Contact Dermatitis

Contact dermatitis is a localized rash or irritation of the skin caused by contact with a foreign substance. Only the superficial regions of the skin are affected in contact dermatitis. Inflammation of the affected tissue is present in the epidermis (the outermost layer of skin) and the outer dermis (the layer beneath the epidermis).Unlike contact urticaria, in which a rash appears within minutes of exposure and fades a

way within minutes to hours, contact dermatitis takes days to fade away. Even then, contact dermatitis fades only if the skin no longer comes in contact with the allergen or irritant. Contact dermatitis results in large, burning, and itchy rashes, and these can take anywhere from several days to weeks to heal. Chronic contact dermatitis can develop when the removal of the offending agent no longer provides expected relief.

Causes

In North/South America, the most common causes of allergic contact dermatitis are plants of the Toxicodendron genus: poison ivy, poison oak, and poison sumac. Specific plant species that can induce

such contact dermatitis include Western Poison Oak, a widespread plant in the western USA. Common causes of irritant contact dermatitis are harsh (highly alkaline) soaps, detergents, and cleaning products.

Types of contact dermatitis

There are three types of contact dermatitis: irritant contact, allergic contact, and photocontact dermatitis. Photocontact dermatitis is divided into two categories: phototoxic and photoallergic.

Chemical irritant contact dermatitis

Physical irritant contact dermatitis

Low humidity

Plants

Allergic contact dermatitis


Common allergens implicated include the following:

• Nickel (nickel sulfate hexahydrate) - metal frequently encountered in jewelry and clasps or buttons on clothing
• Gold (gold sodium thiosulfate) - precious metal often found in jewelry
• Balsam of Peru (Myroxylon pereirae) - a fragrance used in perfumes and skin lotions, derived from tree resin (see also Tolu balsam)
• Thimerosal - a mercury compound used in local antiseptics and in vaccines
• Neomycin - a topical antibiotic common in first aid creams and ointments, cosmetics, deodorant, soap and pet food. Found by itself, or in Polysporin or Triple Antibiotic
• Fragrance mix - a group of the eight most common fragrance allergens found in foods, cosmetic products, insecticides, antiseptics, soaps, perfumes and dental products ^[15]
• Formaldehyde - a preservative with multiple uses, e.g., in paper products, paints, medications, household cleaners, cosmetic products and fabric finishes
• Cobalt chloride - metal found in medical products; hair dye; antiperspirant; metal-plated objects such as snaps, buttons or tools; and in cobalt blue pigment
• Bacitracin - a topical antibiotic found by itself, or as Polysporin or Triple Antibiotic
• Quaternium-15 - preservative in cosmetic products (self-tanners, shampoo, nail polish, sunscreen) and in industrial products (polishes, paints and waxes).^[16]
• Colophony (Rosin) - Rosin, sap or sawdust typically from spruce or fir trees
• Topical steroid - see steroid allergy

Photocontact Dermatitis

Sometimes termed "photoaggravated"(Bourke et al. 2001)^[17], and divided into two categories, phototoxic and photoallergic, PCD is the eczematous condition which is triggered by an interaction between an otherwise unharmful or less harmful substance on the skin and ultraviolet light (320-400 nm UVA) (ESCD 2006), therefore manifesting itself only in regions where the sufferer has been exposed to such rays. Without the presence of these rays, the photosensitiser is not harmful. For this reason, this form of contact dermatitis is usually associated only with areas of skin which are left uncovered by clothing. The mechanism of action varies from toxin to toxin, but is usually due to the production of a photoproduct. Toxins which are associated with PCD include the psoralens. Psoralens are in fact used therapeutically for the treatment of psoriasis, eczema and vitiligo.

Photocontact dermatitis is another condition where the distinction between forms of contact dermatitis is not clear cut. Immunological mechanisms can also play a part, causing a response similar to ACD.

Symptoms

Allergic dermatitis is usually confined to the area where the trigger actually touched the skin, whereas irritant dermatitis may be more widespread on the skin. Symptoms of both forms include the following:

• Red rash. This is the usual reaction. The rash appears immediately in irritant contact dermatitis; in allergic contact dermatitis, the rash sometimes does not appear until 24–72 hours after exposure to the allergen.
• Blisters or wheals. Blisters, wheals (welts), and urticaria (hives) often form in a pattern where skin was directly exposed to the allergen or irritant.
• Itchy, burning skin. Irritant contact dermatitis tends to be more painful than itchy, while allergic contact dermatitis often itches.

While either form of contact dermatitis can affect any part of the body, irritant contact dermatitis often affects the hands, which have been exposed by resting in or dipping into a container (sink, pail, tub, Sun, Swimming Pools With High chlorine ), containing the irritant.

Treatment

Self-care at Home

• Immediately after exposure to a known allergen or irritant, wash with soap and cool water to remove or inactivate most of the offending substance.

- Weak acid solutions [lemon juice, vinegar] can be used to counteract the effects of dermatitis contracted by exposure to basic irritants [phenol etc.].

• If blistering develops, cold moist compresses applied for 30 minutes 3 times a day can offer relief.
• Calamine lotion and cool colloidal oatmeal baths may relieve itching.
• Oral antihistamines such as diphenhydramine (Benadryl, Ben-Allergin) can also relieve itching.
• For mild cases that cover a relatively small area, hydrocortisone cream in nonprescription strength may be sufficient.
• Avoid scratching, as this can cause secondary infections.
• A barrier cream such as those containing zinc oxide (e.g. Desitin, etc.) may help to protect the skin and retain moisture.

Medical Care

If the rash does not improve or continues to spread after 2-3 of days of self-care, or if the itching and/or pain is severe, the patient should contact a dermatologist or other physician or physician assistant. Medical treatment usually consists of lotions, creams, or oral medications.

• Corticosteroids. A corticosteroid medication similar to hydrocortisone may be prescribed to combat inflammation in a localized area. This medication may be applied to your skin as a cream or ointment. If the reaction covers a relatively large portion of the skin or is severe, a corticosteroid in pill or injection form may be prescribed.
• Antihistamines. Prescription antihistamines may be given if nonprescription strengths are inadequate.

Prevention

Since contact dermatitis relies on an irritant or an allergen to initiate the reaction, it is important for the patient to identify the responsible agent and avoid it. This can be accomplished by having patch tests, a method commonly known as allergy testing. The patient must know where the irritant or allergen is found to be able to avoid it. It is important to also note that chemicals sometimes have several different names.^[18]

In an industrial setting the employer has a duty of care to the individual worker to provide the correct level of safety equipment to mitigate the exposure to harmful irritants. This can take the form of protective clothing, gloves or barrier cream depending on the working environment.

When Did AIDS Begin? How?

When Did AIDS

Begin?

Reprinted from Times Magazine, By Christine Gorman

Spring 1988

A new study of the oldest known HIV suggests the virus jumped from animals to humans in the 1940s.

