The thymus gland, which lies in the chest area beneath the breastbone,
plays an important role in the development of the immune system in early
life. Its cells form a part of the body's normal immune system. The gland is
somewhat large in infants, grows gradually until puberty, and then gets
smaller and is replaced by fat with age. In adults with myasthenia gravis, the
thymus gland remains large and is abnormal. It contains certain clusters of
immune cells indicative of lymphoid hyperplasiaa condition usually found
only in the spleen and lymph nodes during an active immune response.
Some individuals with myasthenia gravis develop thymomas (tumors of the
thymus gland). Thymomas are generally benign, but they can become
malignant.
The relationship between the thymus gland and myasthenia gravis is not yet
fully understood. Scientists believe the thymus gland may give incorrect
instructions to developing immune cells, ultimately resulting in autoimmunity
and the production of the acetylcholine receptor antibodies, thereby setting
the stage for the attack on neuromuscular transmission.
Although myasthenia gravis may affect any voluntary muscle, muscles that
control eye and eyelid movement, facial expression, and swallowing are most
frequently affected. The onset of the disorder may be sudden and symptoms
often are not immediately recognized as myasthenia gravis.
In most cases, the first noticeable symptom is weakness of the eye muscles.
In others, difficulty in swallowing and slurred speech may be the first signs.
The degree of muscle weakness involved in myasthenia gravis varies greatly
among individuals, ranging from a localized form limited to eye muscles
(ocular myasthenia), to a severe or generalized form in which many muscles
sometimes including those that control breathingare affected.
Symptoms, which vary in type and severity, may include a drooping of one or
both eyelids (ptosis), blurred or double vision (diplopia) due to weakness of
the muscles that control eye movements, unstable or waddling gait, a
Myasthenia gravis occurs in all ethnic groups and both genders. It most
commonly affects young adult women (under 40) and older men (over 60),
but it can occur at any age.
In neonatal myasthenia, the fetus may acquire immune proteins (antibodies)
from a mother affected with myasthenia gravis. Generally, cases of neonatal
myasthenia gravis are temporary and the child's symptoms usually
disappear within 2-3 months after birth. Other children develop myasthenia
gravis indistinguishable from adults. Myasthenia gravis in juveniles is
uncommon.
Myasthenia gravis is not directly inherited nor is it contagious. Occasionally,
the disease may occur in more than one member of the same family.
Rarely, children may show signs of congenital myasthenia or congenital
myasthenic syndrome. These are not autoimmune disorders, but are caused
by defective genes that produce abnormal proteins instead of those which
normally would produce acetylcholine, acetylcholinesterase (the enzyme that
breaks down acetylcholine), or the acetylcholine receptor and other proteins
present along the muscle membrane.
A myasthenic crisis occurs when the muscles that control breathing weaken
to the point that ventilation is inadequate, creating a medical emergency and
requiring a respirator for assisted ventilation. In individuals whose respiratory
muscles are weak, criseswhich generally call for immediate medical
attentionmay be triggered by infection, fever, or an adverse reaction to
medication.
Ptosis - Ptosis is the medical term for a drooping eyelid. It refers only to the
upper eyelid; it does not refer to lower eyelid sagging.
Prednisone - a corticosteroid which suppress your immune system, and can
help control conditions in which your immune system mistakenly attacks its own
tissues.
