Lou Gehrig's disease

Lou Gehrig's disease is a disorder that's also called amyotrophic lateral sclerosis (say: ah-my-uh-tro-fik lah-tuh-rul skluh-ro-sis), or ALS. The official name comes from these Greek words:

* "a" for without
* "myo" for muscle
* "trophic" for nourishment
* "lateral" for side (of the spinal cord)
* "sclerosis" for hardening or scarring

So, amyotrophic means that the muscles have lost their nourishment. When this happens, they become smaller and weaker. Lateral means that the disease affects the sides of the spinal cord, where the nerves that nourish the muscles are located; and sclerosis means that the diseased part of the spinal cord develops hardened or scarred tissue in place of healthy nerves.

ALS is often called Lou Gehrig's disease after Lou Gehrig, a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s. People in England and Australia call ALS Motor Neurone Disease (MND). The French refer to it as Maladie de Charcot, after the French doctor Jean-Martin Charcot, who first wrote about ALS in 1869.
What Is Lou Gehrig's Disease?

Lou Gehrig's disease damages motor neurons in the brain and spinal cord. Motor neurons are nerve cells that control muscle movement. Upper motor neurons send messages from the brain to the spinal cord, and lower motor neurons send messages from the spinal cord to the muscles. Motor neurons are an important part of the body's neuromuscular system. The neuromuscular system enables our bodies to move and is made up of the brain, many nerves, and muscles. Things that we do every day — like breathing, walking, running, lifting stuff, and even reaching for a glass of water — are all controlled by the neuromuscular system.

Here's how the neuromuscular system works: If you want to make a fist, your brain first sends signals through upper motor neurons to the area in your spinal cord that controls your hand muscles. Then lower motor neurons in your spinal cord signal the muscles in your hand to move and make a fist.

Over time, Lou Gehrig's disease causes these motor neurons in the brain and spinal cord to shrink and disappear, so that the muscles no longer receive signals to move. As a result, the muscles become smaller and weaker. Gradually the body becomes paralyzed (say: par-uh-lized), which means that the muscles no longer work. However, someone with ALS, even at an advanced stage, can still see, hear, smell, and feel touch. The nerves that carry feelings of hot, cold, pain, pressure, or even being tickled, are not affected by Lou Gehrig's disease. The parts of the brain that allow us to think, remember, and learn are also not affected by the disease.

Although this disease can strike anyone, it is extremely rare in kids. According to the ALS Association, most people who develop Lou Gehrig's disease are adults between 40 and 70. Only 2 out of every 100,000 people will get the disease each year. Because it is not contagious, you can't catch ALS from someone who has the disease.

Among ALS cases in the United States, 5% to 10% are hereditary, which means the disease runs in certain families. This is called familial (say: fuh-mee-lee-ul) ALS. At least 90% of cases are not inherited; this is called sporadic (say: spuh-rah-dik) ALS.
How Is the Disease Diagnosed?

Lou Gehrig's disease doesn't always begin or become worse in the same way. The disease is different for every person who has it. In general, muscle weakness, especially in the arms and legs, is an early symptom for more than half of people with ALS. Other early signs are tripping or falling a lot, dropping things, having difficulty speaking, and cramping or twitching of the muscles. As the disease gets worse over time, eating, swallowing, and even breathing may become difficult.

It may take several months to know for sure that someone has Lou Gehrig's disease. The illness can cause symptoms similar to other diseases that affect nerves and muscles, including Parkinson's disease and stroke. A doctor will examine the patient and do special tests to see if it might be one of those other disorders. (It's like using the process of elimination to figure out the answer to a multiple-choice question on a test.)

One of the tests, an electromyogram (say: eh-lek-tro-my-uh-gram), or EMG, can show that muscles are not working because of damaged nerves. Other tests include X-rays, magnetic resonance imaging (MRI), a spinal tap, and blood and urine evaluations. Sometimes a muscle or nerve biopsy is needed. A biopsy is when a doctor takes a tiny sample of tissue from the body to study under a microscope. Examining this tissue can help the doctor figure out what's making someone sick.
How Is the Disease Treated?

Currently, there's no way to prevent or cure Lou Gehrig's disease, but a number of treatments are available to people with the disease. Medicines can control symptoms, such as muscle cramping and difficulty swallowing, and other drugs can slow the development of the disease.

Physical therapy can help people with ALS cope with muscle loss and breathing problems. Special equipment is also provided when it becomes necessary. For instance, a power wheelchair can enable a paralyzed person with ALS to get around. A machine called a ventilator (say: ven-til-ay-ter) can help a someone breathe.

In addition, a nurse or other health assistant may come to the person's home to provide care that the family cannot handle alone. It's normal for family members to feel upset, overwhelmed, and sad if a loved one has ALS. Counseling, as well as support from other family members and friends, can make it easier to deal with the challenges they face.
What's Life Like for Someone With Lou Gehrig's Disease?