The year was 1959, location: The central African city of Leopoldville, now called Kinshasa, shortly before the waves of violent rebellion that followed the liberation of the Belgian Congo. A seemingly healthy man walked into a hospital clinic to give blood for a Western backed study of blood diseases. He walked away and was never heard from again. Doctors analyzed his sample, froze it in a test tube and forgot about it. A quarter-century later, in the mid-1980s, researchers studying the growing AIDS epidemic took a second look at the blood and discovered that it contained HIV, the virus that causes AIDS.

And not just any HIV. The Leopoldville sample is the oldest specimen of the AIDS virus ever isolated and may now help solve the mystery of how and when the virus made the leap from animals (monkeys or chimpanzees) to humans, according to a report published last week in Nature. Dr. David Ho, director of the Aaron Diamond AIDS Research Center in New York City and one of the study's authors, says a careful genetic analysis of the sample's DNA pushes the origin of the AIDS epidemic back at least a decade, to the early '50s or even the '40s.

Over the past 15 years, scientists have identified at least 10 subtypes of the virus. But they couldn't tell whether they were seeing variations on one changeable virus or the handiwork of several different viruses that had made the jump from primates to man. A look at the genetic mutations in the Leopoldville sample strongly suggests that all it took to launch the epidemic was one unlucky turn of events.

By comparing the DNA of the 1959 virus with that of samples taken from the '80s and '90s. Ho and his colleagues constructed a viral family tree in which the Leopoldville isolate sits right at the juncture where three subtypes branch out. The 39-year-old specimen is also strikingly similar to the other seven subtypes. The clear implication: all the viral strains can be traced back to a single event or a closely related group of events. One theory is that AIDS started through contact with infected monkeys in a remote area and spread to the rest of the population through urbanization and mass inoculations.

The findings underscore how rapidly HIV can adapt to its surroundings, making it difficult to develop effective vaccines. No one knows how many more subtypes of HIV will sprout in the next 40 years, but chances are they will be every bit as lethal as the ones we see today, if not more so.

Apert Syndrome : A genetic disorder

Apert Syndrome


Introduction

Apert syndrome is a rare genetic disorder that is characterized by specific craniofacial and limb abnormalities. It is caused by a genetic mutation in the FGFR2 gene on chromosome 10. The mutation can be inherited from a parent who has Apert syndrome or it can be a spontaneous (new) mutation. Studies show that Apert syndrome tends to occur more often in children with older fathers. Furthermore, all new mutations (those that have not been inherited by an affected parent) have been shown to occur exclusively in the FGFR2 gene received by the father. Apert syndrome occurs in 1 out of 100,000 to 160,000 live births and affects males and females equally. The first reported case of the syndrome was in 1848 by S.W. Wheaton, and in 1906, a French physician named E. Apert described nine cases and defined the syndrome.

Features and Characteristics

The following characteristics have been found in children with Apert syndrome:

• Prematurely fused cranial sutures
• Retruded (or sunken) mid-face
• Fused fingers
• Fused toes
• Brachycephaly (short wide head)
• Acrocephaly (high prominent forehead)
• Flattened back of skull
• Prominent eyes - may be spaced widely apart or slant downward
• Strabismus
• Prominent mandible
• Depressed nasal bridge and small anteverted nose
• Down-turned corners of the mouth
• Low set ears (as well as hearing loss)
• Cleft palate
• Severe acne in teens
• Hydrocephalus
• Dental abnormalities (malposition of the teeth, crowding of the teeth, delayed tooth eruption, high-arched narrow palate, thickened ridges that support the teeth)
• Internal organ abnormalities including heart defects and abnormalities of the trachea,
  
• uterus, and brain

Skin manifestations of Apert syndrome

Hyperhidrosis Synonychia Brittle nails Severe acne in puberty Interruption of the eyebrows Hypopigmentation Hyperkeratosis Paronychial infections of plantar skin Excessive skin wrinkling of forehead Dimples at knuckles, shoulders and elbows

Prematurely Fused Cranial Sutures

Retruded Mid-face

Fused Fingers and Toes

Diagnosis

The diagnosis can be made by a skull x-ray, which will confirm premature closure of the skull, and by a clinical exam. The combination of the craniofacial problems and the fused fingers and toes is what distinguishes Apert syndrome from other similar syndromes. Since the defect which causes Apert syndrome has been identified, genetic testing can be provided to confirm a diagnosis.

Treatment

Treating a child with Apert syndrome is best accomplished with a team approach. This would include a craniofacial surgeon, neurosurgeon, ENT specialist, audiologist, speech pathologist, oral surgeon, psychologist, ophthalmologist, and an orthodontist. The majority of treatment methods is surgical and the individual will likely require many operations. Aside from the surgeries required to correct the craniofacial problems and the fused fingers and toes, there may be other potential surgeries to improve the upper airway, address severe eye problems, or correct dental issues.

A child with Cushing syndrome

Allergic Rhitinitis: A seasonal problem

Allergic Rhinitis -2 types: seasonal and Perennial

Seasonal Allergic Rhinitis

Seasonal allergic rhinitis is the medical term for congestion and runny nose caused by exposure to plant allergens, commonly known as hay fever. For most people, spring through fall are the worst seasons for allergies, but symptoms can occur throughout the year.

Causes

1. Pollen Seasonal allergic rhinitis is considered an atopic disease, meaning that it is caused by a genetic predisposition to hypersensitivity to certain substances. In this case, the reaction is to wind-borne pollens. The allergens change with the seasons: tree pollens in spring, grass and weed pollens in summer, and weed pollens in fall. Fungal spores can also cause symptoms. Sufferers may experience hypersensitivity during one or all of these seasons, and will often experience different symptoms in different geographic locations.

Symptoms

1. 
   Swollen eyelid due to allergies
   Seasonal allergic rhinitis is marked by congestion of the nasal mucosa on exposure to the allergen. Itching is also common, especially in the nose, roof of the mouth, throat and eyes. This is often followed by sneezing, tears and clear nasal discharge. The conjunctiva of the eyes may turn red and the eyelids may swell. The nasal lining will appear red and swollen. Some patients will experience headaches, loss of appetite and mood changes. Coughing and wheezing may also occur.

Treatment

2. The best treatment for seasonal allergic rhinitis is to avoid the allergens. However, this is often not possible. Many over-the-counter and prescription drugs are used to treat the symptoms, including antihistamines, eye drops, nasal steroid sprays and in severe cases, systemic steroids.

Natural Remedies

3. Natural remedies for seasonal allergic rhinitis abound. Neti pots, or other forms of nasal lavage, can provide relief from congestion and itchiness of the nose. Herbs used for allergies include eyebright, nettles, yarrow, and Oregon grape root. Some of the many homeopathic remedies include euphrasia, allium, apis and urtica. Acupuncture can also provide relief from allergic symptoms.

Practical Solutions

4. Many allergy sufferers will find relief from making changes in their day-to-day lifestyle. Air purifiers and dust filters can make inside air easier to breathe. The filters on furnaces and air-conditioning units should be changed every 1 to 3 months. Filters come in several grades, and the higher grades--though much more expensive--reduce airborne allergens and improve the quality of indoor air. Carpets, bedspreads and drapes collect dust and allergens, and should be cleaned frequently and carefully. Hardwood floors are a better choice than carpeting for many allergy sufferers.

