-
A study of three patients with amyotrophic lateral sclerosis and a polyneuropathy resembling CIDP.
Echaniz-Laguna A,
Departement de Neurologie, Hopital Civil de Strasbourg, 1 Place de l'Hopital, BP426, 67091 Strasbourg, France.
We report three patients with a syndrome that fulfilled clinical and laboratory criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who failed immunosuppressive treatment and eventually developed progressive amyotrophic lateral sclerosis (ALS). Mean disease duration was 23 months (13-38) before death. Two patients had a family history of ALS without mutations of the SOD1 gene. Postmortem examination in one patient showed an endoneurial infiltration of mononuclear cells in lumbar roots and distal and proximal peripheral nerves, mainly around myelinated fibers, with demyelination and axonal loss, consistent with CIDP. The spinal cord revealed severe neuronal loss in the anterior horn, axonal loss in the corticospinal tract, and large numbers of phagocytes in the anterior and lateral tracts, indicative of ALS. Whether demyelinating polyneuropathy was coincident with ALS or was a cause or consequence of motor neuron degeneration in these patients remains to be elucidated. This unusual combination may provide an important clue in elucidating the pathogenesis of ALS in some patients. Muscle Nerve, 2006.
PMID: 16320313 [PubMed - as supplied by publisher]
What is Amyotrophic
Lateral Sclerosis?
Amyotrophic lateral sclerosis (ALS), some call this disease as Lou Gehrig's disease the name of the baseball player who died with this disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. In ALS, both the upper motor neurons in th brain and the lower motor neurons in the spinal cord degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually the ability of the brain to start and control voluntary movement is lost. Individuals with ALS lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support. The disease does not affect a person's ability to see, smell, taste, hear, or recognize touch, and it does not usually impair a person’s thinking or other cognitive abilities. However, several recent studies suggest that a small percentage of patients may experience problems with memory or decision-making, and there is growing evidence that some may even develop a form of dementia. The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.
Amyotrophic lateral sclerosis (ALS), some call this disease as Lou Gehrig's disease the name of the baseball player who died with this disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. In ALS, both the upper motor neurons in th brain and the lower motor neurons in the spinal cord degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually the ability of the brain to start and control voluntary movement is lost. Individuals with ALS lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support. The disease does not affect a person's ability to see, smell, taste, hear, or recognize touch, and it does not usually impair a person’s thinking or other cognitive abilities. However, several recent studies suggest that a small percentage of patients may experience problems with memory or decision-making, and there is growing evidence that some may even develop a form of dementia. The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.
CIDPUSA studied this
disease and has found a
connection between baseball
players , football players
getting this disease. Some
scientist have said that
there may be a connection
between the grass they play
on and fall on that ground
may be contaminated with
pesticide. These pesticide
may have a role in causing
this disease. This needs to
be further investigated.
Discussed in our E-book.
Some new studies have
come out which show that
CIDP and ALS may be
linked, there have been
case reports that
antiinflammatory and
immununomodulating drugs
have helped these cases.
A possiable for cure for
ALS has not been found
but AL in case reports
has improved. Please see
the links of ALS from
our home page. However,
the FDA has approved the
first drug treatment for
the disease—riluzole.
Riluzole is believed to
reduce damage to motor
neurons and prolongs
survival by several
months, mainly in those
with difficulty
swallowing. Other
treatments are designed
to relieve symptoms and
improve the quality of
life for people with ALS.
Drugs also are available
to help individuals with
pain, depression, sleep
disturbances, and
constipation.
Individuals with ALS may
eventually consider
forms of mechanical
ventilation
(respirators). Some
reports have recommended
the use of antibiotics
to help victims of ALS.
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.
Individuals have
increasing problems with
moving, swallowing, and
speaking or forming
words. Eventually people
with ALS will not be
able to stand or walk,
get in or out of bed on
their own, or use their
hands and arms. In later
stages of the disease,
individuals have
difficulty breathing as
the muscles of the
respiratory system
weaken. Although
ventilation 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
3 to 5 years from the
onset of symptoms.
However, about 10
percent of those
individuals with ALS
survive for 10 or more
years.
| Muscle Nerve. 2005 Nov 30;33(3):356-362 [Epub ahead of print] |