Degenerative Disorders

What is Degenerative Disorder? Degenerative disorder is the result of a continuous process based on degenerative cell changes, affecting tissues or organs, which will increasingly deteriorate over time, whether due to normal bodily wear or lifestyle choices such as exercise or eating habits.

Degenerative Diseases • Parkinson’s Disease

• Alzheimer's Disease

• Huntington’s Disease

• Multiple Sclerosis

Parkinson’s Disease• Parkinson’s disease is a progressive disease of the

nervous system marked by tremor, muscular rigidity, and slow, imprecise movement, chiefly affecting middle-aged and elderly people. It is associated with degeneration of the basal ganglia of the brain and a deficiency of the neurotransmitter dopamine.

What Causes Parkinson's Disease?

• Parkinson's disease is caused by the progressive impairment or deterioration of neurons (nerve cells) in an area of the brain known as the substantia nigra. When functioning normally, these neurons produce a vital brain chemical known as dopamine.

Why Does Parkinson's Disease Occur?• There is increasing evidence that Parkinson's

disease may be inherited (passed on genetically from family members).

• There is considerable controversy surrounding the possibility of a genetic cause of Parkinson's disease. In a small number of families, specific genetic abnormalities leading to the illness have been identified. However, the vast majority of people with Parkinson's disease do not have one of these identified genetic abnormalities.

What Are the Symptoms of Parkinson's Disease?

• Parkinson's disease is a movement disorder that progresses slowly. Some people will first notice a sense of weakness, difficulty walking, and stiff muscles. Others may notice a tremor of the head or hands. Parkinson's is a progressive disorder and the symptoms gradually worsen. The general symptoms of Parkinson's disease include:

• Slowness of voluntary movements, especially in the initiation of such movements as walking or rolling over in bed

• Decreased facial expression, monotonous speech, and decreased eye blinking

• Unsteady balance; difficulty rising from a sitting position

• Abnormal tone or stiffness in the trunk and extremities• Swallowing problems in later stages• Light-headedness or fainting when standing

(orthostatic hypotension)

5 Stages of Parkinson’s

• Stage I is the mildest form of Parkinson’s. At this level, there may be unusual symptoms, but they’re not severe enough to interfere with daily tasks and overall lifestyle. In fact, the signs of the disorder are so minimal at this stage that they’re often missed.

Stage II• Stage II is considered a moderate form of

Parkinson’s, and the symptoms are much more noticeable than those experienced in stage I. Stiffness, tremors, and trembling may be more noticeable, and changes in facial expressions can occur. While muscle stiffness prolongs task completion, stage II does not impair balance.

STAGE III

• Stage III is the mid-stage in Parkinson’s, and it marks a major turning point in the progression of the disease. Many of the symptoms are the same as those in stage II, except now loss of balance and decreased reflexes can also occur. This is why falls are common in stage III.

STAGE IV

• Independence separates Parkinson’s patients from stage III and stage IV. During stage IV, it’s possible to stand without assistance. However, movement may require a walker or other type of assistive device.

STAGE V

• Stage V is the most advanced and debilitating stage of Parkinson’s disease. Advanced stiffness in the legs can also cause freezing upon standing. Patients require wheelchairs, and are often unable to stand without falling. Around-the-clock assistance is required to prevent falls.

Parkinson's Treatment• Parkinson's disease can't be cured, but medications

can help control your symptoms, often dramatically. In some later cases, surgery may be advised.

• Medications may help you manage problems with walking, movement and tremor. These medications increase or substitute for dopamine, a specific signaling chemical (neurotransmitter) in your brain.

Medications

• Carbidopa-levodopa• Carbidopa-levodopa infusion• Dopamine agonists• MAO-B inhibitors• Catechol-O-methyltransferase (COMT) inhibitors• Anticholinergics• Amantadine

Surgical Procedures• Deep brain stimulation. In deep brain stimulation

(DBS), surgeons implant electrodes into a specific part of your brain. The electrodes are connected to a generator implanted in your chest near your collarbone that sends electrical pulses to your brain and may reduce your Parkinson's disease symptoms.

Alzheimer's Disease

• Alzheimer's disease is a neurological disorder in which the death of brain cells causes memory loss and cognitive decline. A neurodegenerative type of dementia, the disease starts mild and gets progressively worse.

Causes of Alzheimer's Disease• Like all types of dementia, Alzheimer's is caused by brain

cell death.3 It is a neurodegenerative disease, which means there is progressive brain cell death that happens over a course of time.

• The total brain size shrinks with Alzheimer's - the tissue has progressively fewer nerve cells and connections.

• As more and more brain cells die, Alzheimer's leads to significant brain shrinkage. When doctors examine Alzheimer's brain tissue under the microscope, they see two types of abnormalities that are considered hallmarks of the disease:

• Plaques - These clumps of a protein called beta-amyloid may damage and destroy brain cells in several ways, including interfering with cell-to-cell communication. Although the ultimate cause of brain-cell death in Alzheimer's isn't known, the collection of beta-amyloid on the outside of brain cells is a prime suspect.

• Tangles - Brain cells depend on an internal support and transport system to carry nutrients and other essential materials throughout their long extensions. This system requires the normal structure and functioning of a protein called tau.

Symptoms• At first, increasing forgetfulness or mild confusion may be

the only symptoms of Alzheimer's disease that you notice. But over time, the disease robs you of more of your memory, especially recent memories. The rate at which symptoms worsen varies from person to person.