Cautions

5. Consult a naturopathic doctor, qualified herbal practitioner, homeopath or acupuncturist for further information on the use of natural remedies for allergy relief. If allergy symptoms are accompanied by fever, lethargy, severe headache, productive cough or unusual discharge from the nose or eyes, contact a physician or qualified practitioner immediately.

Swine Flu :How to Protect yourself

preventive measures for Swine flu—

1. The first preventive measure is to avoid contact with the pigs (swine). If you have pigs in your area then please inform the local municipal office so that he can take care of those pigs by keeping them isolated.

2. Swine flu is communicable disease, so use the face masks to protect from the swine flu antigens.

3. Cover your nose and mouth when coughing or sneezing, using tissue when possible. Dispose this tissue by using only once.

4. Avoid visiting the crowded places like theaters and prayer halls. This can be the spreading ground for Swine flu

5. Maintain good hygiene. Wash your hands frequently with soap and water to reduce the spread of virus. It would be better if you use alcohol sanitizers or Dettol for washing hands.

6. Take a special care of children because they easily get infected with the Swine flu. It is okay if you don’t send them to school for few days. Many schools have even announced holidays.

7. Avoid eating outside food because it may be contaminated and may make you infected with the virus.

8. Don’t use the public urinals because many people spit there, which could lead to the spreading of the disease.

9. Drink the boiled water.

Anthrax: Disease or A Biological Weapon

How the Anthrax Spreads.


ANTHRAX: Danger we face



Anthrax is a deadly disease caused by the bacteria Bacillus anthracis. This disease has animal origin and mainly the victims are the grazing animals. However, the disease can spread to humans, who come in contact either with the affected animals or their burial grounds. Anthrax is not a contagious disease and does not spread from person toperson. This disease is known to spread from animals or by bioterrorism
.

Anthrax bacteria have long life and can survive in soil for many years. Wild or domestic animals that graze in or around the soil can get infected while eating rough or irritant vegetation. Such vegetation when eaten causes wound within the gastrointestinal tract and this causes the bacteria to enter into the tissues. The bacteria start multiplying and begin producing toxins which finally leads to death.

Exposure to infected animals or their products such as skin, wool or meat is the route taken by the bacteria to enter the human body. Workers, who work with dead animals or their products, are at a greater risk to this disease. People who are exposed to low levels of these bacteria may not develop the infection as the body’s defense mechanism is strong enough to withstand it. A dangerous form of anthrax through inhalation while sorting wool was prevalent long back and this was known as Wool Sorter’s disease. Now such infections are very rare as the infected animals are not available any more.

Anthrax pores have been used as biological weapons of mass destruction. The last reported use was by the Rhodesian government against cattle and humans during its war with black nationalists in 1978-1979. Anthrax was deliberately spread using the postal system in United States in 2001. This caused 21 cases of Anthrax infection.

Anthrax infection can come through three routes. Through the skin, while handling products of infected animals; through inhalation by inhaling anthrax pores from animal products; or through gastrointestinal path by eating undercooked meat from infected animals.

Porphyria: A Fact about Vampires?

Porphyria - the true story about Vampire

Legend tells us that vampires come out at night. They are night creatures because the sun can hurt and even kill them. They come out at night to seek fresh blood because without it they will suffer agonizing pain and will die. Their bodies dry up due to lack of blood, and new blood refreshes their bodies and gives them energy and certain powers.

It has been long believed that the condition associated with vampire legends is porphyria. Vampire characteristics are similar to those of porphyrics and this may have led to the misconception in the early 1400-1600's that porphyria sufferers were vampires. Vampire legends are in every country and porphyria is also found throughout the world. Porphyria comes from the Greek word meaning purple.

Of course, we now know that porphyria patients are NOT vampires, but porphyria might have contributed to the origin of the vampire legends.

EXPLAINING PORPHYRIA

Porphyria is a group of disorders caused by the abnormal production of heme which is the base material responsible for making hemoglobin and chlorophyll. Most types of porphyria are inherited. A child needs to inherit the defective gene from only one parent to develop the disease.

Heme is a substance found in all body tissues. The largest amounts of heme are found in the blood and bone marrow, and heme is also found in the liver and red blood cells. Multiple enzymes are required for the body to convert chemical compounds called porphyrins into heme. If any of the enzymes are abnormal, the process is disturbed and cannot continue. This allows the porphyrins to build up in the body.

Excessive porphyrin in the body causes photosensitivity which is oversensitivity to sunlight. When porphyrins are exposed to light and oxygen, they generate a charged, unstable form of oxygen that can damage the skin. Nerve damage, pain and paralysis can occur in some porphyrias. Sometimes an attack is so severe it can also lead to respiratory paralysis and the patient is unable to speak, breath or swallow. At times, this if fatal.


ABOUT:
Congenital Erythropoietic Porphyria - Hypersensitivity to Light

Fewer than 200 cases of congenital erythropoietic porphyria have ever been documented, and not just because physicians can’t pronounce the name. Due to a gene mutation, the skin becomes extremely sensitive to sunlight. Areas of exposed skin can become blistered and infected. Sunlight exposure can also lead to scarring, changes in skin pigmentation and increased hair growth. Such symptoms have unfairly linked people suffering from the condition with the lore of vampires and werewolves. On overcast or very cold winter days, the symptoms of congenital erythropoietic porphyria (also called erythropoietic protoporphyria) are sometimes attenuated, allowing some safe exposure to indirect sunlight.

Synesthesia is not considered to be a disease (though it has not been well studied, either) and tends to affect people who are bright and colorful—er, that is, people who are intelligent and creative

Skin Cancer Photos

Swine Flu Alert : India And Nepal

Swine Flu Now In South Asia

Current Pandemic Level : 6

The WHO has raised the Influenza Pandemic Alert to the highest level which is 6. Already India has seen over 40 confirmed cases of Swine Flu and the threat of a full blown epidemic in India is very real. The best we citizens can do is keep ourselves informed about the happenings and the steps we can take to prevent the spread of the flu. With most of the affected people fitting the profile of a person with internet access, we believe the internet is the first place that people would come looking for information like symptoms, who to contact etc. Swine Flu India is an attempt to bring all the necessary information in one place. India cannot afford to fall sick. Keep yourself and others informed.

Some Vital Stats

Total Confirmed Cases in India		959**
Total Deaths in India		10**
Total Confirmed Cases Worldwide		162380*
Total Deaths Worldwide		1154*
Last Updated Date and Time		11/08/2009 19:26 IST
Sources : *WHO **MoHFW		Nepal = 20 cases Reported

Swine Flu : CDC Recommendation

Novel H1N1 Vaccination Recommendations

With the new H1N1 virus continuing to cause illness, hospitalizations and deaths in the US during the normally flu-free summer months and some uncertainty about what the upcoming flu season might bring, CDC's Advisory Committee on Immunization Practices has taken an important step in preparations for a voluntary novel H1N1 vaccination effort to counter a possibly severe upcoming flu season. On July 29, ACIP met to consider who should receive novel H1N1 vaccine when it becomes available.

