospinal Fluid ( CSF) leak due to tear of cervical root sleeve

DIAGNOSIS

There is no consensus on how to diagnose cervical vertigo. The process is generally uncertain and frustrating. Routine exams for positive confirmation of cervical vertigo include:
§ ENG
§ OAE
§ VEMP
§ Audiogram
§ MRI-neck and MRI-brain
§ CT-angiography
§ Flexion/extension x-rays of neck

TREATMENT

For the usual person in whom cervical vertigo is a diagnosis of exclusion, and pain is prominent the following treatment is recommended, possibly combined to relieve pain and reduce spasm.
§ Physical therapy --- includes gentle mobilization, exercise, and instruction in proper posture and use of the neck
§ Medical management ---includes muscle relaxants for pain and when arthritis is present
§ Antidepressants -- for chronic pain and the reactive depression that often accompanies
§ Cervical blocks --- to reduce painful muscle spasms
§ Surgery --- can be complicated, but is nearly always successful

For more info. http://www.dizziness-and-balance.com/ or http://www.revolutionhealth.com/

PARATHYROID ADENOMA

DEFINITION

Parathyroid adenomas are non-cancerous (benign) tumors of the parathyroid glands, which are located in the neck. The parathyroid glands are four pea-sized organs found near the thyroid gland. There are no other organs in the body that are present 4 times. When the 4 parathyroid glands are formed (before we are born), they migrate from one part of the neck to another. It is this migration of the parathyroid glands that makes finding them tricky later in life. The parathyroid anatomy is the most variable anatomy in the human body. The parathyroid glands can be located from just below the jaw to the area around the heart. These glands make parathyroid hormone (PTH or parathormone). PTH helps the body use and store calcium to keep the calcium in the blood at normal level.

CAUSES AND SYMPTOMS

Parathyroid adenomas are generally caused by a problem with your genes. The parathyroid glands help control the break down of calcium in the body. The most common cause of parathyroid adenomas is hyperparathyroidism, which leads to high levels of calcium in the blood (hypercalcemia). The hypercalcemia caused by hyperparathyroidism is more serious and life threatening than parathyroid cancer itself. Women over 60 are at highest risk, and radiation to your head or neck also increase your risk.
Many people have no symptoms. The condition is often discovered by accident when you are being examined for another problem. Symptoms that may occur include the following:
§ Lethargy
§ Confusion
§ Nausea
§ Constipation
§ Myalgias (muscle pain)
§ Kidney stones
§ Bone fractures

EXAMS AND TREATMENT

Blood tests are done to check the levels of serum calcium, serum phosphorus, serum chloride, and serum bicarbonate.
A 24 urine test is done to check for increased calcium in the urine.
Other exams include:
§ Bone density exam
§ Kidney x-rays (may show kidney stones)
§ Ultrasound or CT (may show kidney stones)
§ MRI or Tc-thallium neck scan (may show enlarged parathyroid glands)
§ Sestambi scan: a radionuclide scan used to find an overactive parathyroid gland.

Surgery is the most important treatment. The goal when performing parathyroid surgery is to remove only the bad parathyroid gland and leave the good one. Remember, in about 95% of the time, its only one parathyroid gland that has become diseased (it grows into a benign tumor about the size of an olive or grape). Some postmenopausal women might consider estrogen replacement and many patients with mild hyperparathyroidism are not treated but are monitored for escalation of symptoms.

For more info go to: http://www.nlm.nih.gov/medlineplus/ency/artcle/001188.htm
http://www.cancer.gov/ , http://parathyroid.com/

MOYAMOYA DISEASE

WHAT IS MOYAMOYA DISEASE ?

Moyamoya disease is a rare, cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called basal ganglia. In Japanese the name "moyamoya" means "puff of smoke" and describes the look of the tangle of tiny vessels formed to compensate for the blockage. In the 1960's, this disease was first described in Japan and since has been found in individuals in the United States, Europe, Australia, and Africa. Moyamoya disease primarily affects children, but it can occur in adults also. Because it tends to run in families, researchers think that it is the result of inherited genetic abnormalities. Approximately 10% of the cases are genetic caused and are termed primary Moyamoya disease. Secondary Moyamoya disease refers to cases in which the disease is a result of another underlying disorder. In this case it is very important for the physician to determine the root underlying cause.

SIGNS AND SYMPTOMS

The signs most typically include paralysis of the feet, legs or upper extremities. Headaches, mental retardation, various vision problems, speech deficits, and psychiatric problems.

In children, the first symptom of Moyamoya disease is often a stroke, or recurrent transient ischemic attacks (TIA's, commonly referred to as "mini-strokes"), frequently followed by muscular weakness affecting one side of the body or seizures. Adults often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels.

TREATMENT

There is no cure for moyamoya disease, but treatment may help manage the signs and symptoms. Treatment may include:

• Corticosteroid medications


• Blood-thinning medications to reduce the risk of blood clots


• Revascularization surgery to bypass blocked arteries or open narrowed blood vessels in order to restore blood to the brain

For more info go to: http://www.rarediseases.org , http://www.ninds.nih.gov/disorders , http://www.mayoclinic.com

Without treatment, Moyamoya disease can be fatal as a result of intracerebral hemorrhage.

PARANASAL SINUS CANCER

DEFINITION
The paranasal sinus is the spaces within the bones behind the nose and cheeks. Para nasal means “around or near the nose.” Sinuses are cavities or small tunnels. Each sinus is lined with cells that produce mucus to prevent the lining of the nose from drying out, and moisten the air that we breathe. They also give your voice an individual tone, lighten the weight of the skull, and provide a bony frame work for the eyes and face.

CAUSES
Paranasal sinus cancer is a rare type of cancer that can occur at any age, but is very rare in people under 40. Men are twice as likely to be diagnosed with it than women. Smoking has been found to increase the risk of developing cancer in this area of the body. Also people who use snuff for many years, or those who work in the wood or furniture industry and are exposed to wood dust, are more likely to develop it. Paranasal sinus cancer is not infectious or inherited and cannot be passed on to other people. Like many other forms of cancer, the exact causes are unknown.

SIGNS AND SYMPTOMS
The symptoms can vary depending on which sinuses are affected. The most common symptoms include:
Pain behind the nose or upper teeth
Blocked sinuses which do not clear
Swelling around the eyes and double vision
Numbness of the cheek, lip, upper teeth or side of the nose
Persistent nosebleeds, headaches, and speech changes

DIAGNOSIS AND TREATMENT
Paranasal sinus cancer is most likely to be cured when diagnosed at an early stage. Therefore, any of the above symptoms should be reported to your doctor if they don’t improve in over a few days. The following tests are commonly used to make a firm diagnosis:
X-rays--- to show the size and position of the cancer
CT or MRI scan---to find the exact site of the cancer, or check for any spread
Nasopharyngoscopy---biopsy to examine abnormal tissue for cancer cells
Treatment of paranasal sinus cancer will depend on the position and stage of the cancer, the type, the grade, and your health in general. The following treatments alone or in combination with one another maybe used for your situation:
Surgery---can be quite extensive and in some cases skin grafts or flaps are needed
Radiotherapy---uses high energy rays to destroy the cancer cells
Chemotherapy---uses anti-cancer (cytotoxic) drugs to destroy cancer cells
For more information on paranasal sinus cancer go to http://www.cancerbackup.org.uk/Cancertype/Headneck/typesofheadneckcancers/... http://www.cancer.org/docroot/cri/content/cri