Dilation and curettage

From Wikipedia, the free encyclopedia

Dilation and curettage
(D&C)
Background
Abortion type Surgical
First use Late
19th
century
Gestation 4-12
weeks
&8age
WHO recommends only when manual
vacuum aspiration is unavailable
United States 1.7
(2003)
Medical note8
Undertaken under heavy sedation or general
anesthesia. Risk oI perIoration. Day-case
procedure
InIobox reIerences
Dilation and curettage
3907;03943
MeSH D004107
DiIation (or diIatation) and curettage (D&C) refers to the dilation (widening/opening)
of the cervixand surgical removal of part of the lining of the uterus and/or contents of the
uterus by scraping and scooping (curettage). t is a
diagnostic gynecological procedure.
[1][2]

D&C normally is referred to a procedure involving a curette, also called sharp
curettage.
[1]
However, some sources use the term D&C to refer more generally to any
procedure that involves the processes of dilation and removal of uterine contents, which
includes the more common suction curettage procedures of manual and
electric vacuum aspiration.
[3]

Content8
|hide|
1 Clinical uses
2 Complications
3 See also
4 ReIerences
External links
[edit]Clinical uses
D&Cs are commonly performed for the diagnosis of gynecological conditions leading to
'abnormal uterine bleeding';
[4]
to resolve abnormal uterine bleeding (too much, too often
or too heavy amenstrual flow);
[2]
to remove the excess uterine lining in women who have
conditions such aspolycystic ovary syndrome (which cause a prolonged buildup of
tissue with no natural period to remove it);
[citation needed]
to remove tissue in the uterus that
may be causing abnormal vaginal bleeding,
[1]
including postpartum retained
placenta;
[5]
to remove retained tissue (also known as retained POC or retained products
of conception) in the case of a missed or incomplete miscarriage;
[3]
and as a method
of abortion that is now uncommon.
[1]
n contrast, D&C remains 'standard care' for
missed and incomplete miscarriage in many countries despite the existence of
alternatives currently used for abortions.
[clarification needed]

ecause medical and non-invasive methods of abortion now exist, and because D&C
requires heavy sedation or general anesthesia and has higher risks of complication, the
procedure has been declining as a method of abortion. The World Health
Organization recommends D&C as a method of surgical abortion only when manual
vacuum aspiration is unavailable.
[6]
According to the Centers for Disease Control and
Prevention, D&C only accounted for 2.4% of abortions in the United States in the year
2002,
[7]
down from 23.4% in 1972.
[8]
Most D&Cs are now carried out for miscarriage
management and other indications such as diagnosis.
Hysteroscopy is a valid alternative to D&C for many surgical indications from diagnosis
of uterine pathology to removal of fibroids and even retained products of conception. t
is less risky due to the ability of the doctor to view inside the uterus during surgery,
unlike with blind D&C.
Medical management of miscarriage and medical abortion using drugs such
as misoprostol and mifepristone are safe, non-invasive and cheaper alternatives to
D&C.
[edit]Complications
Complications may arise from either the introduction or spreading of infection, adverse
reaction to general anesthesia required during the surgery or from instrumentation itself,
as the procedure is performed blindly (without the use of any imaging technique such
as ultrasound orhysteroscopy).
One risk of sharp curettage is uterine perforation. Although normally no treatment is
required for uterine perforation, a laparoscopy may be done to verify that bleeding has
stopped on its own. nfection of the uterus or fallopian tubes is also a possible
complication, especially if the woman has an untreated sexually transmitted infection.
[1]

Another risk is intrauterine adhesions, or Asherman's syndrome. One study found that in
women who had one or two sharp curettage procedures for miscarriage, 14-16%
developed some adhesions.
[9]
Women who underwent three sharp curettage
procedures for miscarriage had a 32% risk of developing adhesions.
[9]
The risk of
Asherman's syndrome was found to be 30.9% in women who had D&C following a
missed miscarriage,
[10]
and 25% in those who had a D&C 14 weeks
postpartum.
[11][12][13]
Untreated Asherman's syndrome, especially if severe, also
increases the risk of complications in future pregnancies, such as ectopic
pregnancy, miscarriage, and abnormal placentation (eg.placenta previa and placenta
accreta).
Excessive dilatation may also lead to cervical incompetence in the future which
increases the chance of miscarriage.
Amyotrophic lateral sclerosis
From Wikipedia, the free encyclopedia
(Redirected from Lou Gehrig's disease)
%his article is about progressive Motor Neuron Disease (MND) affecting both the upper motor
neurons and lower motor neurons. For MND affecting either but not necessarily both, see Motor
neurone disease.
"ALS" redirects here. For other uses, see ALS (disambiguation).
Amyotroph|c Latera| Sc|eros|s
(Lou Gehr|gs D|sease)
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2otrophic IateraI scIerosis ($), also referred to as ou Gehrig's disease, is a
form ofmotor neuron disease caused by the degeneration of neurons located in
the ventral horn of thespinal cord and the cortical neurons that provide
their afferent input. The condition is often calledLou Gehrig's disease in North America,
after the famous New York Yankees baseball player who was diagnosed with the
disease in 1939. The disorder is characterized by rapidly progressiveweakness, muscle
atrophy and fasciculations, spasticity, dysarthria, dysphagia, and respiratory
compromise. Sensory function generally is spared, as is autonomic, and oculomotor
activity. ALS is a progressive,
[1]
fatal, neurodegenerative disease with most affected
patients dying of respiratory compromise and pneumonia after 2 to 3 years; although
occasional individuals have a more indolent course and survive for many years.
[2]