Novel H1N1 Vaccine

Every flu season has the potential to cause a lot of illness, doctor’s visits, hospitalizations and deaths. CDC is concerned that the new H1N1 flu virus could result in a particularly severe flu season this year. Vaccines are the best tool we have to prevent influenza. CDC hopes that people will start to go out and get vaccinated against seasonal influenza as soon as vaccines become available at their doctor’s offices and in their communities (this may be as early as August for some). The seasonal flu vaccine is unlikely to provide protection against novel H1N1 influenza. However a novel H1N1 vaccine is currently in production and may be ready for the public in the fall. The novel H1N1 vaccine is not intended to replace the seasonal flu vaccine – it is intended to be used along-side seasonal flu vaccine.

CDC’s Advisory Committee on Immunization Practices (ACIP), a

panel made up of medical and public health experts, met July 29, 2009, to make recommendations on who should receive the new H1N1 vaccine when it becomes available. While some issues are still unknown, such as how severe the virus will be during the fall and winter months, the ACIP considered several factors, including current disease patterns, populations most at-risk for severe illness based on current trends in illness, hospitalizations and deaths, how much vaccine is expected to be available, and the timing of vaccine availability.

The groups recommended to receive the novel H1N1 influenza vaccine include:

• Pregnant women because they are at higher risk of complications and can potentially provide protection to infants who cannot be vaccinated;
• Household contacts and caregivers for children younger than 6 months of age because younger infants are at higher risk of influenza-related complications and cannot be vaccinated. Vaccination of those in close contact with infants less than 6 months old might help protect infants by “cocooning” them from the virus;
• Healthcare and emergency medical services personnel because infections among healthcare workers have been reported and this can be a potential source of infection for vulnerable patients. Also, increased absenteeism in this population could reduce healthcare system capacity;
• All people from 6 months through 24 years of age
  • Children from 6 months through 18 years of age because we have seen many cases of novel H1N1 influenza in children and they are in close contact with each other in school and day care settings, which increases the likelihood of disease spread, and
  • Young adults 19 through 24 years of age because we have seen many cases of novel H1N1 influenza in these healthy young adults and they often live, work, and study in close proximity, and they are a frequently mobile population; and,
• Persons aged 25 through 64 years who have health conditions associated with higher risk of medical complications from influenza.

We do not expect that there will be a shortage of novel H1N1 vaccine, but flu vaccine availability and demand can be unpredictable and there is some possibility that initially, the vaccine will be available in limited quantities. So, the ACIP also made recommendations regarding which people within the groups listed above should be prioritized if the vaccine is initially available in extremely limited quantities. For more information see the CDC press release CDC Advisors Make Recommendations for Use of Vaccine Against Novel H1N1.

Once the demand for vaccine for the prioritized groups has been met at the local level, programs and providers should also begin vaccinating everyone from the ages of 25 through 64 years. Current studies indicate that the risk for infection among persons age 65 or older is less than the risk for younger age groups. However, once vaccine demand among younger age groups has been met, programs and providers should offer vaccination to people 65 or older.

Health Hazards of Radiation

Effect Of Hiroshima Bombing
Radiation Induced Health Hazards

Hiroshima Bombing: 6 August 1945

THE ATOMIC BOMBING OF HIROSHIMA
Hiroshima (August 6, 1945)


In the early morning hours of August 6, 1945, a B-29 bomber named Enola Gay took off from the island of Tinian and headed north by northwest toward Japan. The bomber's primary target was the city of Hiroshima, located on the deltas of southwestern Honshu Island facing the Inland Sea. Hiroshima had a civilian population of almost 300,000 and was an important military center, containing about 43,000 soldiers.

The bomber, piloted by the commander of the 509th Composite Group, Colonel Paul Tibbets, flew at low altitude on automatic pilot before climbing to 31,000 feet as it neared the target area. At approximately 8:15 a.m. Hiroshima time the Enola Gay released "Little Boy," its 9,700-pound uranium bomb, over the city. Tibbets immediately dove away to avoid the anticipated shock wave. Forty-three seconds later, a huge explosion lit the morning sky as Little Boy detonated 1,900 feet above the city, directly over a parade field where soldiers of the Japanese Second Army were doing calisthenics. Though already eleven and a half miles away, the Enola Gay was rocked by the blast. At first, Tibbets thought he was taking flak. After a second shock wave (reflected from the ground) hit the plane, the crew looked back at Hiroshima. "The city was hidden by that awful cloud . . . boiling up, mushrooming, terrible and incredibly tall," Tibbets recalled. The yield of the explosion was later estimated at 15 kilotons (the equivalent of 15,000 tons of TNT).

On the ground moments before the blast it was a calm and sunny Monday morning. An air raid alert from earlier that morning had been called off after only a solitary aircraft was seen (the weather plane), and by 8:15 the city was alive with activity -- soldiers doing their morning calisthenics, commuters on foot or on bicycles, groups of women and children working outside to clear firebreaks. Those closest to the explosion died instantly, their bodies turned to black char. Nearby birds burst into flames in mid-air, and dry, combustible materials such as paper instantly ignited as far away as 6,400 feet from ground zero. The white light acted as a giant flashbulb, burning the dark patterns of clothing onto skin (right) and the shadows of bodies onto walls. Survivors outdoors close to the blast generally describe a literally blinding light combined with a sudden and overwhelming wave of heat. (The effects of radiation are usually not immediately apparent.) The blast wave followed almost instantly for those close-in, often knocking them from their feet. Those that were indoors were usually spared the flash burns, but flying glass from broken windows filled most rooms, and all but the very strongest structures collapsed. One boy was blown through the windows of his house and across the street as the house collapsed behind him. Within minutes 9 out of 10 people half a mile or less from ground zero were dead.

People farther from the point of detonation experienced first the flash and heat, followed seconds later by a deafening boom and the blast wave. Nearly every structure within one mile of ground zero was destroyed, and almost every building within three miles was damaged. Less than 10 percent of the buildings in the city survived without any damage, and the blast wave shattered glass in suburbs twelve miles away. The most common first reaction of those that were indoors even miles from ground zero was that their building had just suffered a direct hit by a bomb. Small ad hoc rescue parties soon began to operate, but roughly half of the city's population was dead or injured. In those areas most seriously affected virtually no one escaped serious injury. The numerous small fires that erupted simultaneously all around the city soon merged into one large firestorm, creating extremely strong winds that blew towards the center of the fire. The firestorm eventually engulfed 4.4 square miles of the city, killing anyone who had not escaped in the first minutes after the attack. One postwar study of the victims of Hiroshima found that less than 4.5 percent of survivors suffered leg fractures. Such injuries were not uncommon; it was just that most who could not walk were engulfed by the firestorm.

