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/