Content8
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Slgns and sympLoms
4 lnlLlal sympLoms
4 2 ulsease progresslon
2 Cause
3 aLhophyslology
4 3 SCu
4 32 LacLaLe uyscrasla PypoLhesls
4 33 CLher facLors
4 ulagnosls
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8 LLymology
9 PlsLory
0 8esearch
See also
2 8eferences
3 lurLher readlng
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[edit]Signs and symptoms
The disorder causes muscle weakness and atrophy throughout the body caused by
degeneration of the upper and lower motor neurons. Unable to function, the muscles
weaken and atrophy. Affected individuals may ultimately lose the ability to initiate and
control all voluntary movement, although bladder and bowel sphincters and the muscles
responsible for eye movement are usually, but not always, spared.
Cognitive function is generally spared for most patients although some (~5%) also
have frontotemporal dementia.
[3]
A higher proportion of patients (~30-50%) also have
more subtle cognitive changes which may go unnoticed but are revealed by
detailed neuropsychological testing. Sensory nerves and the autonomic nervous
system, which controls functions like sweating, are generally unaffected but may be
involved for some patients.
[edit]nitiaI s2pto2s
The earliest symptoms of ALS are typically obvious weakness and/or muscle atrophy.
Other presenting symptoms include muscle fasciculation (twitching), cramping, or
stiffness of affected muscles; muscle weakness affecting an arm or a leg; and/or slurred
and nasal speech. The parts of the body affected by early symptoms of ALS depend on
which motor neurons in the body are damaged first. About 75% of people contracting
the disease experience "limb onset" ALS i.e. first symptoms in the arms ("upper limb",
not to be confused with "upper motor neuron") or legs ("lower limb", not to be confused
with "lower motor neuron"). Patients with the leg onset form may experience
awkwardness when walking or running or notice that they are tripping or stumbling,
often with a "dropped foot" which drags gently along the ground. Arm-onset patients
may experience difficulty with tasks requiring manual dexterity such as buttoning a shirt,
writing, or turning a key in a lock. Occasionally, the symptoms remain confined to one
limb for a long period of time or for the whole length of the illness; this is known
as monomelic amyotrophy.
About 25% of cases are "bulbar onset" ALS. These patients first notice difficulty
speaking clearly or swallowing. Speech may become slurred, nasal in character, or
quieter. Other symptoms include difficulty swallowing, and loss of tongue mobility. A
smaller proportion of patients experience "respiratory onset" ALS where
the intercostal muscles that support breathing are affected first.
Regardless of the part of the body first affected by the disease, muscle weakness and
atrophy spread to other parts of the body as the disease progresses. Patients
experience increasing difficulty moving, swallowing (dysphagia), and speaking or
forming words (dysarthria). Symptoms of upper motor neuron involvement include tight
and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia) including an
overactive gag reflex. An abnormal reflex commonly called abinski's sign (the big toe
extends upward and other toes spread out) also indicates upper motor neuron damage.
Symptoms of lower motor neuron degeneration include muscle weakness and atrophy,
muscle cramps, and fleeting twitches of muscles that can be seen under the skin
(fasciculations). Around 1545% of patients experiencepseudobulbar affect, also known
as "emotional lability", which consists of uncontrollable laughter, crying or smiling,
attributable to degeneration of bulbar upper motor neurons resulting in exaggeration of
motor expressions of emotion.
To be diagnosed with ALS, patients must have signs and symptoms of both upper and
lower motor neuron damage that cannot be attributed to other causes.
[edit]Disease progression
Although the sequence of emerging symptoms and the rate of disease progression vary
from person to person, eventually most patients are not able to stand or walk, get in or
out of bed on their own, or use their hands and arms. Difficulty swallowing and chewing
impair the patient's ability to eat normally and increase the risk of choking or aspirating
food/liquids into the lungs. Aspiration pneumonia and weight maintenance can then
become a problem. ecause the disease usually does not affect cognitive abilities,
patients are aware of their progressive loss of function and may become anxious and
depressed. A small percentage of patients go on to develop frontotemporal
dementia characterized by profound personality changes; this is more common among
those with a family history of dementia. A larger proportion of patients experience mild
problems with word-generation, attention, or decision-making. Cognitive function may
be affected as part of the disease process or could be related to poor breathing at night
(nocturnal hypoventilation). Health care professionals need to explain the course of the
disease and describe available treatment options so that patients can make informed
decisions in advance.
As the diaphragm and intercostal muscles (rib cage) weaken, forced vital capacity and
inspiratory pressure diminish. n bulbar onset ALS, this may occur before significant
limb weakness is apparent. ilevel positive pressure ventilation (frequently referred to
by the tradenameiPAP) is frequently used to support breathing, first at night, and later
during the daytime as well. t is recommended that long before iPAP becomes
insufficient, patients must decide whether to have a tracheostomy and long term
mechanical ventilation. At this point, some patients choose palliative hospice care. Most
people with ALS die of respiratory failure or pneumonia. Death usually occurs within two
to five years of diagnosis. Although the disease can strike at any age, most people are
between forty and seventy years of age when the disease strikes and men are affected
slightly more frequently than women. An estimated 5,000 people in the United States
are diagnosed with the disease each year.
[4]
ALS, a progressive disease, leads to death
in half of the people diagnosed within three years and ninety percent within six years. n
a population based study in Minnesota, USA, looking back over 85 years, 14 percent of
people with ALS survived more than 5 years. Those who survived 5 years or longer
were clinically similar to the total population ALS population in terms of gender, age,
gender, and site of onset, but they had a longer time from symptomatic onset to time of
diagnosis
[5]