Even after the flames had subsided, relief from the outside was slow in coming. For hours after the attack the Japanese government did not even know for sure what had happened. Radio and telegraph communications with Hiroshima had suddenly ended at 8:16 a.m., and vague reports of some sort of large explosion had begun to filter in, but the Japanese high command knew that no large-scale air raid had taken place over the city and that there were no large stores of explosives there. Eventually a Japanese staff officer was dispatched by plane to survey the city from overhead, and while he was still nearly 100 miles away from the city he began to report on a huge cloud of smoke that hung over it. The first confirmation of exactly what had happened came only sixteen hours later with the announcement of the bombing by the United States. Relief workers from outside the city eventually began to arrive and the situation stabilized somewhat. Power in undamaged areas of the city was even restored on August 7th, with limited rail service resuming the following day. Several days after the blast, however, medical staff began to recognize the first symptoms of radiation sickness among the survivors. Soon the death rate actually began to climb again as patients who had appeared to be recovering began suffering from this strange new illness. Deaths from radiation sickness did not peak until three to four weeks after the attacks and did not taper off until seven to eight weeks after the attack. Long-range health dangers associated with radiation exposure, such as an increased danger of cancer, would linger for the rest of the victims' lives, as would the psychological effects of the attack.

No one will ever know for certain how many died as a result of the attack on Hiroshima. Some 70,000 people probably died as a result of initial blast, heat, and radiation effects. This included about twenty American airmen being held as prisoners in the city. By the end of 1945, because of the lingering effects of radioactive fallout and other after effects, the Hiroshima death toll was probably over 100,000. The five-year death total may have reached or even exceeded 200,000, as cancer and other long-term effects took hold.

At 11:00 a.m., August 6 (Washington D.C. time), radio stations began playing a prepared statement from President Truman (right) informing the American public that the United States had dropped an entirely new type of bomb on the Japanese city of Hiroshima -- an "atomic bomb." Truman warned that if Japan still refused to surrender unconditionally, as demanded by the Potsdam Declaration of July 26, the United States would attack additional targets with equally devastating results. Two days later, on August 8, the Soviet Union declared war on Japan and attacked Japanese forces in Manchuria, ending American hopes that the war would end before Russian entry into the Pacific theater. By August 9th, American aircraft were showering leaflets all over Japan informing its people that "We are in possession of the most destructive explosive ever devised by man. A single one of our newly developed atomic bombs is actually the equivalent in explosive power to what 2,000 of our giant B-29s can carry on a single mission. This awful fact is one for you to ponder and we solemnly assure you it is grimly accurate. We have just begun to to use this weapon against your homeland. If you still have any doubt, make inquiry as to what happened to Hiroshima when just one atomic bomb fell on that city." Meanwhile, Tibbets's bomber group was simply waiting for the weather to clear in order to drop its next bomb, the plutonium weapon nicknamed "Fat Man" (right) that was destined for the city of Nagasaki.

Neurocysticercosis: Tanea Solium

Tanea Solium: Devastaiting effect.

Introduction
Neurocysticercosis (NCC) is the most common parasitic disease of the nervous system and is the main cause of acquired epilepsy in developing countries. Lately, it has also been a problem in industrialized countries because of immigration of tapeworm carriers from areas of endemic disease. Tanae Solium.

Clinical Features:
History
NCC is a pleomorphic disease, although it sometimes produces no clinical manifestation. This pleomorphism is due to variations in the locations of the lesions, the number of parasites, and the host's immune response.



•Many patients are asymptomatic; others report vague symptoms such as headache or dizziness.
•The onset of symptoms is usually subacute to chronic, with the exception of seizures, which present in an acute fashion. Patients may present with the following:

◦Epilepsy
■Epilepsy is the most common presentation (70%)
■Seizures secondary to NCC may be generalized or partial.

◦Headache
■Chronic headaches associated with nausea and vomiting (simulating migraines)
■Headaches associated with intracranial hypertension and indicative of hydrocephalus
■Headaches due to meningitis

◦Intracranial hypertension
■Most often, intracranial hypertension is due to obstruction of cerebrospinal fluid (CSF) circulation caused by basal or ventricular cysticercosis. It may also result from large cysts displacing midline structures, granular ependymitis, arachnoiditis, or the so-called cysticercotic encephalitis caused by the inflammatory response to a massive infestation of cerebral parenchyma with cysticerci.
■These patients may have seizures and deterioration of their mental status, mainly due to the host's inflammatory reaction as an exaggerated response to the massive infestation.

◦Strokes5
■Ischemic cerebrovascular complications of NCC include lacunar infarcts6 and large cerebral infarcts due to occlusion or vascular damage.
■Hemorrhage also can occur, and has been reported as a result of rupture of mycotic aneurysms of the basilar artery.
■Strokes may be responsible for the following signs and symptoms: paresis or plegias, involuntary movements, gait disturbances, or paresthesias.

◦Neuropsychiatric disturbances
■These range from poor performance on neuropsychological tests to severe dementia.
■These symptoms appear to be related more to the presence of intracranial hypertension than to the number or location of parasites in the brain.

◦Diplopia: This is a result of intracranial hypertension or arachnoiditis producing entrapment or compression of cranial nerves III, IV, or VI.

◦Hydrocephalus
■Ten to thirty percent of patients with NCC develop communicating hydrocephalus due to inflammation and fibrosis of the arachnoid villi or inflammatory reaction to the meninges and subsequent occlusion of the foramina of Luschka and Magendie.
■Noncommunicating hydrocephalus may be a consequence of intraventricular cysts.
•Other forms of neurocysticercosis

◦Ocular cysticercosis: This occurs most commonly in the subretinal space. Patients may present with decreased visual acuity, visual field defects, or monocular blindness.

◦Systemic cysticercosis: This is most common in the Asian continent. The parasites may be located in the subcutaneous tissue or muscle. Peripheral nerve involvement as well as involvement of liver or spleen have been reported.

Physical
Twenty percent or less of infected patients have abnormal neurological findings. Physical findings will depend on where the cyst is located in the nervous system and include the following:

•Cognitive decline
•Dysarthria
•Extraocular movement palsy or paresis
•Hemiparesis or hemiplegia, which may be related to stroke, or Todd paralysis
•Hemisensory loss
•Movement disorders
•Hyper/hyporeflexia
•Gait disturbances
•Meningeal signs

Causes
NCC can be acquired via fecal-oral contact with carriers of the adult tapeworm. This usually indicates the presence of a tapeworm carrier in the immediate environment (ie, household) or by accidental ingestion of contaminated food. Cases of autoingestion, in which persons with teniasis may ingest the eggs of T solium into their intestine, have been reported.

Laboratory Studies
•CSF analysis
◦Analysis of the CSF is indicated in every patient presenting with new-onset seizures or neurological deficit in whom neuroimaging shows a solitary lesion but does not offer a definitive diagnosis.
Eosinophilia in the CSF suggests neurocysticercosis (NCC); however, eosinophils also are elevated in neurosyphilis and tuberculosis of the CNS.

•Stool examination
◦Taeniasis and NCC coexist in 10-15% of patients with NCC. A recent study found that intestinal taeniasis is very common in patients with massive infestation with cysticerci but without cysticercotic encephalitis.
◦Tapeworm carriers may be identified by examining the stool of the relatives of a patient with cysticercosis encephalitis.

