$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.