Huntington's disease

Definition

Huntington's disease is a disorder passed down through families in which nerve cells in the brain waste away, or degenerate.

Alternative Names

Huntington chorea

Causes

American doctor George Huntington first described the disorder in 1872.

Huntington's disease is caused by a genetic defect on chromosome #4. The defect causes a part of DNA, called a CAG repeat, to occur many more times than it is supposed to. Normally, this section of DNA is repeated 10 to 35 times. But in persons with Huntington's disease, it is repeated 36 to 120 times.

As the gene is passed on from one generation to the next, the number of repeats - called a CAG repeat expansion - tend to get larger. The larger the number of repeats, the greater your chance of developing symptoms at an earlier age.

There are two forms of Huntington disease. The most common is adult-onset Huntington's disease. Persons with this form usually develop symptoms in their mid 30s and 40s.

Early-onset form of Huntington disease is less common and begins in childhood or adolescence. Symptoms may resemble those of Parkinson's disease with rigidity, slow movements, and tremor.

If one of your parents has Huntington's disease, you have a 50% chance of getting the gene for the disease. If you get the gene from your parents, you will develop the disease at some point in your life, and can pass it onto your children. If you do not get the gene from your parents, you cannot pass the gene onto your children.

Symptoms

• Abnormal and unusual movements
  • Head turning to shift eye position
  • Facial movements, including grimaces
  • Slow, uncontrolled movements
  • Quick, sudden, jerking movements of arms, legs, face, and other body parts
  • Unsteady gait
• Behavior changes
  • Antisocial behaviors
  • Hallucinations
  • Irritability
  • Moodiness
  • Restlessness or fidgeting
  • Paranoia
  • Psychosis
• Dementia that slowly gets worse, including
  • Loss of memory
  • Loss of judgment
  • Speech changes
  • Personality changes
  • Disorientation or confusion

Additional symptoms that may be associated with this disease:

• Anxiety, stress, and tension
• Difficulty swallowing
• Speech impairment

In children:

• Rigidity
• Slow movements
• Tremor

Exams and Tests

The doctor will perform a physical exam. The doctor may see signs of dementia and abnormal movements. Reflexes may be abnormal. The gait is often "prancing" and wide. Speech may be hesitant or enunciation poor.

A head CT scan may show loss of brain tissue, especially deep in the brain.

Other tests that may show signs of Huntington's disease include:

• Head MRI scan
• PET (isotope) scan of the brain

DNA marker studies may be available to determine if you carry the gene for Huntington's disease.

Treatment

There is no cure for Huntington's disease, and there is no known way to stop the disease from getting worse. The goal of treatment is to slow down the course of the disease and help the person function for as long and as comfortably as possible.

Medications vary depending on the symptoms. Dopamine blockers may help reduce abnormal behaviors and movements. Drugs like tetrabenazine and amantadine are used to try to control extra movements. There has been some evidence to suggest that co-enzyme Q10 may also help slow down the course of the disease.

Depression and suicide are common among persons with Huntington's disease. It is important for all those who care for a person with Huntington's disease to monitor for symptoms and treat accordingly.

There is a progressive need for assistance and supervision, and 24-hour care may eventually be needed.

Support Groups

Huntington's Disease Society of America - www.hdsa.org

Outlook (Prognosis)

Huntington's disease causes progressive disability. Persons with this disease usually die within 15 to 20 years. The cause of death is often infection, although suicide is also common.

It is important to realize that the disease affects everyone differently. The number of CAG repeats may determine how the severity of symptoms. Persons with few repeats may have mild abnormal movements later in life and slow disease progression, while those with large repeats may be severely affected at a young age.

Possible Complications

• Loss of ability to care for self
• Loss of ability to interact
• Injury to self or others
• Increased risk of infection
• Depression
• Death

When to Contact a Medical Professional

Call your health care provider if symptoms of this disorder develop.

Prevention

Genetic counseling is advised if there is a family history of Huntington's disease. Experts also recommend genetic counseling for couples with a family history of this disease who are considering having children.