ALS predominantly affects the motor neurons, and in the majority of cases the disease
does not impair a patient's mind, personality, intelligence, or memory. Nor does it affect
a person's ability to see, smell, taste, hear, or feel touch. Control of eye muscles is the
most preserved function, although some patients with an extremely long duration of
disease (20+ years) may lose eye control too. Unlike multiple sclerosis, bladder and
bowel control are usually preserved in ALS, although as a result of immobility and diet
changes, intestinal problems such as constipation can require intensive management.
[edit]Cause
For patients without a family history of the disease, which includes ~95% of cases, there
is no known cause for ALS.
There is a known hereditary factor in familial ALS (FALS), where the condition is known
to run in families, although this accounts for only around 5% of all cases. An inherited
genetic defect on chromosome 21 (coding for superoxide dismutase) is associated with
approximately 20% of familial cases of ALS.
[6][7]
This mutation is believed to
be autosomal dominant. The most common ALS causing SOD1 mutation in North
America is A4V, characterized by an exceptionally rapid progression from onset to
death. The children of those diagnosed with familial ALS have a higher risk factor for
developing the disease; however, those who have close family members who have
been diagnosed with sporadic ALS have no greater a risk factor than the general
population, suggesting again an environmental or other non-genetic cause.
[8]

Some environmental causative factors have been suggested for the increased
incidence in the western Pacific. Prolonged exposure to a dietary neurotoxin
called MAA is one suspected risk factor in Guam;
[9]
this neurotoxin produced by
cyanobacteria is one of several possible neurotoxic compounds found in the seed of
the cycad ycas circinalis,
[10]
a tropical plant found in Guam, which was used in the
human food supply during the 1950s and early 1960s.
The very high incidence of the disease among talian soccer players (more than five
times higher than normally expected) has raised the concern of a possible link between
the disease and the use of pesticides on the soccer fields (several of which have been
linked to neuronal toxicity).
[11][12]
A 2004 talian study trying to link a high incidence of
ALS in soccer players to performance-enhancing drugs failed when the group was
compared to cyclists that also used performance-enhancing drugs but without
contracting ALS. A possible conclusion was that soccer players experience frequent
head trauma (heading the ball, falls and collisions sustained during games) compared to
cyclists who wear head protection and rarely have falls.
[13][14]

