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Disease Profile

HaNDL 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.

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)

Migrainous syndrome with cerebrospinal fluid pleocytosis; Syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis: HaNDL; Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis;

Summary

Transient Headache and Neurologic Deficits With Cerebrospinal Fluid Lymphocytosis (HaNDL syndrome) is a headache disorder in which individuals experience severe to moderate headache attacks, neurological symptoms, and an increase in the amount of a type of white blood cell in the cerebrospinal fluid (lymphocytic pleocytosis).[1] HaNDL syndrome is most often diagnosed in adulthood, although individuals with ages ranging from 7 to 52 have been reported with this condition. The most common neurological symptoms associated with HanDL syndrome include: weakness on one side of the body (hemiparesis), a feeling of altered sensation down one side of the body (hemisensory disturbances), and a loss of the ability to understand or express speech (aphasia). These symptoms usually last between 15 minutes and 2 hours.[2] The cause of HaNDL syndrome is not well understood; however, researchers have suggested a few potential causes, including migraines, inflammation of the tissue that lines the brain and spinal cord, and viral infections.[2][3] For some individuals, HaNDL syndrome may resolve without treatment, while for others, management of headache and neurological symptoms may be needed.[1][2]

Symptoms

The signs and symptoms of HaNDL syndrome include severe to moderate headache attacks, neurological symptoms, and an increase in the amount of a type of white blood cell in the cerebrospinal fluid (lymphocytic pleocytosis).[1][2]

Headaches associated with HaNDL syndrome may be limited to one side of the head or may involve both sides. They can last from one hour to a week and are often associated with nausea, vomiting, and sensitivity to light. Some individuals may also have a fever. Often, the headache occurs after neurological symptoms begin.

Neurological symptoms usually last between 15 minutes and 2 hours, although this may range from 5 minutes to 3 days. Most people additionally experience repeated headaches and symptoms for weeks to months after the initial attack. Neurological symptoms usually differ from one episode to the next. 

The most common neurological symptoms are:

  • Weakness on one side of the body (hemiparesis)
  • A feeling of altered sensation down one side of the body (hemisensory disturbances)
  • A loss of the ability to understand or express speech (aphasia)

Visual signs and symptoms occur less commonly and may include: 

  • Decreased vision
  • Loss of vision on the left or right side of the visual field (homonymous hemianopsia)
  • Perceived flashes of light (photopsias)

People with this condition may also experience swelling of the nerve that runs from the retina in the eye to the brain (papilledema) and dysfunction of the cranial nerve in the brain (sixth nerve palsy). Occasionally, individuals may have episodes of confusion.[1]

Diagnosis

HaNDL syndrome is considered a diagnosis of exclusion, meaning it is important to rule out other conditions that may cause similar symptoms in the diagnostic process. Tests that may be used to diagnose this condition include but are not limited to:[2][3]

Diagnostic guidelines were published in the International Classification of Headache Disorders, 3rd edition, which state that HaNDL syndrome is classified as a headache associated with a noninfectious inflammatory disease. The diagnostic criteria includes:[2]

  • Episodes of migraine-like headache lasting at least four hours, during or shortly before neurological symptoms occur.
  • The finding of an abnormally increased level of a type of white blood cells (lymphocytes) in the cerebrospinal fluid (lymphocytic pleocytosis). 
  • Either worsening or improving neurological symptoms depending on the level of lymphocytic pleocytosis. 
  • Exclusion of other similar conditions.

The diagnostic guidelines are freely available to the general public and can be viewed on the International Classification of Headache Disorders website.

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.

    In-Depth Information

    • PubMed is a searchable database of medical literature and lists journal articles that discuss HaNDL syndrome. Click on the link to view a sample search on this topic.

      Selected Full-Text Journal Articles

        References

        1. Valença MM, de Oliveira DA, de L. Martins HA. Alice in Wonderland Syndrome, Burning Mouth Syndrome, Cold Stimulus Headache, and HaNDL: Narrative Review. Headache. October 2015; 55(9):1233-1248. https://www.ncbi.nlm.nih.gov/pubmed/26422755.
        2. Lay CL, Sun-Edelstein C. Syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL). UpToDate. November 17, 2016; https://www.uptodate.com/contents/syndrome-of-transient-headache-and-neurologic-deficits-with-cerebrospinal-fluid-lymphocytosis-handl.
        3. Armiento R, Kornberg AJ. Altered conscious state as a presentation of the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL syndrome) in a paediatric patient. J Paediatr Child Health. July 2016; 52(7):774-776. https://www.ncbi.nlm.nih.gov/pubmed/27243666.

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