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Diseases of gums can cause barreness

The Israeli doctors have established possible communication between the bacteria causing diseases of gums, with barreness at men.

Doctor Avigdor Klinberg specializing on gingival diseases in stomatologic branch of hospital, sampled at patients of branch on treatment of barreness.

The research which is spent within three years, has shown, that at 65 % of men whose spermogram had unsatisfactory parameters, diseases of gums also have been detected, informs Rol.ru. However, at 48 % of patients with sick gums, parameters of spermogram were in the full order.

According to doctors, barreness is caused by order 500 types of the bacteria which are being a mouth, and they are capable to get into blood system and to harm to various internal bodies.

Also 50 % of men were revealed, that, in whose sperm in general are absent sperm cells, have chronic illness of gums, and bacteria causing it, also have been found in the sperm.

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Nerve Compression Syndromes

Common nerve compression syndromes include carpal tunnel syndrome, cubital tunnel syndrome, and radial tunnel syndrome. Compression of nerves often causes paresthesias; these paresthesias can often be reproduced by tapping the compressed nerve, usually with the examiner's fingertip (Tinel's sign). Suspected nerve compression can be confirmed by testing nerve conduction velocity and distal latencies, which accurately measure motor and sensory nerve conduction. Initial treatment is usually conservative, but surgical decompression may be necessary if conservative measures fail or if there are significant motor or sensory deficits.

Carpal Tunnel Syndrome

Carpal tunnel syndrome is compression of the median nerve as it passes through the carpal tunnel in the wrist. Symptoms include pain and paresthesias in the median nerve distribution. Diagnosis is suggested by symptoms and signs and is confirmed by nerve conduction velocity testing. Treatments include ergonomic improvements, analgesia, splinting, and sometimes corticosteroid injection or surgery.

Carpal tunnel syndrome is very common and most often occurs in women aged 30 to 50. Risk factors include RA or other wrist arthritis (sometimes the presenting manifestation), diabetes mellitus, hypothyroidism, acromegaly, amyloidosis, hemodialysis, and pregnancy-induced edema in the carpal tunnel. Activities or jobs that require repetitive flexion and extension of the wrist may contribute, but rarely. Most cases are idiopathic.

Symptoms and Signs

Symptoms include pain of the hand and wrist associated with tingling and numbness, classically distributed along the median nerve (the palmar side of the thumb, the index and middle fingers, and the radial half of the ring finger) but possibly involving the entire hand. Typically, the patient wakes at night with burning or aching pain and with numbness and tingling and shakes the hand to obtain relief and restore sensation. Thenar atrophy and weakness of thumb opposition and abduction may develop late.

Diagnosis

• Clinical evaluation
• Nerve conduction testing

The diagnosis is strongly suggested by Tinel's sign, in which median nerve paresthesias are reproduced by tapping at the volar surface of the wrist over the site of the median nerve in the carpal tunnel. Reproduction of tingling with wrist flexion (Phalen's sign) is also suggestive. However, clinical differentiation from other types of peripheral neuropathy may sometimes be difficult. If symptoms are severe or the diagnosis is uncertain, nerve conduction testing should be done on the affected arm for diagnosis and to exclude a more proximal neuropathy.

Treatment

• Splinting
• Sometimes corticosteroid/anesthetic injection
• Sometimes surgical decompression

Changing the position of computer keyboards and making other ergonomic corrections may occasionally provide relief. Otherwise, treatment includes wearing a lightweight neutral wrist splint (see Fig. 4: Hand Disorders: Neutral wrist splint.), especially at night, and taking mild analgesics (eg, acetaminophen, NSAIDs). If these measures fail to control symptoms, a corticosteroid (eg, a mixture of 1.5 mL dexamethasone, 4 mg/mL, and 1.5 mL 1% lidocaine) should be injected into the carpal tunnel at a site just ulnar to the palmaris longus tendon and proximal to the distal crease at the wrist. If bothersome symptoms persist or recur or if hand weakness and thenar wasting develop, the carpal tunnel can be surgically decompressed by using an open or endoscopic technique.

