Rare Infectious Disease News

Disease Profile

Klumpke paralysis

Prevalence
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.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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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

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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.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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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.

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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.

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Not applicable

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Other names (AKA)

Lower brachial plexus palsy; Dejerine-Klumpke palsy; Klumpke's palsy

Categories

Nervous System Diseases

Summary

Klumpke paralysis is a rare type of birth injury to the nerves around a newborn’s shoulder, known as the brachial plexus. Most types of brachial plexus injuries affect the shoulder and upper arm. Klumpke paralysis affects the movement of the lower arm and hand. Signs and symptoms include weakness and loss of movement of the lower arm and hand. Some babies experience drooping of the eyelid on the opposite side of the face as well.[1][2] This symptom may also be referred to as Horner syndrome.[2]

Klumpke paralysis is caused by an injury to the nerves of the brachial plexus that which may result during birth due to a a difficult delivery. This injury can cause a stretch injury (neuropraxia), scarring, or tearing of the brachial plexus nerves. Tearing is called an "avulsion” when the tear is at the spine, and “rupture” when it is not. Diagnosis of Klumpke paralysis is made at birth by physical examination. Sometimes x-rays and other tests are done to determine the extent of the nerve damage. Most infants with Klumpke paralysis have the more mild form of injury (neuropraxia) and often recover within 6 months.[1][3] Some infants will require surgery.[2] Rarely, infants with Klumpke paralysis will have some permanent damage.[2]

Treatment

The affected arm may be immobilized across the body for 7 to 10 days.[4] For mild cases, gentle massage of the arm and range-of-motion exercises may be recommended.[5] For torn nerves (avulsion and rupture injuries), symptoms may improve with surgery.[4]

Most infants recover from the stretch injuries (neuropraxia). After 4 months, additional treatment options may include:[5]

  • Surgery on the nerves (e.g., nerve grafts and neuroma excision)
  • Tendon transfers to help the muscles that are affected by nerve damage work better

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

    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

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Klumpke paralysis. Click on the link to view a sample search on this topic.

        Selected Full-Text Journal Articles

          References

          1. Abzug JM, Kozin SH. Evaluation and management of brachial plexus birth palsy. Orthop Clin North Am. 2014; 45(2):225-32. https://www.ncbi.nlm.nih.gov/pubmed/24684916.
          2. O’Berry P, Brown M, Phillips L, Evans SH. Obstetrical brachial plexus palsy. Curr Prob Pediatr Adolesc Health Care. 2017; 47(7):151-155. https://www.ncbi.nlm.nih.gov/pubmed/28709767.
          3. Smith K and Patel V. Congenital brachial plexus palsy. Paed Child Health. 2016; 26(4):152-156. https://www.sciencedirect.com/science/article/pii/S1751722216000263?via%3Dihub.
          4. NINDS Erb-Duchenne and Dejerine-Klumpke Palsies Information Page National. Institute of Neurological Disorders and Stroke. Updated 8/19/2018; https://www.ninds.nih.gov/Disorders/All-Disorders/Erb-Duchenne-and-Dejerine-Klumpke-Palsies-Information-Page.
          5. Brachial palsy in newborns. MedlinePlus. Updated 12/13/2017; https://www.nlm.nih.gov/medlineplus/ency/article/001395.htm. Accessed 2/24/2010.
          6. Hill A. Neurological problems of the newborn. In: Bradley WG et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Butterworth-Heinemann; 2008;

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