According to the ALS Association, about half of all people with ALS live at least 3 years after they find out they have the disease, and 20% (or 1 in five) live 5 years or more. As many as 10% will survive more than 10 years.

Stephen Hawking has been living with Lou Gehrig's disease for about 40 years — ever since his diagnosis at age 21. He is the most famous long-term survivor of the disease. Born in England, Hawking is a famous physicist who furthered our understanding of the universe. He has written a lot of books, including the bestseller A Brief History of Time. He has done these things despite being confined to a wheelchair for more than 20 years, being able to move only a few fingers, and needing a voice synthesizer and special computer to speak and write.

Living with Lou Gehrig's disease is physically difficult, but it is reassuring to know that the mind is not affected. People with the disease can think as clearly as ever, are able to maintain relationships with friends and family, and should be treated respectfully and normally. Communication can be difficult because the disease affects the person's breathing and the muscles needed for speech and arm movement. With patience, the families of patients with ALS can learn to communicate effectively with their loved one.

Researchers continue to study ALS as they try to understand why it happens, and how the disease damages the motor neurons in the brain and spinal cord. As they learn more about the disease, researchers can continue to develop new and better treatments.

Cause:-

In a paper published in the June 1 issue of the Journal of Biological Chemistry, UK molecular and cellular biochemistry assistant professor Haining Zhu reports on how mutations in the gene that makes superoxide dismutase 1 (SOD1) slows down the intracellular transport of molecules in neurons.

The results of the study by Zhu and his colleagues show that the defective transport of molecules is probably due to the aberrant interaction between the disease-causing SOD1 mutants and a motor complex that is essential to the intracellular transport in neurons. This provides new insight into ways to prevent or slow ALS.

ALS is characterized by the death of neurons that control voluntary muscles, leading to muscle weakness and atrophy. Around 25 percent of the familial forms of the disease have been linked to the mutation in the gene that makes SOD1.

Until now, how the defective variants of the SOD1 protein may interact with other cellular components to cause the disease has remained unclear. Previous studies have shown slowing of transport process in ALS patients and in related animal models. However, it is unknown how the transport process is compromised. The new results from the study by Zhu and his colleagues provide a molecular mechanism for the defective transport in ALS.

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Dupuytren's contracture

Dupuytren's contracture is an uncommon hand deformity in which the connective tissue under the skin of your palm contracts and toughens over time.

Knots of tissue form under the skin, eventually forming a thick cord that can pull one or more of your fingers into a bent position. Once this occurs, the fingers affected by Dupuytren's contracture bend normally but they can't be straightened completely, making it difficult to use your hand. Dupuytren's contracture can complicate everyday activities such as placing your hands in your pockets, putting on gloves or shaking hands.

Dupuytren's contracture is rarely painful, though sometimes the bumps of tissue on your palm can be sensitive to touch. Various treatments are available for Dupuytren's contracture to slow its progression and relieve your symptoms.


Dupuytren's contracture is a fairly common disorder of the fingers. It most often affects the ring or little finger, sometimes both, and often in both hands. Although the exact cause is unknown, it occurs most often in middle-aged, white men and is genetic in nature, meaning it runs in families. This condition is seven times more common in men than women. It is more common in men of Scandinavian, Irish, or Eastern European ancestry. Interestingly, the spread of the disease seems to follow the same pattern as the spread of Viking culture in ancient times. The disorder may occur suddenly but more commonly progresses slowly over a period of years. The disease usually doesn't cause symptoms until after the age of 40.

This guide will help you understand

* how Dupuytren's contracture develops
* how the disorder progresses, and how you can measure its progression
* what treatments are available

Anatomy

What part of the hand is affected?

The palm side of the hand contains many nerves, tendons, muscles, ligaments, and bones. This combination allows us to move the hand in many ways. The bones give our hand structure and form joints. Bones are attached to bones by ligaments. Muscles allow us to bend and straighten our joints. Muscles are attached to bones by tendons. Nerves stimulate the muscles to bend and straighten. Blood vessels carry needed oxygen, nutrients, and fuel to the muscles to allow them to work normally and heal when injured. Tendons and ligaments are connective tissue. Another type of connective tissue, called fascia, surrounds and separates the tendons and muscles of the hand.

Lying just under the palm is the palmar fascia, a thin sheet of connective tissue shaped somewhat like a triangle. This fascia covers the tendons of the palm of the hand and holds them in place. It also prevents the fingers from bending too far backward when pressure is placed against them. The fascia separates into thin bands of tissue at the fingers. These bands continue into the fingers where they wrap around the joints and bones. Dupuytren's contracture forms when the palmar fascia tightens, causing the fingers to bend.