Cubital Tunnel Syndrome

(Ulnar Neuropathy)

Cubital tunnel syndrome is compression or traction of the ulnar nerve at the elbow.

The ulnar nerve is commonly irritated at the elbow or, rarely, the wrist. Cubital tunnel syndrome is most often caused by leaning on the elbow or by prolonged and excessive elbow flexion. It is less common than carpal tunnel syndrome. Baseball pitching (particularly sliders), which can injure the medial elbow ligaments, confers risk.

Symptoms and Signs

Symptoms include numbness and paresthesia along the ulnar nerve distribution (in the ring and little fingers and the ulnar aspect of the hand) and elbow pain. In advanced stages, weakness of the intrinsic muscles of the hand and the flexors of the ring and little fingers may develop. Weakness interferes with pinch between the thumb and index finger and with hand grip.

Diagnosis

Diagnosis is often possible clinically. However, if clinical diagnosis is equivocal and when surgery is being considered, nerve conduction studies are done. Cubital tunnel syndrome is differentiated from ulnar nerve entrapment at the wrist (in Guyon's canal) by the presence of sensory deficits (on sensory testing or with Tinel's sign) over the ulnar dorsal hand and by the presence of ulnar nerve deficits proximal to the wrist on muscle testing or nerve conduction velocity testing.

Treatment

Treatment involves splinting at night, with the elbow extended at 45°, and use of an elbow pad during the day. Surgical decompression can help if conservative treatment fails.

Radial Tunnel Syndrome

(Posterior Interosseous Nerve Syndrome)

Radial tunnel syndrome is compression of the radial nerve in the proximal forearm.

Compression at the elbow can result from trauma, ganglia, lipomas, bone tumors, or radiocapitellar (“elbow”) synovitis.

Symptoms and Signs

Symptoms include lancinating pain in the dorsum of the forearm and lateral elbow. Pain is precipitated by attempted extension of the wrist and fingers and forearm supination. Sensory loss is rare because the radial nerve is principally a motor nerve at this level. This disorder is sometimes confused with backhand tennis elbow (lateral epicondylitis). When weakness of the extensor muscles is the primary finding, the condition is referred to as posterior interosseus nerve palsy.

Diagnosis

Lateral epicondylitis can cause similar tenderness around the lateral epicondyle but does not cause Tinel's sign or tenderness along the course of the radial nerve.

Treatment

Splinting allows avoidance of the forceful or repeated motion of supination or wrist dorsiflexion, reducing pressure on the nerve. If wristdrop or weakened digital extension develops, or conservative treatment fails to provide relief after 3 mo, surgical decompression may be needed.

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OHSU finds association between Epstein-Barr virus, inflammatory diseases of the mouth

2008 NOV 24 -- Researchers at Oregon Health & Science University's School of Dentistry (www.ohsu.edu/sod) have found that a significant percentage of dental patients with the inflammatory diseases irreversible pulpitis and apical periodontitis also have the Epstein-Barr virus. The Epstein-Barr virus is an important human pathogen found in more than 90 percent of the world population. It is associated with many diseases, including infectious mononucleosis, malignant lymphomas, and naspharyngeal carcinoma.

The findings are published online (www.jendodon.com/article/S0099-2399(08)00879-0/abstract) in the Journal of Endodontics, one of the leading peer-reviewed endodontology journals. The study also is expected to be published in the December 2008 (volume 34, issue 12) issue of the Journal of Endodontics.

Although the number of studies examining the role of herpesviruses in oral disease has been increasing, the majority of studies have focused on periodontitis, with no systematic attempt to examine herpesvirus in endodontic patients with varying inflammatory diseases. The OHSU study assessed the presence of human cytomegalovirus (HCMV), Epstein-Barr virus (EPV), herpes simplex virus (HSV-1), and Varicella zoster virus (VZV) in 82 endodontic patients, including patients with irreversible pulpitis and apical periodontitis, and compared them with 19 healthy patients. The goal of the study was to determine the potential association of herpesvirus with clinical symptoms, including acute pain and size of radiographic bone destruction.