1. What are the clinical features of osteoporosis?
There typically are no symptoms in the early stages of bone loss. But once
bones have been weakened by osteoporosis, you may have signs and symptoms
that include:
Back pain, caused by a fractured or collapsed vertebra
Loss of height over time
A stooped posture
A bone fracture that occurs much more easily than expected
A thorough history should be obtained to screen for and identify the presence
of known risk factors for osteoporosis and osteoporotic fracture. Specifically, the
history should focus on the following:
Age (>50 years), sex (female), and race (white or Asian)
Family history of osteoporosis, particularly maternal history of fractures
Reproductive factors, especially regarding early menopause and estrogen
replacement therapy: postmenopausal women are at high risk, as are women who
have undergone hysterectomy and oophorectomy
Hypogonadal states: men with hypogonadism secondary to any genetic or
other conditions are at higher risk ; the USPSTF notes that there is insufficient
current evidence to assess the risk versus benefit of screening for osteoporosis in
men
Smoking: smokers are at higher risk
Alcohol consumption
Low levels of physical activity: immobility increases the risk; spinal cord
injury and stroke cause physical impairment and are common causes of immobility
Strenuous exercise that results in amenorrhea (such as that which occurs
in marathon runners)
Calcium and vitamin D intake
History of low-trauma "fragility" fracture in patients aged 40 years or older:
a fragility fracture is defined as a fracture due to trauma that would not normally
cause fracture (a force equal to or less than that resulting from a fall from standing
height)
Signs of vertebral fracture (see below)
Coexisting medical conditions associated with bone loss
Medications associated with bone loss
Risk factors for falls in older patients: these include poor balance,
orthostatic hypotension, weakness of the lower extremity muscles and
deconditioning, use of medications with sedative effects, poor vision or hearing, and
cognitive impairment
Primary osteoporosis
Primary osteoporosis is the most common type of osteoporosis. It is more
common in women than men. A person reaches peak bone mass (density) at about
age 30; after that, the rate of bone loss slowly increases, while the rate of bone
building decreases. Whether a person develops osteoporosis depends on the
thickness of the bones in early life, as well as health, diet, and physical activity at all
ages.
In women, accelerated bone loss usually begins after monthly menstrual
periods stop, when a woman's production of estrogen slows down (usually between
the ages of 45 and 55). In men, gradual bone thinning typically starts at about 45 to
50 years of age, when a man's production of testosterone slows down. Osteoporosis
usually does not have an effect on people until they are 60 or older. Women are
usually affected at an earlier age than men because they start out with lower bone
mass.
Secondary osteoporosis
Secondary osteoporosis has the same symptoms as primary osteoporosis. But
it occurs as a result of having certain medical conditions, such as
hyperparathyroidism, hyperthyroidism, or leukemia. It may also occur as a result of
taking medicines known to cause bone breakdown, such as oral or high-dose
inhaled corticosteroids (if used for more than 6 months), too high a dose of thyroid
replacement, or aromatase inhibitors (used to treat breast cancer). Secondary
osteoporosis can occur at any age.
Osteogenesis imperfecta
Osteogenesis imperfecta is a rare form of osteoporosis that is present at
birth. Osteogenesis imperfecta causes bones to break for no apparent reason.
Idiopathic juvenile osteoporosis
Idiopathic juvenile osteoporosis is rare. It occurs in children between the ages
of 8 and 14 or during times of rapid growth. There is no known cause for this type of
osteoporosis, in which there is too little bone formation or excessive bone loss. This
condition increases the risk of fractures.
4. How do you diagnose osteoporosis?
A DEXA scan can be used to help diagnose osteoporosis. It's a quick, safe and
painless procedure that usually takes about five minutes, depending on the part of
the body being scanned. The scan measures your bone mineral density and
compares it to the bone mineral density of a healthy young adult and someone
who's the same age and sex as you.
5. What is the treatment for osteoporosis?
Three factors essential for keeping your bones healthy throughout your life
are:
Adequate amounts of calcium
Adequate amounts of vitamin D
Regular exercise
Amyotrophic lateral sclerosis (ALS) Lou Gehrig's disease, and rarely Charcot
diseaseis a neurodegenerative disorder with various causes. The term motor
neuron disease (MND) is sometimes used interchangeably with ALS, while others
use it to refer to a group of similar conditions that include ALS.[3] ALS is
characterized by muscle spasticity, rapidly progressive weakness due to muscle
wasting. This results in difficulty speaking, swallowing, and breathing. The disease
usually starts around the age of 60, except in cases that are directly inherited when
the usual age of onset is around 50.
12. Is there any treatment for ALS? What is the prognosis?
A thymectomy is the surgical removal of the thymus gland. The thymus has
been demonstrated to play a role in the development of MG. It is removed in an
effort to improve the weakness caused by MG, and to remove a thymoma if present.
About 10% of MG patients have a tumor of the thymus called a thymoma. Most of
these tumors are benign and tend to grow very slowly; on occasion they are
malignant (cancerous).