According to the ALS Association, veterans of the United States military are at an
increased risk of contracting ALS (again, possibly implying a link to neurotoxic chemical
exposure). n its report ALS in the Military,
[15]
the group pointed to an almost 60%
greater chance of the disease in military veterans than the general population. For Gulf
War veterans, the chance is seen as twice that of veterans not deployed to the Persian
Gulf in a joint study by the Veterans Affairs Administration and the DOD, another
epidemiologic association suggesting a link to toxic exposure.
[16][17][18]

A 2010 study has raised questions about the diagnosis of ALS in some veterans and
athletes, suggesting that repeated concussions may cause a chronic traumatic
encephalopathy that mimics ALS; this might explain the higher rate of ALS diagnoses in
those populations.
[unreliable medical source?][19]

[edit]Pathophysiology
Genet|c assoc|at|ons |nc|ude
1ype CMM Gene Locus
ALS 03400 5Ou1 222
ALS2 20300 Al52 233
ALS3 606640 ? 82
ALS4 602433 51\ 9343
ALS3 602099 ? 332
ALS6 608030 lu5 6p2
ALS 60803 ? 20p3
ALS8 60862 vAl8 2033
ALS9 6893 ANC 42
ALS0 62069 1Aku8l p362
ALS 623 llC4 62
ALS2 63433 Ol1N 0p3p4
The defining feature of ALS is the death of both upper
and lower motor neurons in the motor cortex of the
brain, the brain stem, and the spinal cord. Prior to
their destruction, motor neurons develop
proteinaceous inclusions in their cell bodies
and axons. This may be partly due to defects in protein degradation.
[20]
These inclusions
often contain ubiquitin, and generally incorporate one of the ALS-associated
proteins: SOD1, TAR DNA binding protein (TDP-43, or TARDP), or FUS. nterestingly,
these inclusions do not stain with the dyes Congo Red orThioflavin S, and are therefore
non-amyloid aggregates.
[21][22]
This is in contrast to the aggregates and plaques seen in
many other neurodegenerative diseases of protein aggregation, including Alzheimer's
disease, Parkinson's disease, Huntington's disease, and prion diseases.
[edit]$D1
The cause of ALS is not known, though an important step toward determining the cause
came in 1993 when scientists discovered that mutations in the gene that produces the
Cu/Znsuperoxide dismutase (SOD1) enzyme were associated with some cases
(approximately 20%) of familial ALS. This enzyme is a powerful antioxidant that protects
the body from damage caused by superoxide, a toxic free radical generated in the
mitochondria. Free radicals are highly reactive molecules produced by cells during
normal metabolism again largely by the mitochondria. Free radicals can accumulate and
cause damage to both mitochondrial and nuclear DNA and proteins within cells. To
date, over 110 different mutations in SOD1 have been linked with the disease, some of
which have a very long clinical course (e.g. H46R), while others, such as A4V, being
exceptionally aggressive. Evidence suggests that failure of defenses against oxidative
stress up-regulates programmed cell death (apoptosis), among many other possible
consequences. Although it is not yet clear how the SOD1 gene mutation leads to motor
neuron degeneration, researchers have theorized that an accumulation of free radicals
may result from the faulty functioning of this gene. Current research, however, indicates
that motor neuron death is not likely a result of lost or compromised dismutase activity,
suggesting mutant SOD1 induces toxicity in some other way (a gain of function).
[23][24]

Studies involving transgenic mice have yielded several theories about the role of SOD1
in mutant SOD1 familial amyotrophic lateral sclerosis. Mice lacking the SOD1 gene
entirely do not customarily develop ALS, although they do exhibit an acceleration of
age-related muscle atrophy (sarcopenia) and a shortened lifespan (see article
on superoxide dismutase). This indicates that the toxic properties of the mutant SOD1
ALS3 83090 A1\N2 2242
ALS4 63934 vcl 9p33
are a result of a gain in function rather than a loss of normal function. n addition,
aggregation of proteins has been found to be a common pathological feature of both
familial and sporadic ALS (see article on proteopathy). nterestingly, in mutant SOD1
mice (most commonly, theG93A mutant), aggregates (misfolded protein accumulations)
of mutant SOD1 were found only in diseased tissues, and greater amounts were
detected during motor neuron degeneration.
[25]
t is speculated that aggregate
accumulation of mutant SOD1 plays a role in disrupting cellular functions by
damaging mitochondria, proteasomes, protein folding chaperones, or other
proteins.
[26]
Any such disruption, if proven, would lend significant credibility to the theory
that aggregates are involved in mutant SOD1 toxicity. Critics have noted that in
humans, SOD1 mutations cause only 2% or so of overall cases and the etiological
mechanisms may be distinct from those responsible for the sporadic form of the
disease. To date, the ALS-SOD1 mice remain the best model of the disease for
preclinical studies but it is hoped that more useful models will be developed.
[edit]actate Dscrasia Hpothesis
Researchers specializing in the neurobiology of aging have proposed a novel molecular
model for the pathogenesis of ALS called the lactate dyscrasia hypothesis that involves
an adenosine triphosphate (ATP)-dependent muscle neuronal lactate shuttle (MNLS) at
the neuromuscular junction (NMJ) to regulate the flow of lactate from muscle to neurons
and vice versa. Failure of the MNLS due to respiratory chain dysfunction is proposed to
result in lactate toxicity and degeneration of nerve endings at the NMJ leading to nerve
terminus dysjunction from the muscle cell.
[27]

