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$Unique_ID{BRK04137}
$Pretitle{}
$Title{Primary Lateral Sclerosis}
$Subject{Primary Lateral Sclerosis Central Motor Neuron Disease PLS Motor
Neuron Disease ALS (Amyotrophic Lateral Sclerosis) Werdnig-Hoffmann Disease
Multifocal Motor Neuropathy (MMN)}
$Volume{}
$Log{}
Copyright (C) 1989, 1992 National Organization for Rare Disorders, Inc.
645:
Primary Lateral Sclerosis
** IMPORTANT **
It is possible that the main title of the article (Primary Lateral
Sclerosis) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
Central Motor Neuron Disease
PLS
Motor Neuron Disease
Information on the following diseases can be found in the Related
Disorders section of this report:
ALS (Amyotrophic Lateral Sclerosis)
Werdnig-Hoffmann Disease
Multifocal Motor Neuropathy (MMN)
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the
"Resources" section of this report.
Primary Lateral Sclerosis is a rare neurological disease affecting the
central motor neurons. It causes progressive muscle weakness in the facial
area, hands, arms, legs and feet. It is associated with spasticity and
hyperactive deep-tendon reflexes of the muscles in these areas.
Symptoms
Primary Lateral Sclerosis is a disease of the nerve cells which control
muscles. Initially there are few symptoms and there is a slow progression of
the neurological dysfunction. There may be spastic reactions of the muscles
in the hands, feet or legs which may progress to paralysis. The ability to
speak may also be affected as the muscles used in speaking may become
involved. The senses and intelligence of the patient are unaffected. The
disease may progress slowly over a number of years with wasting and weakening
of the affected muscles.
Causes
Like many other motor neuron diseases, the exact cause of Primary Lateral
Sclerosis is not known.
Affected Population
Primary Lateral Sclerosis is a rare disorder that affects males and females
in equal numbers.
Related Disorders
Symptoms of the following disorders can be similar to those of Primary
Lateral Sclerosis. Comparisons may be useful for a differential diagnosis:
Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease) is a disorder
of the motor neuron cells. It generally affects both the upper and lower
motor neurons and results in the progressive wasting and weakening of those
muscles which have lost their nerve supply. There are a number of different
forms of ALS, all exhibiting some of the classical symptoms. Slight patchy
weakness, clumsiness of the hands, weakness in the legs, slowing of speech,
difficulty in swallowing, and night cramps in the leg muscles are a few of
these symptoms. (For more information on this disorder, choose "ALS" as your
search term in the Rare Disease Database).
Werdnig-Hoffmann Disease is a severe and usually rapidly progressive
motor neuron disease that affects infants. It is characterized by a
generalized atrophy and weakness of the muscles of the trunk and extremities,
as a result of degenerative changes in the ventral horn cells of the spinal
cord. This weakness, referred to as the amyotonia congenital syndrome, is
also found in other neuromuscular diseases. (For more information on this
disorder, choose "Werdnig-Hoffmann" as your search term in the Rare Disease
Database).
Multifocal Motor Neuropathy is a neurologic disease that has symptoms
similar to Amyotrophic Lateral Sclerosis (ALS). However, lower motor neurons
are mainly affected. Patients with this disease have slowly progressive
muscle wasting and weakness without spasticity and stiffness. This disorder
may respond to immunosuppressive drug treatment. For the patients who
respond the weakness may not only stop progressing but may also improve.
Therapies: Standard
Treatment of Primary Lateral Sclerosis involves the use of drugs to help
control the symptoms. Baclofen is prescribed for spasticity, Quinine for
cramps, and Diazepam for muscular contractions. Other treatments may include
physical therapy to prevent stiffness of joints, and speech therapy may be
needed to aid the patient whose ability to speak has been impaired by muscle
weakness. Other treatment is symptomatic and supportive.
Therapies: Investigational
Scientists are conducting extensive ongoing research on motor neuron diseases
in the areas of nerve growth factors, axonal transport, androgen receptor in
motor neurons, and DNA/RNA changes.
Syntex-Synergen Neuroscience of Boulder, CO, is sponsoring an orphan
product for the treatment of motor neuron diseases including Primary Lateral
Sclerosis. The chemical name is ciliary neurotrophic factor, recombinant
human.
This disease entry is based upon medical information available through
June 1992. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Primary Lateral Sclerosis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Institute of Neurological Disorders & Stroke (NINDS)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
(800) 352-9424
The Amyotrophic Lateral Sclerosis Society
21021 Ventura Blvd., Suite 321
Woodland Hills, CA 91364
(818) 340-7500
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 2239, 2243.
CHRONIC PROGRESSIVE SPINOBULBAR SPASTICITY. A RARE FORM OF PRIMARY
LATERAL SCLEROSIS. J.L. Gastaut, et al.; Arch Neurol (May, 1988, issue 45
(5)). Pp. 509-513.
CLINICAL AND ELECTROPHYSIOLOGICAL STUDIES IN PRIMARY LATERAL SCLEROSIS.
L.S. Russo, Jr. Arch Neurol (October, 1982, issue 39 (10)). Pp. 662-664.
PRIMARY LATERAL SCLEROSIS; A CASE REPORT. M.F. Beal, et al.; Arch Neurol
(October, 1981, issue 38 (10)). Pp. 630-633.
PRIMARY LATERAL SCLEROSIS, A DEBATED ENTITY. K.A. Sotaniemi, et al.; Acta
Neurol Scand (April, 1985, issue 71 (4)). Pp. 334-336.