The condition commonly first shows up as a thick nodule (knob) or a short cord in the palm of the hand, just below the ring finger. More nodules form, and the tissues thicken and shorten until the finger cannot be fully straightened. Dupuytren's contracture usually affects only the ring and little finger. The contracture spreads to the joints of the finger, which can become permanently immobilized.

Related Document: A Patient's Guide to Hand Anatomy

Causes

Why do I have this problem?

No one knows exactly what causes Dupuytren's contracture. The condition is rare in young people but becomes more common with age. When it appears at an early age, it usually progresses rapidly and is often very severe. The condition tends to progress more quickly in men than in women.

People who smoke have a greater risk of having Dupuytren's contracture. Heavy smokers who abuse alcohol are even more at risk. Recently, scientists have found a connection with the disease among people who have diabetes. It has not been determined whether or not work tasks can put a person at risk or speed the progression of the disease.

Symptoms

What does Dupuytren's contracture feel like?

Normally, we are able to control when we bend our fingers and how much. How much we flex our fingers determines how small an object we can hold and how tightly we can hold it. People lose this control as the disorder develops and the palmar fascia contracts, or tightens. This contracture is like extra scar tissue just under the skin. As the disorder progresses, the bending of the finger becomes more and more severe, which limits the motion of the finger.

Without treatment, the contracture can become so severe that you cannot straighten your finger, and eventually you may not be able to use your hand effectively. Because our fingers are slightly bent when our hand is relaxed, many people put up with the contracture for a long time. Patients with this condition usually seek medical advice for cosmetic reasons or the loss of use of their hand. At times, the nodules can be very painful. For this reason many patients are worried that something serious is wrong with their hand.

Diagnosis

How do doctors identify the problem?

Your doctor will ask you the history of your problem, such as how long you have had it, whether you've noticed it getting worse, and whether it has kept you from doing your daily activities. The doctor will then examine your hand and finger.

Your doctor can tell if you have a Dupuytren's contracture by looking at and feeling the palm of your hand and your fingers. Usually, special tests are unnecessary. Abnormal fascia will feel thick. Cords and small nodules in the fascia may be felt as small knots or thick bands under the skin. These nodules usually form first in the palm of the hand. As the disorder progresses, nodules form along the finger. These nodules can be felt through the skin, and you may have felt them yourself. Depending on the stage of the disorder, your finger may have started to contract, or bend.

The amount you are able to bend your finger is called flexion. The amount you are able to straighten the finger is called extension. Both are measured in degrees. Normally, the fingers will straighten out completely. This is considered zero degrees of flexion (no contracture). As the contracture causes your finger to bend more and more, you will lose the ability to completely straighten out the affected finger. How much of the ability to straighten out your finger you have lost is also measured in degrees.

Measurements taken at later follow-up visits will tell how well treatments are working or how fast the disorder is progressing. The progression of the disorder is unpredictable. Some patients have no problems for years, and then suddenly nodules will begin to grow and their finger will begin to contract.

The tabletop test may also done. The tabletop test will show if you can flatten your palm and fingers on a flat surface. You can follow the progression of the disorder by doing the tabletop test yourself. Your doctor will tell you what to look for and when you should return for a follow-up visit.

Treatment

What can be done for the condition?

There are two types of treatment for Dupuytren's contracture: surgical and nonsurgical. The best course of treatment is determined by how far the contractures have advanced.
Nonsurgical Treatment

In the early stages of this disorder, frequent examination and follow-up is recommended. Your doctor may inject cortisone into the painful nodules. Cortisone can be effective at temporarily easing pain and inflammation. Heat and stretching treatments given by a physical or occupational therapist may also be prescribed to control pain and to try to slow the progression of the contracture.

Treatment also consists of wearing a splint that keeps the finger straight. This splint is usually worn at night.

The nodules of Dupuytren's contracture are almost always limited to the hand. If you receive regular examinations and follow your doctor's advice, you may be able to slow the problems caused by this disorder. However, Dupuytren's contracture is known to progress, so surgery may be needed at some point to release the contracture and to prevent disability in your hand.
Surgery

No hard and fast rule exists as to when surgery is needed. Surgery is usually recommended when the joint at the knuckle of the finger reaches 30 degrees of flexion. When patients have severe problems and require surgery at a younger age, the problem often comes back later in life. When the problem comes back or causes severe contractures, surgeons may decide to fuse the individual finger joints together. In the worst case, amputation of the finger may be needed if the contracture restricts the nerves or blood supply to the finger.

Surgery for the main knuckle of the finger (at the base of the finger) has better long-term results than when the middle finger joint is tight. Tightness is more likely to return after surgery for the middle joint.

Tissue Release

The goal of tissue release surgery is to release the fibrous attachments between the palmar fascia and the tissues around it, thereby releasing the contracture. Once released, finger movement should be restored to normal. If the problem is not severe, it may be possible to free the contracture simply by cutting the cord under the skin. If the palmar fascia is more involved and more than one finger is bent, your surgeon may take out the whole sheet of fascia.