[edit]ther factors
Studies also have focused on the role of glutamate in motor neuron degeneration.
Glutamate is one of the chemical messengers orneurotransmitters in the brain.
Scientists have found that, compared to healthy people, ALS patients have higher levels
of glutamate in theserum and spinal fluid.
[7]
Riluzole is currently the only FDA approved
drug for ALS and targets glutamate transporters. t only has a modest effect on survival,
however, suggesting that excess glutamate is not the sole cause of the disease.
[edit]Diagnosis
No test can provide a definite diagnosis of ALS, although the presence of upper and
lower motor neuron signs in a single limb is strongly suggestive. nstead, the diagnosis
of ALS is primarily based on the symptoms and signs the physician observes in the
patient and a series of tests to rule out other diseases. Physicians obtain the patient's
full medical history and usually conduct a neurologic examination at regular intervals to
assess whether symptoms such as muscle weakness, atrophy of muscles,
hyperreflexia, and spasticity are getting progressively worse.


MR (axial FLAR) demonstrates increased T2 signal within the posterior part of the internal capsule,
consistent with the clinical diagnosis of ALS.
ecause symptoms of ALS can be similar to those of a wide variety of other, more
treatable diseases or disorders, appropriate tests must be conducted to exclude the
possibility of other conditions. One of these tests is electromyography (EMG), a special
recording technique that detects electrical activity in muscles. Certain EMG findings can
support the diagnosis of ALS. Another common test measures nerve conduction
velocity (NCV). Specific abnormalities in the NCV results may suggest, for example, that
the patient has a form of peripheral neuropathy(damage to peripheral nerves)
or myopathy (muscle disease) rather than ALS. The physician may order magnetic
resonance imaging (MR), a noninvasive procedure that uses a magnetic field and radio
waves to take detailed images of the brain and spinal cord. Although these MR scans
are often normal in patients with ALS, they can reveal evidence of other problems that
may be causing the symptoms, such as a spinal cord tumor, multiple sclerosis,
a herniated disk in the neck,syringomyelia, or cervical spondylosis.
ased on the patient's symptoms and findings from the examination and from these
tests, the physician may order tests on blood and urine samples to eliminate the
possibility of other diseases as well as routine laboratory tests. n some cases, for
example, if a physician suspects that the patient may have a myopathy rather than ALS,
a muscle biopsy may be performed.
nfectious diseases such as human immunodeficiency virus (HV), human T-cell
leukaemia virus (HTLV), Lyme disease,
[28]
syphilis
[29]
and tick-borne
encephalitis
[30]
viruses can in some cases cause ALS-like symptoms. Neurological
disorders such as multiple sclerosis, post-polio syndrome, multifocal motor
neuropathy, CDP, and spinal muscular atrophy can also mimic certain facets of the
disease and should be considered by physicians attempting to make a diagnosis.
ecause of the prognosis carried by this diagnosis and the variety of diseases or
disorders that can resemble ALS in the early stages of the disease, patients should
always obtain a second neurological opinion.
[edit]Treatment
[edit]$Iowing progression
Riluzole (Rilutek) as of 2011 is the only treatment that has been found to improve
survival but only to a modest extent.
[31]
t lengthens survival by several months, and may
have a greater survival benefit for those with a bulbar onset. t also extends the time
before a person needs ventilation support. Riluzole does not reverse the damage
already done to motor neurons, and people taking it must be monitored for liver damage
(occurring in ~10% of people taking the drug).
[32]
t is approved by Food and Drug
Administration (FDA) and recommended by theNational nstitute for Clinical
Excellence (NCE).
[edit]$2pto2atic
Other treatments for ALS are designed to relieve symptoms and improve the quality of
life for patients. This supportive care is best provided by multidisciplinary teams of
health care professionals such as physicians; pharmacists; physical, occupational, and
speech therapists; nutritionists; social workers; and home care and hospice nurses.
Working with patients and caregivers, these teams can design an individualized plan of
medical and physical therapy and provide special equipment aimed at keeping patients
as mobile and comfortable as possible.
Physicians can prescribe medications to help reduce fatigue, ease muscle cramps,
control spasticity, and reduce excess saliva and phlegm. Drugs also are available to
help patients with pain, depression, sleep disturbances,
and constipation. Pharmacists can advise on best use of medications. This is
particularly helpful with regards to patients with dysphagia, which many ALS patients
experience. They would also monitor a patient's medications to reduce risk of drug
interactions.
Physical therapists play a large role in rehabilitation for individuals with ALS.
Specifically, physical therapists can set goals and promote benefits for individuals with
ALS by delaying loss of strength, maintaining endurance, limiting pain, preventing
complications, and promoting functional independence.
[33]
There is also a strong
emphasis on the importance of patient and caregiver education that can be reinforced
by physical therapists or occupational therapists.
[33]
Research is controversial as to
whether implementing a specific exercise program for these individuals may be
beneficial; moreover, it is important for a physical therapist to address and understand
the risks associated with implementing these types of programs for each and every
person with ALS and the severity of their condition. The controversy lies in the fact that
because ALS is characteristic of the degeneration of upper and lower motor neurons,
that these neurons may react differently to specific exercise programs.
[33]
ecause
spasticity is a common characteristic for individuals with ALS, physical therapists aim to
reduce this by implementing range of motion activities with minimal resistance.
[33]
n
addition to range of motion activities, positioning techniques and splinting have also
been shown to reduce spasticity; moreover, these techniques can also play an integral
role in the reduction of pain for people with ALS.
[33]
Overall, physical therapists have
been proven to have positive effects on individuals with ALS by prescribing techniques
and equipment to assist with conserving energy, emphasizing the importance of
education, limiting pain, and help to maintain a level of function appropriate for each of
their clients with ALS.
[33]

