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Disease Profile

Wallenberg syndrome

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.

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US Estimated

Europe Estimated

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)

Lateral medullary syndrome; Posterior inferior cerebellar artery syndrome; PICA syndrome;

Summary

Wallenberg syndrome is a condition that affects the nervous system. Signs and symptoms may include swallowing difficulties, dizziness, hoarseness, nausea and vomiting, nystagmus, and problems with balance. Some people have uncontrollable hiccups, loss of pain and temperature sensation on one side of the face, and/or weakness or numbness on one side of the body. Wallenberg syndrome is often caused by a stroke in the brain stem. Treatment addresses each symptom and may include a feeding tube for swallowing problems, speech and/or swallowing therapy, and medication for pain. While some people's symptoms may improve within weeks or months, others may have long-term neurological problems.[1]

Symptoms

Wallenberg syndrome may cause a variety of symptoms depending on the specific cause and the exact location of the damage to the brain. Symptoms may include:[2][1]

  • pain and temperature sensory loss on one side of the face as well as on the opposite side of the body
  • rapid involuntary movements of the eyes (nystagmus)
  • problems with balance and gait (walking) coordination
  • vomiting
  • vertigo
  • nystagmus
  • dysphagia
  • hoarseness
  • uncontrollable hiccups
  • Horner syndrome (decreased pupil size, a drooping eyelid and decreased sweating on the affected side of the face) with visual deficits

Cause

The most common underlying cause of Wallenberg syndrome is a brain stem stroke in the vertebral or posterior inferior cerebellar arteries of the brain stem.[1][3] However, several other disorders or conditions reportedly have been associated with Wallenberg syndrome, including:[3][4][5][6][7]

  • mechanical trauma to the vertebral artery in the neck
  • vertebral arteritis (inflammation of the wall of the artery)
  • metastatic cancer
  • hematoma
  •  aneurysm of the vertebral artery
  • herpetic brainstem encephalitis (relating to herpes)
  • head injury
  • arteriovenous malformations (AVMs)
  • multiple sclerosis
  •  varicella infection
  • brainstem tuberculoma (a rare form of tuberculosis)

    • Treatment

    Treatment for Wallenberg syndrome focuses primarily on relieving symptoms and rehabilitation. A feeding tube may be needed for severe swallowing problems. Speech and/or swallowing therapy may be helpful. Medications may be used to control pain.[1] Treatment may also depend on the underlying cause and/or how quickly it is identified.

    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

      Organizations Providing General Support

        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 The Internet Stroke Center, a project supported in part by the NIH Specialized Programs of Translational Research in Acute Stroke (SPOTRIAS) Network and the National Institute of Neurological Disorders and Stroke (NINDS), offers information on Wallenberg syndrome.

          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 Wallenberg syndrome. Click on the link to view a sample search on this topic.

            References

            1. Wallenberg's Syndrome Information Page. NINDS. https://www.ninds.nih.gov/disorders/all-disorders/wallenbergs-syndrome-information-page#disorders-r1. Accessed 5/22/2017.
            2. Louis DW, Dholakia N, Raymond MJ. Wallenberg Syndrome with Associated Motor Weakness in a Two-Week-Postpartum Female. Case Rep Neurol. September 23, 2015; 7(3):186-190. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4611068/.
            3. Qiu W, Wu JS, Carroll WM, Mastaglia FL, Kermode AG. Wallenberg syndrome caused by multiple sclerosis mimicking stroke. J Clin Neurosci. December 2009; 16(12):1700-1702. https://www.ncbi.nlm.nih.gov/pubmed/19800798.
            4. DB Smith and BK Demasters. Demyelinating disease presenting as Wallenberg's syndrome. Report of a patient. Stroke. 1981; 12:877-888. https://stroke.ahajournals.org/content/12/6/877.long.
            5. S.O. Kovacs, K. Kuban, R. Strand. Lateral medullary syndrome following varicella infection. Am J Dis Child. 1993; 147:823-825. https://www.ncbi.nlm.nih.gov/pubmed/8352215.
            6. M.J. Lawson-Smith, S.J. Smith, J.C. Leach et al. Lateral medullary syndrome caused by penetrating head injury. J Clin Neurosci. 2006; 13:792-794. https://www.ncbi.nlm.nih.gov/pubmed/16914313.
            7. Verma R, Sharma P. Lateral medullary syndrome due to brain stem tuberculoma. J Assoc Physicians India. June 2011; 59:382-384. https://www.ncbi.nlm.nih.gov/pubmed/21751596.

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