Palmar Fascia Removal

Removal of the entire palmar fascia will usually give a very good result. The cure is often permanent but depends a great deal on the success of doing the physical or occupational therapy as prescribed. Little ill effect is caused by removing the entire palmar fascia, although the fingers may bend backward slightly more than normal. If you decide to have this surgery, you must commit to doing the therapy needed to make your surgery as successful as possible.

Skin Graft Method

A skin graft may be needed if the skin surface has contracted so much that the finger cannot relax as it should and the palm cannot be stretched out flat. Surgeons graft skin from the wrist, elbow, or groin. The skin is grafted into the area near the incision to give the finger extra mobility for movement.

Related Document: A Patient's Guide to Dupuytren's Contracture Surgery

Rehabilitation

What should I expect after treatment?
Nonsurgical Rehabilitation

The ability of nonsurgical treatments to slow or actually reverse the contracture is not all that promising. The contracture usually requires surgery at some point. Heat, stretching, and a finger splint seem to help the most. These treatments may be directed by a physical or occupational therapist. Sessions may be scheduled for a few visits per week up for up to six weeks, but after that, you'll probably be instructed to continue using the splint and doing the stretches as part of a home program for several months.
After Surgery

Your hand will be bandaged with a well-padded dressing and a splint for support after surgery. Physical or occupational therapy sessions may be needed after surgery for up to six weeks. Visits will include heat treatments, soft tissue massage, and vigorous stretching. Therapy treatments after surgery can make the difference in a successful result after surgery.

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Causes For Swollen Penis

Allergic reaction - response of body to a foreign substance allergic reaction is considered a medical emergency. Urgent care may be required.
cramping abdominal pain, hives, itching, vaginal or vulvar itching or irritation

Balanitis - inflammation of glans penis
burning urination in males, discharge from penis, pain in penile foreskin, pain in penis

Insect bites - bites by various insects
a painful swelling in skin, lump or swelling on leg(s), lump(s) in skin, redness in skin

Poison ivy or oak - reaction to poison ivy or oak
blister(s) or bump(s) on skin, hives, itching, rash

Trauma urethra - injury to urethra trauma urethra is considered a medical emergency. Urgent care may be required.
burning urination in females, difficulty urinating in females, impotence, painful urination in females

Paraphimosis - foreskin trapped behind glans paraphimosis is considered a medical emergency. Urgent care may be required.
difficulty urinating in males, pain in penis, painful urination in males

The penis can be affected by several different disorders ranging in severity from swelling to nerve damage and impotence. Men experiencing these problems should always consult a licensed physician for proper diagnosis and treatment. Below are a few of the best known medical disorders that can affect the male sexual organ.

Penile Oedema

One of the more common disorders of the penis, called Oedema, is characterized by a swelling of the foreskin. Penile Oedema is often caused by excessive sexual activity, including masturbation. This is usually not a severe condition as long as the foreskin remains in it's normal position and blood flow is not obstructed. If swelling persists, however, a doctor should be consulted as this may point to chronic heart disease.

Paraphimosis

Paraphimosis (also called capistration) is a condition in which the prepuce, after having been retracted behind the glans penis, is constricted and does not return to it's forward position. This disorder generally occurs due to fluid trapped in the foreskin and can be the result of intense sexual activity. It is often temporary but if it persists for several hours a physician should be contacted.

Peyronie's Disease

Peyronie's disease is a disorder that is characterized by a curvature of the penis caused by the build up of fibrous scar tissue. While surgery may be necessary to correct this condition, in some cases the use of special traction devices has been shown to be effective in straightening the penis. Read "Peyronie's Disease: Is surgery the only option?" for more information.

Pudendal Nerve Damage

The pudendal nerve, as it relates to the external genital organs, is sometimes subject to damage due to accidents or even from sitting on hard bicycle seats for extended periods of time. It is characterized by pain while sitting and a numbing of the genitals. This condition should not be confused with the loss of sensation caused by permanent nerve damage associated with diabetes or peripheral neuropathy.

Priapism

Priapism is a painful medical condition characterized by a persistent erection of the penis which is not related to sexual arousal. Complications from priapism can include ischaemia, thrombosis and impotence. Although less common, severe cases of this condition can result in gangrene which would make amputation of the penis necessary.

Impotence

Now commonly called "erectile dysfunction", impotence is probably the best known of all penis disorders. Impotence is the inability to reach or maintain an erection and is increasingly common in men over the age of 40. While not a life-threatening condition, impotence does effect sexual performance and can have serious psychological implications.

The penis disorders listed above are just a few of the medical conditions related to the male genitalia. As always, consult your physician if you think you might be experiencing any problem associated with your penis.

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