Physical therapy and special equipment such as assistive technology can also enhance
patients' independence and safety throughout the course of ALS. ut physical therapists
must be mindful when prescribing assistive devices, keeping in mind the patients and
their attitudes. Devices should make the patient feel hopeful, not helpless. Gentle, low-
impact aerobic exercise such as walking, swimming, and stationary bicycling can
strengthen unaffected muscles, improve cardiovascular health, and help patients fight
fatigue and depression. Range of motion and stretching exercises can help prevent
painful spasticity and shortening (contracture) of muscles. Physical therapists can
recommend exercises that provide these benefits without overworking muscles. They
can suggest devices such as ramps, braces, walkers, and wheelchairs that help
patients remain mobile. Examples of devices prescribed can include cervical
collars.
[34]
n ALS, there will be a progression of cervical extensor
weakness.
[34]
Weakness of the muscles will cause the patient's head to fall forward,
leading to acute neck pain, potential for chronic cervical conditions to develop and
tightness of anterior neck muscles.
[34]
A forward head posture will interfere in patients
ADLs, making them more dependent on caretakers. A cervical collar can help restore
their independence and comfort. When there is mild to moderate weakness of the
cervical extensor, the physical therapist may prescribe soft foam collar.
[34]
When more
severe weakness is observed, a more rigid collar will be beneficial.
[34]
Occupational
therapist can provide or recommend equipment and adaptations to enable people to
retain as much independence in activities of daily living as possible.
ALS patients who have difficulty speaking may benefit from working with a speech-
language pathologist. These health professionals can teach patients adaptive strategies
such as techniques to help them speak louder and more clearly. As ALS progresses,
speech-language pathologists can recommend the use of augmentative and alternative
communication such as voice amplifiers, speech-generating devices (or voice output
communication devices) and/or low tech communication techniques such as alphabet
boards or yes/no signals. These methods and devices help patients communicate when
they can no longer speak or produce vocal sounds. With the help of occupational
therapists, speech-generating devices can be activated by switches or mouse emulation
techniques controlled by small physical movements of, for example, the head, finger or
eyes. n every case, the appropriate therapist should be mindful of the patients'
preferences, attitudes, and likely progression over time.
Patients and caregivers can learn from speech-language pathologists and nutritionists
how to plan and prepare numerous small meals throughout the day that provide enough
calories, fiber, and fluid and how to avoid foods that are difficult to swallow. Patients
may begin using suction devices to remove excess fluids or saliva and prevent choking.
When patients can no longer get enough nourishment from eating, doctors may advise
inserting a feeding tube into the stomach. The use of a feeding tube also reduces the
risk of choking and pneumonia that can result from inhaling liquids into the lungs. The
tube is not painful and does not prevent patients from eating food orally if they wish.
When the muscles that assist in breathing weaken, use of ventilatory assistance
(intermittent positive pressure ventilation (PPV), bilevel positive airway
pressure (PAP), or biphasic cuirass ventilation (CV)) may be used to aid breathing.
Such devices artificially inflate the patient's lungs from various external sources that are
applied directly to the face or body. When muscles are no longer able to maintain
oxygen and carbon dioxide levels, these devices may be used full-time. CV has the
added advantage of being able to assist in clearing secretions by using high-frequency
oscillations followed by several positive expiratory breaths.
[35]
Patients may eventually