Using a variety of methods, the OHSU team found the Epstein-Barr virus DNA and RNA in significantly higher percentages (43.9 percent and 25.6 percent respectively) compared with healthy patients (0 percent). Human cytomegalovirus DNA and RNA were found in measurable numbers in both endodontic patients (15.9 percent and 29.3 percent respectively) and in healthy patients (42.1 percent and 10.5 percent respectively). Herpes simplex virus DNA was found in low percentages of endodontic patients (13.4 percent) and only one patient showed the presence of Varicella zoster virus.

While a previous study examined the incidence of herpes viruses in apical periodontitis, "this is the first time irreversible pulpitis has been analyzed for the presence of herpes viruses and associated with Epstein-Barr virus," noted Curt Machida, Ph.D., OHSU professor of integrative biosciences and principal investigator, whose lab was host for the study. "The incidence of irreversible pulpitis and apical periodontitis, caused by bacteria and possibly the latent herpes virus, is painful and can greatly impair the body's natural immune system. Studies such as ours could someday lead to more effective treatments of inflammatory diseases of the mouth."

Keywords: Carcinoma, Chickenpox, Chronic Fatigue Syndrome, Cytomegalovirus, Dentistry, EBV, Endodontics, Epstein-Barr Virus, HSV-1, Hematology, Herpes Zoster Virus, Herpesvirus, Infectious Disease, Infectious Mononucleosis, Lymphoma, Oncology, Periodontitis, Pulpitis, Varicella Zoster, Varicella-Zoster Virus, Viral Research, Virology, Oregon Health & Science University.

This article was prepared by Clinical Oncology Week editors from staff and other reports. Copyright 2008, Clinical Oncology Week via NewsRx.com.

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Can Seaweed Battle Cancer?

If you read my article about the health effects of ginger, you already know that I am an avid fan of sushi. Having grown up in California where sushi is commonplace and fresh fish is caught by the hour, the delicacy consisting of raw fish, rice, spices, and seaweed, is a luxury we often take for granted. While I usually drool over the ginger, fresh fish, and soy-sauce laden rice, science might be telling me to start appreciating the thin black “skin” of seaweed wrapped around them instead.

While many beachgoers and ocean swimmers get tied up or tangled in the slimy legs of seaweed, the term “seaweed” is actually a blanket word for any variety of algae formed in the sea. Algae comes in red, green, or brown and is harvested for a number of commercial uses. Besides being an additive in some products such as toothpaste, paint, or cosmetics, seaweed is also being developed as a weight-loss tool, fertilizer, as a potential biofuel, and it could be the next “superfood” if more uses are found.

In the food spectrum, seaweed is not just used to wrap sushi or cut up into Asian-style soups. Seaweed is known around the globe as a tasty treat and can be made into pasta like spaghetti in Italy or in a type of bread in Scotland.

Seaweed has been found to be a large source of iodine, an element that is necessary for the health and maintenance of the thyroid gland and when left untreated, can lead to discomfort and possibly a goiter. The strength and elasticity found in strips of this sea-faring organism is sometimes used to dress wounds and has also been documented as used in molds for dental work. Seaweed has been used in many studies hoping to find cures or lessening agents within it against diseases like tuberculosis, annoying ailments such as arthritis or tapeworms, even the common influenza has been tested with seaweed. The results of these tests have been relatively inconclusive and no generalizations could be made.

However, most of the studies in recent years that have been done regarding the effects of seaweed, are against cancer. Hoping to find the ability to shrink tumors with algae, many researchers have devoted years to observing the patterns and data surrounding this important compound.

A 2005 study conducted at the Georgia Institute of Technology found a small strain of red algae off the coast of Fiji called Callophycus serratus, that looks promising for a new, natural cure for cancer. With ten new natural compounds from the strain to work with, researchers found that some of the compounds were born antibacterial fighters and have held up in the lab against known diseases that resist antibiotics. It was said that the natural germ-killers were made by the seaweed to ward off predators and disease. Another compound was able to kill tumor cells in the lab by being able to turn on the suicide switch in the individual cells, which scientists call a head start to a new possible line of anticancer drugs. Georgia Tech biochemists insist that they are only in the “test tube level” and many more studies will be conducted.