•Immunological tests
◦Enzyme-linked immunosorbent assay (ELISA) is the most widely used test of CSF; it has a sensitivity of 50% and a specificity of 65% for NCC.

Imaging Studies
•CT scan

•MRI

considering biopsy.

Medical Care
Treatment of neurocysticercosis depends upon the viability of the cyst and its complications. Management includes symptomatic treatment as well as treatment directed against the parasite.

•If the parasite is dead, the treatment is directed primarily against the symptoms (eg, anticonvulsants for management of seizures). Monotherapy is usually sufficient. Duration of the treatment remains undefined, and depends neither on the type of seizure at presentation nor on other risk factors for recurrence, such as age at onset and number of seizures before diagnosis. Calcification remains an epileptogenic focus. Treating patients with viable cysts with a course of anticysticercal drugs in order to achieve better control of seizures is common practice.
•If the parasite is viable or active and the patient has vasculitis, arachnoiditis, or encephalitis, a course of steroids or immunosuppressants is recommended before the use of anticysticercal drugs. Antiparasitic treatment8 with albendazole is also useful in cysticercosis of the racemose type. If only parenchymal, subarachnoid, or spinal cysts are present without the complications mentioned, anticysticercal treatment can be considered, with the concomitant use of steroids, even in patients with massive brain infection. Reports indicate that multiple trials with anticysticercal treatment may be required for giant subarachnoid cysts.
•A recent double-blind, placebo-controlled study has shown that in patients with seizures due to viable parenchymal cysts, antiparasitic therapy decreases the burden of parasites and is safe and effective, at least in reducing the number of seizures with generalization.

Surgical Care
•In the presence of hydrocephalus due to intraventricular cyst, placement of a ventricular shunt is recommended, followed by surgical extirpation of the cyst and subsequent medical treatment.
•In cases of multiple cysts in the subarachnoid space (ie, the racemose form), surgical extirpation, on an urgent basis, is recommended.
•If the obstruction is due to arachnoiditis, placement of a ventricular shunt should be followed by administration of steroids and subsequent medical therapy.
•Because of frequent shunt dysfunctions due to entry of inflammatory tissue as well as parasitic debris inside the ventricular cavities, Sotelo designed a device that functions at a constant flow without the valvular mechanism of Pudenz-type shunts.
•Neuroendoscopy is a new tool with great potential for use in the management of ventricular cysticercosis.
•Surgical treatment in the particular case of medically refractory epilepsy due to a single lesion has been reported. Evaluation in an epilepsy center is indicated.

Alzeimer's Disease or just a poor Memroy?

Alzheimer's Disease Fact Sheet

Alzheimer’s disease (AD) is an irreversible, progressive brain disease that slowly destroys memory and thinking skills, and eventually even the ability to carry out the simplest tasks. In most people with AD, symptoms first appear after age 60.

AD is the most common cause of dementia among older people. Dementia is the loss of cognitive functioning—thinking, remembering, and reasoning—to such an extent that it interferes with a person’s daily life and activities. According to recent estimates, as many as 2.4 to 4.5 million Americans are living with AD.

AD is named after Dr. Alois Alzheimer. In 1906, Dr. Alzheimer noticed changes in the brain tissue of a woman who had died of an unusual mental illness. Her symptoms included memory loss, language problems, and unpredictable behavior. After she died, he examined her brain and found many abnormal clumps (now called amyloid plaques) and tangled bundles of fibers (now called neurofibrillary tangles). Plaques and tangles in the brain are two of the main features of AD. The third is the loss of connections between nerve cells (neurons) in the brain.

Changes in the Brain in AD

Although we still don’t know what starts the AD process, we do know that damage to the brain begins as many as 10 to 20 years before any problems are evident. Tangles begin to develop deep in the brain, in an area called the entorhinal cortex, and plaques form in other areas. As more and more plaques and tangles form in particular brain areas, healthy neurons begin to work less efficiently. Then, they lose their ability to function and communicate with each other, and eventually they die. This damaging process spreads to a nearby structure, called the hippocampus, which is essential in forming memories. As the death of neurons increases, affected brain regions begin to shrink. By the final stage of AD, damage is widespread and brain tissue has shrunk significantly.

Very Early Signs and Symptoms

Memory problems are one of the first signs of AD. Some people with memory problems have a condition called amnestic mild cognitive impairment (MCI). People with this condition have more memory problems than normal for people their age, but their symptoms are not as severe as those with AD. More people with MCI, compared with those without MCI, go on to develop AD.

Other changes may also signal the very early stages of AD. For example, recent research has found links between some movement difficulties and MCI. Researchers also have seen links between some problems with the sense of smell and cognitive problems. Brain imaging and biomarker studies of people with MCI and those with a family history of AD are beginning to detect early changes in the brain like those seen in AD. These findings will need to be confirmed by other studies but appear promising. Such findings offer hope that some day, we may have tools that could help detect AD early, track the course of the disease, and monitor response to treatments.

Mild AD

As AD progresses, memory loss continues and changes in other cognitive abilities appear. Problems can include getting lost, trouble handling money and paying bills, repeating questions, taking longer to complete normal daily tasks, poor judgment, and mood and personality changes. People often are first diagnosed in this stage.

Moderate AD

In this stage, damage occurs in areas of the brain that control language, reasoning, sensory processing, and conscious thought. Memory loss and confusion increase, and people begin to have problems recognizing family and friends. They may be unable to learn new things, carry out tasks that involve multiple steps (such as getting dressed), or cope with new situations. They may have hallucinations, delusions, and paranoia, and may behave impulsively.

Severe AD

By the final stage, plaques and tangles have spread throughout the brain and brain tissue has shrunk significantly. People with severe AD cannot communicate and are completely dependent on others for their care. Near the end, the person may be in bed most or all of the time as the body shuts down.

What Causes AD

Scientists don’t yet fully understand what causes AD, but it is clear that it develops because of a complex series of events that take place in the brain over a long period of time. It is likely that the causes include genetic, environmental, and lifestyle factors. Because people differ in their genetic make-up and lifestyle, the importance of these factors for preventing or delaying AD differs from person to person.

The Basics of AD

Scientists are conducting studies to learn more about plaques, tangles, and other features of AD. They can now visualize plaques by imaging the brains of living individuals. They are also exploring the very earliest steps in the disease process. Findings from these studies will help them understand the causes of AD.

One of the great mysteries of AD is why it largely strikes older adults. Research on how the brain changes normally with age is shedding light on this question. For example, scientists are learning how age-related changes in the brain may harm neurons and contribute to AD damage. These age-related changes include inflammation and the production of unstable molecules called free radicals.

Genetics

In a very few families, people develop AD in their 30s, 40s, and 50s. These people have a mutation, or permanent change, in one of three genes that they inherited from a parent. We know that these gene mutations cause AD in these “early-onset” familial cases.