consider forms of mechanical ventilation (respirators) in which a machine inflates and
deflates the lungs. To be effective, this may require a tube that passes from the nose or
mouth to the windpipe (trachea) and for long-term use, an operation such as
a tracheostomy, in which a plastic breathing tube is inserted directly in the patient's
windpipe through an opening in the neck.
Patients and their families should consider several factors when deciding whether and
when to use one of these options. Ventilation devices differ in their effect on the
patient's quality of life and in cost. Although ventilation support can ease problems with
breathing and prolong survival, it does not affect the progression of ALS. Patients need
to be fully informed about these considerations and the long-term effects of life without
movement before they make decisions about ventilation support. Some patients under
long-term tracheostomy intermittent positive pressure ventilation with deflated cuffs or
cuffless tracheostomy tubes (leak ventilation) are able to speak, provided their bulbar
muscles are strong enough. This technique preserves speech in some patients with
long-term mechanical ventilation.
Social workers and home care and hospice nurses help patients, families, and
caregivers with the medical, emotional, and financial challenges of coping with ALS,
particularly during the final stages of the disease. Social workers provide support such
as assistance in obtaining financial aid, arranging durable power of attorney, preparing
a living will, and finding support groups for patients and caregivers. Home nurses are
available not only to provide medical care but also to teach caregivers about tasks such
as maintaining respirators, giving feedings, and moving patients to avoid painful skin
problems and contractures. Home hospice nurses work in consultation with physicians
to ensure proper medication, pain control, and other care affecting the quality of life of
patients who wish to remain at home. The home hospice team can also counsel
patients and caregivers about end-of-life issues.
Researchers have stated that "ALS patients have a chronically deficient intake of
energy and recommended augmentation of energy intake."
[36]
oth animal
[37]
and
human research
[36][38]
suggest that ALS patients should be encouraged to consume as
many calories as possible and not to restrict their calorie intake.
Many ALS patients use complementary and alternative medicines in an attempt to slow
their disease.
[citation needed]
This may include popular vitamin supplements such as Vitamin
C, high doses of vitamins and nutrients ("mega-dosing"), traditional Chinese medicine,
or other forms of therapy such as acupuncture, reiki, or massage. To date there have
been no studies demonstrating that such treatment approaches have an effect on the
progression of the disease. Given the lack of therapeutic options, people with ALS can
be vulnerable to snake oil scams involving complicated medical terminology or
potentially exciting technologies such as stem cell transplantation. Practitioners of these
scams promise amazing results but carry out little or no real follow up or study of the
patients they have treated in order to prove their assertions. The risks of false hope,
financial harm, and potentially medically harm, are a threat to the wellbeing of ALS
patients and their families.
[edit]Prognosis
Eventually most people with ALS are not able to stand or walk, get in or out of bed on
their own, use their hands and arms, or communicate. n later stages of the disease,
individuals have difficulty breathing as the muscles of the respiratory system weaken.
Although respiratory support can ease problems with breathing and prolong survival, it
does not affect the progression of ALS. Most people with ALS die from respiratory
failure, usually within three to five years from the onset of symptoms. The median
survival time from onset to death ranges from 20 to 48 months, but 10 to 20% of ALS
patients have a survival longer than 10 years.
[39]