Four years ago the researchers speculated that if the studies continue to work, that it may take at least a decade for any of these types of drugs to become available to the public. Another downside to these findings is a common one among researchers, the dosage of the drug would have to be incredibly high in order for it to work and it may do more harm than good for the patients. Another Georgia Tech biologist Mark Hay says that curing cancer is easy, but making it easier on the patient is completely different,

“We can cure cancer with a shotgun, so curing cancer is not the problem…. Curing cancer without harming patients is a whole different thing that's very difficult to figure out. Many compounds have been found to have a negative effect on cancer cells, but almost none of them have gone to market because of adverse side effects that make them unusable.”

While it may be awhile before we are able to take seaweed capsules to ward off cancer or those extra few inches around the middle, it looks like seaweed is a great source of nutrition and shouldn’t be shooed away from the table.

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Multiple Sclerosis Relapse Rate Reduced By Nearly Half With Use of Two New Drugs

Cladribine and fingolimod, two new oral drugs, have been shown to decrease the relapse rate in people who suffer from multiple sclerosis. The drugs could become the first treatments for the disease that does not require regular injections or infusions, if they gain approval by the U.S. Food and Drug Administration (FDA).

Multiple sclerosis (MS) is a chronic and frequently disabling disease that attacks the central nervous system. According to the National Multiple Sclerosis Society, the condition affects about 400,000 Americans and is the most frequent cause of neurological disability other than trauma in people from ages 20 to 50. Symptoms vary among patients and can range from mild to severe and may cause numbness of the limbs, paralysis and even loss of vision. The progression and severity of the disease are unpredictable.

Relapsing/Remitting Multiple Sclerosis (RRMS) is one of four recognized forms of MS. However, approximately 85 percent of people presenting with Multiple Sclerosis are first diagnosed with RRMS. This form of MS is characterized by relapses that may last for days, weeks or even months, during which new symptoms can appear and old symptoms may resurface or worsen. These relapses are followed by periods of remission in which the patient fully or partially recovers from the effects that occur during relapse periods. Recovery may be gradual or practically instantaneous.

In the Phase III study of 1,200 patients with the RRMS, approximately 80 percent of MS patients who took the drug cladribine remained relapse-free for two years compared to only 61 percent of those participants given a placebo. Side effects of cladribine were mild and included headaches, colds, flu, and nausea. The patients were followed for almost two years and monitored with the use of MRI scans. The study participants all had been living with MS for an average of six to seven years, and suffered from at least one relapse in the year prior to the study.

During the study, led by Gavin Giovannoni, M.D., of the Barts and London School of Medicine and Dentistry, participants received either four courses of low-dose cladribine tablets, six courses of higher-dose cladribine tablets, or a placebo. According to Giovannoni, this means, “that individuals with MS only have to take tablets for 8 to 20 days a year. That should improve compliance.”

The study participants who received cladribine were found to be 55 to 58 percent less likely to suffer from a relapse within a year than those given a placebo. In addition, the cladribine recipients were 33 percent less likely to experience deterioration in their disabilities, such as increase in the difficulties of walking. Based on MRI scans, participants taking cladribine were also found to have considerably brain and spinal cord lesions that are typical for MS patients.

Cladribine is already licensed and marketed under the name Leustatin for the treatment of leukemia and is believed to work in the fight against MS by suppressing the autoimmune responses thought to cause the disease. Merck Serono plans to apply for FDA approval within months.

In another successful phase III study of over 1,200 patients with RRMS, 80 to 84 percent of MS patients who were given daily dosages of the drug fingolimod remained relapse-free after one year in comparison to only 67 percent of study participants who received Avonex, a typical injectable drug used to treat MS. Participants in this study had been living with MS for an average of seven years, and all had suffered from an average of two relapses within two years prior to the study. Jeffrey Cohen, M.D., of the Cleveland Clinic, led the study.