However, most people with AD have “late-onset” AD, which usually develops after age 60. Many studies have linked a gene called APOE to late-onset AD. This gene has several forms. One of them, APOE ε4, increases a person’s risk of getting the disease. About 40 percent of all people who develop late-onset AD carry this gene. However, carrying the APOE ε4 form of the gene does not necessarily mean that a person will develop AD, and people carrying no APOE ε4 forms can also develop AD.

Lifestyle Factors

A nutritious diet, exercise, social engagement, and mentally stimulating pursuits can all help people stay healthy. New research suggests the possibility that these factors also might help to reduce the risk of cognitive decline and AD. Scientists are investigating associations between cognitive decline and heart disease, high blood pressure, diabetes, and obesity. Understanding these relationships and testing them in clinical trials will help us understand whether reducing risk factors for these diseases may help with AD as well.

How AD Is Diagnosed

AD can be definitively diagnosed only after death by linking clinical course with an examination of brain tissue and pathology in an autopsy. But doctors now have several methods and tools to help them determine fairly accurately whether a person who is having memory problems has “possible AD” (the symptoms may be due to another cause) or “probable AD” (no other cause for the symptoms can be found). To diagnose AD, doctors:

• ask questions about the person’s overall health, past medical problems, ability to carry out daily activities, and changes in behavior and personality
• conduct tests of memory, problem solving, attention, counting, and language
• carry out medical tests, such as tests of blood, urine, or spinal fluid
• perform brain scans, such as a computerized tomography (CT) scan or a magnetic resonance imaging (MRI) test

These tests may be repeated to give doctors information about how the person’s memory is changing over time.

Early diagnosis is beneficial for several reasons. Having an early diagnosis and starting treatment in the early stages of the disease can help preserve function for months to years, even though the underlying AD process cannot be changed. Having an early diagnosis also helps families plan for the future, make living arrangements, take care of financial and legal matters, and develop support networks.

In addition, an early diagnosis can provide greater opportunities for people to get involved in clinical trials. In a clinical trial, scientists test drugs or treatments to see which are most effective and for whom they work best. (See the box, below, for more information.)

How AD Is Treated

AD is a complex disease, and no single “magic bullet” is likely to prevent or cure it. That’s why current treatments focus on several different aspects, including helping people maintain mental function; managing behavioral symptoms; and slowing, delaying, or preventing AD.

Helping People with AD Maintain Mental Function

Four medications are approved by the U.S. Food and Drug Administration to treat AD. Donepezil (Aricept®), rivastigmine (Exelon®), and galantamine (Razadyne®) are used to treat mild to moderate AD (donepezil can be used for severe AD as well). Memantine (Namenda®) is used to treat moderate to severe AD. These drugs work by regulating neurotransmitters (the chemicals that transmit messages between neurons). They may help maintain thinking, memory, and speaking skills, and help with certain behavioral problems. However, these drugs don’t change the underlying disease process and may help only for a few months to a few years.

Managing Behavioral Symptoms

Common behavioral symptoms of AD include sleeplessness, agitation, wandering, anxiety, anger, and depression. Scientists are learning why these symptoms occur and are studying new treatments—drug and non-drug—to manage them. Treating behavioral symptoms often makes people with AD more comfortable and makes their care easier for caregivers.

Slowing, Delaying, or Preventing AD

AD research has developed to a point where scientists can look beyond treating symptoms to think about addressing the underlying disease process. In ongoing AD clinical trials, scientists are looking at many possible interventions, such as cardiovascular treatments, antioxidants, immunization therapy, cognitive training, and physical activity.

Supporting Families and Caregivers

Caring for a person with AD can have high physical, emotional, and financial costs. The demands of day-to-day care, changing family roles, and difficult decisions about placement in a care facility can be hard to handle. Researchers are learning a lot about AD caregiving, and studies are helping experts develop new ways to support caregivers.

Becoming well-informed about AD is one important long-term strategy. Programs that teach families about the various stages of AD and about flexible and practical strategies for dealing with difficult caregiving situations provide vital help to those who care for people with AD.

Developing good coping skills and a strong support network of family and friends also are important ways that caregivers can help themselves handle the stresses of caring for a loved one with AD. For example, staying physically active provides physical and emotional benefits. Some AD caregivers have found that participating in an AD support group is a critical lifeline. These support groups allow caregivers to find respite, express concerns, share experiences, get tips, and receive emotional comfort. The Alzheimer’s Association, Alzheimer’s Disease Centers, and many other organizations sponsor in-person and online AD support groups across the country. There are a growing number of groups for people in the early stage of AD and their families. Support networks can be especially valuable when caregivers face the difficult decision of whether and when to place a loved one in a nursing home.

House, MD: Season 5

Season 5 of House MD began airing on September 16, 2008. From September to December 2008, it aired every Tuesday at 8.00 PM EST. House MD was moved to Monday 8.00 PM EST starting January 19, 2009. The season finale “Both Sides Now” was shown last May 11, 2009. The season finale was really a cliffhanger. House thought all along that he slept with Cuddy. But the fact is Cuddy never went home with him and he spent the night popping Vicodin instead of detoxing.

Kutner appeared, presumably for the last time, as part of House’s hallucination. The hallucination arc has been on going for three episodes and the finale is the best among the hallucination story arc.

No explanation on Kutner’s suicide. I guess the writers would just leave it as that. Kutner’s suicide is really a blow to all House MD fans. House checked in a rehab facility and I guess this is where Season 6 would begin.

The link for the Season 5 finale is up. Again thanks to Simon of HouseMDVideos.com for providing excellent online links to all the House MD episodes. We would have to wait for at least 4 more months for Season 6. But until then, catch all the Season 5 episodes below.

Season 5: September 16, 2008 to present

1. Dying Changes Everything
2. Not Cancer
3. Adverse Events
4. Birthmarks
5. Lucky Thirteen
6. Joy
7. The Itch
8. Emancipation
9. Last Resort
10. Let Them Eat Cake
11. Joy to the World
12. Painless
13. Big Baby
14. The Greater Good
15. Unfaithful
16. The Softer Side
17. The Social Contract
18. Here Kitty
19. Locked In
20. Simple Explanation
21. Saviors
22. House Divided
23. Under My Skin
24. Both Sides Now

Acromegaly: Know it Before it Know you

Acromegaly

Acromegaly is the Greek word for "extremities" and "enlargement." When the pituitary gland produces excess growth hormones, this results in excessive growth - called acromegaly. The excessive growth occurs first in the hands and feet, as soft tissue begins to swell. Acromegaly affects mostly middle-aged adults. Untreated, the disease can lead to severe illness and death.

symptoms

Symptoms of acromegaly vary depending on how long the patient has had the disease. The following are the most common symptoms of acromegaly. However, each individual may experience symptoms differently. Symptoms may include:

• swelling of the hands and feet
• facial features become coarse as bones grow
• body hair becomes coarse as the skin thickens and/or darkens
• increased perspiration accompanied with body odor
• protruding jaw
• voice deepening
• enlarged lip, nose, and tongue
• thickened ribs (creating a barrel chest)
• joint pain
• degenerative arthritis
• enlarged heart
• enlargement of other organs
• strange sensations and weakness in arms and legs
• snoring
• fatigue and weakness
• headaches
• loss of vision
• irregular menstrual cycles in women
• breast milk production in women
• impotence in men

The symptoms of acromegaly may resemble other conditions or medical problems. Always consult your physician for a diagnosis.

diagnosis

Due to the subtlety of the symptoms, acromegaly is often not diagnosed until years later. In addition to a complete medical history and medical examination, diagnostic procedures for acromegaly may include:

• serial photos taken over the years (to observe physical changes in the patient)
• x-rays (to detect bone thickening)
• blood tests (to check the growth hormone level)

Treatment for acromegaly:

Specific treatment for acromegaly will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

Treatment of acromegaly depends on the cause of the disease. Ninety percent of acromegaly cases are caused by benign tumors on the pituitary gland. Because the tumor is compressing the pituitary gland, the hormone production can be altered. Some other acromegaly cases are caused by tumors of the pancreas, lungs, or adrenal glands.