• If you have Alzheimer's, you may be the first to notice that you're having unusual difficulty remembering things and organizing your thoughts. Or you may not recognize that anything is wrong, even when changes are noticeable to your family members, close friends or co-workers.

Three stages of Alzheimer's

• Stage 1 – Mild/Early (lasts 2-4 yrs) – Frequent recent memory loss, particularly of recent conversations and events. Repeated questions, some problems expressing and understanding language. Mild coordination problems: writing and using objects becomes difficult. Depression and apathy can occur, accompanied by mood swings. Need reminders for daily activities, and may have difficulty driving.

• Stage 2 – Moderate/Middle (lasts 2-10 yrs) – Can no longer cover up problems. Pervasive and persistent memory loss, including forgetfulness about personal history and inability to recognize friends and family. Rambling speech, unusual reasoning, and confusion about current events, time, and place. More likely to become lost in familiar settings, experience sleep disturbances, and changes in mood and behavior, which can be aggravated by stress and change

• Stage 3 – Severe/Late (lasts 1-3+ yrs) – Confused about past and present. Loss of ability to remember, communicate, or process information. Generally incapacitated with severe to total loss of verbal skills. Unable to care for self. Falls possible and immobility likely. Problems with swallowing, incontinence, and illness. Extreme problems with mood, behavior, hallucinations, and delirium. In this stage, the person will need round the clock intensive support and care.

How is Alzheimer's disease treated?

• Alzheimer's disease is complex, and it is unlikely that any one drug or other intervention can successfully treat it. Current approaches focus on helping people maintain mental function, manage behavioral symptoms, and slow or delay the symptoms of disease.

What drugs are currently available to treat Alzheimer's?

• Cholinesterase inhibitors. These drugs work by boosting levels of a cell-to-cell communication by providing a neurotransmitter (acetylcholine) that is depleted in the brain by Alzheimer's disease. The improvement is modest. Cholinesterase inhibitors can improve neuropsychiatric symptoms, such as agitation or depression, as well.

• Memantine (Namenda). This drug works in another brain cell communication network and slows the progression of symptoms with moderate to severe Alzheimer's disease. It's sometimes used in combination with a cholinesterase inhibitor. Side effects may include constipation, dizziness and headache.

Alternative Medicine• Omega-3 fatty acids. Omega-3 fatty acids in fish may

help prevent cognitive decline. Studies done on fish oil supplements haven't shown any benefit, however.

• Curcumin. This herb comes from turmeric and has anti-inflammatory and antioxidant properties that might affect chemical processes in the brain. So far, clinical trials have found no benefit for treating Alzheimer's disease.

• Ginkgo. Ginkgo is a plant extract containing several substances. A large study funded by the NIH found no effect in preventing or delaying Alzheimer's disease.

• Vitamin E. Although vitamin E isn't effective for preventing Alzheimer's, taking 2,000 international units daily may help delay the progression in people who already have the disease. However, study results have been mixed, with only some showing this benefit. Further research into the safety of 2,000 international units daily of Vitamin E in a dementia population will be needed before it can be routinely recommended.

Huntington’s Disease

• Huntington's disease is an inherited condition that damages certain nerve cells in the brain.

• This brain damage gets progressively worse

over time and can affect movement, cognition (perception, awareness, thinking, judgment) and behavior.

Symptoms: (Physical)

• Forms of nervous activity like fidgeting, minor twitching in fingers and toes;

• Excessive restlessness;• Some clumsiness;• Slight alterations in handwriting; and• Minor difficulty with normal daily physical

skills like driving.

Symptoms: (Emotional)

• Short-term memory loss;• Less ability to organize routine tasks or to

cope with new situations;• Periods of depression, apathy and irritability;

and• Impulsiveness.

Treatment:

• There's no cure for Huntington's disease and its progress can't be reversed or slowed down.

• As the condition progresses, it may put a strain on family and relationships. Treatments for Huntington's disease aim to improve any mood disturbance; this is done to maintain skills used in daily living that can deteriorate over time.

Treatment:

• Medication can help manage some of the symptoms, such as irritability or excessive movement. Therapies such as speech and language therapy and occupational therapy can help with communication and day-to-day living.

Multiple Sclerosis (MS):

• Multiple sclerosis (MS) is an unpredictable, often disabling disease of the central nervous system that disrupts the flow of information within the brain, and between the brain and body.

Multiple Sclerosis (MS) Symptoms:

• In multiple sclerosis (MS), damage to the myelin coating around the nerve fibers in the central nervous system (CNS) and to the nerve fibers themselves interferes with the transmission of nerve signals between the brain, spinal cord and the rest of the body.

Treatment:

• There is no cure for multiple sclerosis. Treatment typically focuses on speeding recovery from attacks, slowing the progression of the disease and managing MS symptoms. Some people have such mild symptoms that no treatment is necessary.

• Corticosteroids, such as oral prednisone and intravenous methylprednisolone, are prescribed to reduce nerve inflammation. Side effects may include insomnia, increased blood pressure, mood swings and fluid retention.

• Plasma exchange (plasmapheresis). The liquid portion of part of your blood (plasma) is removed and separated from your blood cells. The blood cells are then mixed with a protein solution (albumin) and put back into your body. Plasma exchange may be used if your symptoms are new, severe and haven't responded to steroids