Participants taking fingolimod were found to be 38 to 52 percent less likely to suffer from a relapse within a year and also experienced fewer lesions than participants who received the Avonex injections. The length of the study was insufficient to allow conclusions to be drawn on fingolimod’s effects on disabilities. The most common side effects experienced included, headache, head colds and fatigue. However, eight cases of skin cancer as well as four cases of breast cancer did occur and it remains unclear as to whether or not fingolimod was responsible for these events.

Fingolimod also suppresses the autoimmune responses thought to cause MS. There are two other ongoing studies of fingolimod for which results are expected within a few months. The maker of the drug, Novartis, anticipates application to the FDA for approval by the end of this year.

The phase III studies for both cladribine and fingolimod were presented at the annual meeting of the American Academy of Neurology. Because both drugs suppress the autoimmune responses, concerns remain regarding the safety of long-term use, as T cells are necessary for fighting infections, and particularly viral infections. Cladribine damages the ability of the T cells to reproduce and proliferate while fingolimod locks T cells inside the lymph nodes, preventing them from traveling through the bloodstream and into the brain and spinal cord. More studies of both drugs will be required to determine safe measures for their use.

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Tincture Tonics?

When watching an older movie, particularly one that is set prior to 1950, oftentimes terms are used that are rare or extinct in modern day life. Such as when characters would faint in a scene, a smaller role would always be advised to procure the “smelling salts” because the ill person had “the vapors.” The word tincture reminds me of this old-timey speak, although I admit that I did not know exactly what one was. Apparently tinctures are still widely used today and relevant in modern alternative medicine for a variety of ailments.

Common in chemistry classes (a course I was never good in), a tincture is a solution that uses alcohol as the main ingredient acting as a solvent to dissolve the solid portion of the solution. A tincture involves the mixing of the leaves, stems, or crushed seeds of a plant/medicinal herb distilled over time in a high percentage of alcohol (usually 40 to 60, but can also be up to 90 percent ethanol alcohol).

To make a healing tincture, herb bits are placed in a small jar and ethanol is poured over them. After letting this mixture stand for several weeks, while stirring occasionally, your tincture is ready for consumption (but should only be consumed in the event of an actual ailment). This process using alcohol helps to keep the herbs lasting for many years past their normal expiration because they are living compounds. It should be noted that tinctures can also be made with vinegar, although this type doesn’t last as long as the alcohol version.

Easily stored and travel-sized, most tinctures are made in small glass or plastic vials—around one ounce in size—perfect for a first aid kit or toiletries bag. Ready to use, these tinctures are easily administered either under the tongue or a few drops mixed into water, juices, or teas.

If you do not have the resources to purchase a high-quality tincture (they can be expensive depending on the uniqueness of the herb being used, time it took the herbalist, and where you find them), it is just as easy to make one yourself. However, make sure you use a recipe so the percentages are just right and use them with caution and as recommended. Favorite alcohol-based tonics that are made as tinctures are formuated with gin, brandy, and vodka, mainly because they are the top 100-proof alcohols available for purchase. Cheap alcohol will make the tincture less effective, so buy the best you can afford (or the best-tasting in your opinion).

Tinctures are generally used for chronic pain conditions like arthritis and ongoing persistent or recurring ailments such as bronchitis, asthma, menopause, migraines, joint and muscle spasms, stomach and bowel problems such as colitis, irritable bowel syndrome, motion sickness, nausea, tremors associated with Parkinson’s disease, and many others.

These tiny potions can be taken as often as a couple of times a day or just a few times a week or monthly as necessary. Always check with your nutritionist or physician before self-medicating or using a tincture as a complement to Western medical treatments and always make sure the herbs being used or mixed are supposed to combine in order to treat your specific condition. If you happen to be on medication, also check with your healthcare provider in case you are unable to use a tincture based on alcohol content or possibly because of the drug interaction with different herbs.

Next time you watch an old movie and someone with tuberculosis needs a tincture in order to get through his or her day, maybe you (as I will) know what exactly that means, even if you don’t venture to your nearest health food store or attempt to make your own alternative tonic.

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