[edit]Epidemiology
ALS is one of the most common neuromuscular diseases worldwide, and people of all
races and ethnic backgrounds are affected. One or two out of 100,000 people develop
ALS each year.
[40]
ALS most commonly strikes people between 40 and 60 years of age,
but younger and older people can also develop the disease. Men are affected slightly
more often than women.
"Familial ALS" accounts for approximately 5%10% of all ALS cases and is caused by
genetic factors. Of these, approximately 1 in 10 is linked to a mutation
in copper/zinc superoxide dismutase (SOD1), an enzyme responsible for
scavenging free radicals. A recent study has identified a gene called FUS ("Fused in
Sarcoma", ALS6) as being responsible for 1 in 20 cases of fALS.
[41][42]

Although the incidence of ALS is thought to be regionally uniform, there are three
regions in the West Pacific where there has in the past been an elevated occurrence of
ALS. This seems to be declining in recent decades. The largest is the area
of Guam inhabited by theChamorro people, who have historically had a high incidence
(as much as 143 cases per 100,000 people per year) of a condition calledLytico-odig
disease which is a combination of ALS, Parkinsonism, and dementia.
[43]
Two more
areas of increased incidence are West Papua and the Kii Peninsula of Japan.
[44][45]

Although there have been reports of several "clusters" including three American
football players from the San Francisco 49ers, more than fifty football players in
taly,
[11]
three football-playing friends in the south of England,
[46]
and reports of conjugal
(husband and wife) cases in the south of France,
[47][48][49][50][51]
these are statistically
plausible chance events
[citation needed]
. Although many authors consider ALS to be caused
by a combination of genetic and environmental risk factors, so far the latter have not
been firmly identified, other than a higher risk with increasing age.
n 2010, a VA study found that head trauma can produce symptoms that resemble ALS
but that are actually chronic traumatic encephalopathy (CTE). Postmortem brain studies
conducted on two American football players showed evidence of CTE, rather than
ALS.
[52]

[edit]Etymology
Amyotrophic comes from the Greek language: A- means "no", myo refers to "muscle",
and trophic means "nourishment"; amyotrophictherefore means "no muscle
nourishment," which describes the characteristic atrophication of the sufferer's disused
muscle tissue. Lateralidentifies the areas in a person's spinal cord where portions of the
nerve cells that are affected are located. As this area degenerates it leads to scarring or
hardening ("sclerosis") in the region.
[edit]History
1|me||ne
ear Lvent
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869 lrench docLor !eanMarLln CharcoL flrsL
Jobloos Jlscoss
descrlbes ALS ln sclenLlflc llLeraLure
33

88
Cn AmyoLrophlc LaLeral Sclerosls ls LranslaLed lnLo Lngllsh and publlshed ln a Lhreevolume
edlLlon of LecLures on Lhe ulseases of Lhe nervous SysLem
939
ALS becomes a coose clbte ln Lhe unlLed SLaLes when baseball legend Lou ehrlgs career
and Lwo years laLer hls llfels ended by Lhe dlsease Pe glves hls farewell speech on !uly 4
930s ALS epldemlc occurs among Lhe Chamorro people on uam
99 8esearchers llnk chromosome 2 Lo lALS (lamlllal ALS)
993 SCu gene on chromosome 2 found Lo play a role ln some cases of lALS
996 8lluLek becomes Lhe flrsL luAapproved drug for ALS
998 1he Ll Lscorlal crlLerla ls developed as Lhe sLandard for classlfylng ALS paLlenL ln cllnlcal research
[edit]Research
A number of clinical trials are underway globally for ALS; a comprehensive listing of
trials in the US can be found at ClinicalTrials.gov.
KNS-760704 is under clinical investigation in ALS patients. t is hoped that the drug will
have a neuroprotective effect. t is one enantiomer ofpramipexole, which is approved for
the treatment of Parkinson's disease and restless legs syndrome.
[54]
The single-
enantiomer preparation is essentially inactive at dopamine receptors, is not dose limited
by the potent dopaminergic properties of pramipexole.
[55]
Results of a Phase clinical
trial conducted by Knopp Neurosciences and involving 102 patients were reported in
2010; the trial found a dose-dependent slowing in loss of function.
[56]

Olesoxime (TRO19622) is being tested in a phase 3 clinical study, as part of the
MitoTarget Project.
[57]
The molecule has a cholesterol-like structure and displays strong
neuroprotective properties, and it should be as effective as a cocktail of three
neurotrophic factors in keeping motor neurons alive in culture. The ongoing clinical
study aims to test efficacy, safety, tolerability and plasma levels in patients with ALS, to
see whether a single daily dose of two capsules under certain circumstances can
improve survival and symptoms of ALS patients. The trial started in May 2009, all the
patients are recruited and results are expected in the last quarter of 2011. The study is
taking place in France, elgium, Germany, the UK and Spain.
The new discovery of RNAi has some promise in treating ALS. n recent studies, RNAi
has been used in lab rats to shut off specific genes that lead to ALS. Cytrx Corporation
has sponsored ALS research using RNAi gene silencing technology targeted at the
mutant SOD1 gene.
[58]
Cytrx's orally-administered drug Arimoclomol is currently in
clinical evaluation as a therapeutic treatment for ALS.