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Disease Profile
Central pain syndrome
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
#N/A
Age of onset
#N/A
ICD-10
#N/A
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Thalamic syndrome (former); Dejerine Roussy syndrome (former); Posterior thalamic syndrome (former);
Categories
Nervous System Diseases
Summary
Central pain
Many different names have been used for this disorder, including Dejerine-Roussy syndrome, thalamic pain syndrome, central post-stroke syndrome and others. The current name acknowledges that damage to various areas of the central nervous system can cause central pain, and that a stroke is not necessarily the cause. When CPS is due to a stroke, it may be referred to as the more specific term "central post-stroke pain."[1]
Symptoms
The severity of pain is usually related to the cause of the
People with CPS experience one or more types of pain sensations, the most prominent being burning. Mingled with the burning may be sensations of pins and needles, pressing, lacerating, aching, or brief, intolerable bursts of sharp pain. Some people also experience numbness. The burning and loss-of-touch sensations are usually most severe on the distal parts of the body, such as the feet or hands.[2]
Diagnosis
Treatment
In general, first-line management includes the use of tricyclic antidepressants such as nortriptyline, anticonvulsants such as gabapentin, or topical lidocaine. Second-line management involves the use of opioid analgesics such as tramadol, along with first-line medication. Third-line management may include other antidepressant or anticonvulsant medications.[5]
Lowering stress levels appears to reduce pain.[2] Other treatment alternatives have included the administration of a sympathetic blockade (a type of nerve block) and a guanethidine block, as well as psychological evaluation and treatment. Rarely, surgery is necessary.[6] Stereotactic radiosurgery of the pituitary has been used with some success.[4] Other forms of potential treatments that have been discussed in the literature include transcutaneous electrical nerve stimulation (TENS); deep brain stimulation; and motor cortex stimulation.[1][7]
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
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American Chronic Pain Association (ACPA)
P.O. Box 850
Rocklin, CA 95677-0850
Toll-free: 800-533-3231
Telephone: 916-632-0922
Fax: 916-652-8190
E-mail: [email protected]
Website: https://theacpa.org/ -
Central Pain Syndrome Foundation (CPSF)
8760 State Highway 303 NE, #i
Bremerton, WA 98311
Telephone: (360) 471-3631
E-mail: [email protected]
Website: https://www.cps.foundation/home -
Pain Relief Foundation
Clinical Sciences Centre
University Hospital Aintree, Lower Lane
Liverpool L9 7AL
United Kingdom
Telephone: 0151 529 5820
Fax: 0151 529 5821
Website: https://painrelieffoundation.org.uk/
Organizations Providing General Support
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American Stroke Association
National Center
7272 Greenville Avenue
Dallas, TX 75231
Telephone: 888-478-7653
Website: https://www.strokeassociation.org/
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
In-Depth Information
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Central pain syndrome. Click on the link to view a sample search on this topic.
References
- Central Pain Syndrome. National Organization for Rare Disorders. 2015; https://rarediseases.org/rare-diseases/central-pain-syndrome/.
- NINDS Central Pain Syndrome Information Page. National Institute of Neurological Disorders and Stroke (NINDS). https://www.ninds.nih.gov/disorders/central_pain/central_pain.htm.
- Klit H, Finnerup NB, Jensen TS. Central post-stroke pain: clinical characteristics, pathophysiology, and management. Lancet Neurology. 2009; 8:859-868. https://www.ncbi.nlm.nih.gov/pubmed/19679277.
- YiLi Zhou. CHAPTER 48 – Principles of Pain Management. Bradley: Neurology in Clinical Practice, 5th ed.[Electronic version]. Deutschland: Butterworth-Heinemann; 2008 ; 905.
- Jay GW. Classic Central Pain Syndromes: Review of Neurologic Causes of Pain. Practical Pain Manag. 2015; https://www.practicalpainmanagement.com/classic-central-pain-syndromes-review-neurologic-causes-pain.
- Gould R, Barnes SS. Shoulder Pain and Hemiplegia. Medscape. 2015; https://emedicine.medscape.com/article/328793-overview.
- Schott GD. From thalamic syndrome to central poststroke pain. Journalof Neurology, Neurosurgery, and Psychiatry. December, 1996; 61(6):560-564. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC486645/pdf/jnnpsyc00012-0002.pdf.
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