The goal of treatment is to restore the pituitary gland to normal function, producing normal levels of growth hormone.

Treatment may include removal of the tumor, radiation therapy, and injection of a growth hormone blocking drug.

Left untreated, acromegaly can lead to diabetes mellitus and hypertension. The disease also increases a patient's risk for cardiovascular disease and colon polyps that may lead to cancer.

Anencephaly: Newborn Deformity

ANENCEPHALY
is a neural tube defect (a disorder involving incomplete development of the brain, spinal cord, and/or their protective coverings). The neural tube is a narrow sheath that folds and closes between the 3rd and 4th weeks of pregnancy to form the brain and spinal cord of the embryo. Anencephaly occurs when the "cephalic" or head end of the neural tube fails to close, resulting in the absence of a major portion of the brain, skull, and scalp. Infants with this disorder are born without both a forebrain (the front part of the brain) and a cerebrum (the thinking and coordinating area of the brain). The remaining brain tissue is often exposed--not covered by bone or skin. The infant is usually blind, deaf, unconscious, and unable to feel pain. Although some individuals with anencephaly may be born with a rudimentary brain stem, the lack of a functioning cerebrum permanently rules out the possibility of ever gaining consciousness. Reflex actions such as respiration (breathing) and responses to sound or touch may occur. The cause of anencephaly is unknown. Although it is believed that the mother's diet and vitamin intake may play a role, scientists believe that many other factors are also involved.

treatment

There is no cure or standard treatment for anencephaly. Treatment is supportive.

prognosis

The prognosis for individuals with anencephaly is extremely poor. If the infant is not stillborn, then he or she will usually die within a few hours or days after birth. [Editor's Note: The unborn child may have been diagnosed as having anencephaly, but be born with a less severe form of the disease, allowing the infant to live for years or more

Gullain Barre Syndrome and Miller Fisher

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy (AIDP), an autoimmune disorder affecting the peripheral nervous system, usually triggered by an acute infectious process. It is included in the wider group of peripheral neuropathies. There are several types of GBS, but unless otherwise stated, GBS refers to the most common form, AIDP. It is frequently severe and usually exhibits as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes. With prompt treatment by plasmapheresis or intravenous immunoglobulins and supportive care, the majority of patients will regain full functional capacity. However, death may occur if severe pulmonary complications and dysautonomia are present.

Pathophysiology

All forms of Guillain-Barré syndrome are due to an immune response to foreign antigens (such as infectious agents) that are mistargeted at host nerve tissues instead (a form of antigenic mimicry). The targets of such immune attack are thought to be gangliosides, which are complex glycosphingolipids present in large quantities on human nerve tissues, especially in the nodes of Ranvier. An example is the GM1 ganglioside, which can be affected in as many as 20-50% of cases, especially in those preceded by Campylobacter jejuni infections. Another example is the GQ1b ganglioside, which is the target in the Miller Fisher syndrome variant

The most common antecedent infection is Campylobacter jejuni. However, 60% of cases do not have a known cause.

The end result of such autoimmune attack on the peripheral nerves is inflammation of myelin and conduction block, leading to a muscle paralysis that may be accompanied by sensory or autonomic disturbances.

Serum sickness can rarely manifest as the Guillain-Barre syndrome (GBS)

Signs and symptoms

1. weakness which affects the lower limbs first, and rapidly progresses in an ascending fashion.
   
2. Frequently, the lower cranial nerves may be affected, leading to bulbar weakness, (oropharyngeal dysphagia, that is difficulty with swallowing, drooling, and/or maintaining an open airway) and respiratory difficulties.
3. Most patients require hospitalization and about 30% require ventilatory assistance.
   
4. Facial weakness is also commonly a feature, but eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant
   
5. Sensory loss, if present, usually takes the form of loss of proprioception (position sense) and areflexia (complete loss of deep tendon reflexes), an important feature of GBS.
   
6. Loss of pain and temperature sensation is usually mild. In fact, pain is a common symptom in GBS, presenting as deep aching pain, usually in the weakened muscles, which patients compare to the pain from overexercising.
   
7. These pains are self-limited and should be treated with standard analgesics. Bladder dysfunction may occur in severe cases but should be transient. If severe, spinal cord disorder should be suspected.
8. Fever should not be present, and if it is, another cause should be suspected.
9. In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, orthostatic hypotension, and cardiac arrhythmias.

Six different subtypes of Guillain-Barre syndrome (GBS) exist:

• Acute inflammatory demyelinating polyneuropathy (AIDP)
  

• Miller Fisher syndrome (MFS)
  

• Acute motor axonal neuropathy (AMAN)

• Acute motor sensory axonal neuropathy (AMSAN)
  

• Acute panautonomic neuropathy

• Bickerstaff’s brainstem encephalitis (BBE)

Diagnosis

The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. CSF and ECD is used almost every time to verify symptoms, but because of the acute nature of the disorder, they may not become abnormal until after the first week of onset of signs and symptoms.

There currently is no cure for Guillain-Barre syndrome. However, treatments have been proven effective against this syndrome.

• CSF

Typical CSF findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (100 - 1000 mg/dL), without an accompanying pleocytosis (increased cell count). A sustained pleocytosis may indicate an alternative diagnosis such as infection.

Electrodiagnostics

Electromyography (EMG) and nerve conduction study (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.

Diagnostic criteria

Required

• Progressive, relatively symmetrical weakness of 2 or more limbs due to neuropathy
• Areflexia
• Disorder course <>
• Exclusion of other causes (see below)

Supportive

• relatively symmetric weakness accompanied by numbness and/or tingling
• mild sensory involvement
• facial nerve or other cranial nerve involvement
• absence of fever
• typical CSF findings obtained from lumbar puncture
• electrophysiologic evidence of demyelination from electromyogram

Treatment

Supportive care with monitoring of all vital functions is the cornerstone of successful management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm.

Early intubation should be considered in any patient with a vital capacity (VC) <20>2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disorder, or autonomic instability.

Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible.

Either high-dose intravenous immunoglobulins (IVIg) at 400 mg/kg for 5 days or

plasmapheresis

Appendicectomy Video: Watch it