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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Juvenile Huntington disease


Other Names for this Disease

  • Huntington disease, juvenile onset
  • JHD
  • Juvenile onset HD
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Symptoms

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What are the signs and symptoms of Juvenile Huntington disease (HD)?

A common sign of juvenile HD is a rapid decline in school performance. Symptoms can also include subtle changes in handwriting and slight problems with movement, such as slowness, rigidity, tremor, and rapid muscular twitching, called myoclonus. Several of these symptoms are similar to those seen in Parkinson's disease, and they differ from the chorea seen in individuals who develop the disease as adults. People with juvenile HD may also have seizures and mental disabilities. The earlier the onset, the faster the disease seems to progress. The disease progresses most rapidly in individuals with juvenile or early-onset HD, and death often follows within 10 years.[1]
Last updated: 3/15/2011

The Human Phenotype Ontology provides the following list of signs and symptoms for Juvenile Huntington disease. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Abnormality of movement 50%
Abnormality of the voice 50%
Behavioral abnormality 50%
Cerebral cortical atrophy 50%
Developmental regression 50%
EEG abnormality 50%
Hypertonia 50%
Rigidity 7.5%
Abnormality of eye movement -
Autosomal dominant inheritance -
Bradykinesia -
Chorea -
Dementia -
Depression -
Gliosis -
Hyperreflexia -
Neuronal loss in central nervous system -
Personality changes -

Last updated: 12/1/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Huntington Disease. National Institute of Neurological Disorders and Stroke (NINDS). August 2010; http://www.ninds.nih.gov/disorders/huntington/detail_huntington.htm. Accessed 3/15/2011.


Other Names for this Disease
  • Huntington disease, juvenile onset
  • JHD
  • Juvenile onset HD
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.