| HC | hair cell; hairy cell; handicapped; head circumference; head compression; health care; healthy contr... |
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| HD | Haab-Dimmer [syndrome]; Hajna-Damon [broth]; Hansen disease; hearing distance; heart disease; helix ... |
| HDA | heteroduplex analysis; Huntington Disease Association; hydroxydopamine |
| HC | Huntington chorea |
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| UHDRS | Unified Huntington Disease Rating Scale |
| Huntington | George, U.S. Physician, 1850-1916. See: Huntington's chorea, Huntington's disease. (05 Mar 2000) |
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| Huntington chorea | <neurology> An inherited adult-onset disease of the central nervous system. It is characterised by dementia and bizarre involuntary movements. The disease is progressive and there is currently no known cure. The identification of the gene (huntingtin) on chromosome 4p now allows for direct mutation analysis. The gene contains a trinucleotide repeat (CAG) that is found to be expanded in length in affected patients. The normal allele size ranges from 11 to 34 triplet repeat units, while 42 repeats or greater is considered diagnostic of Huntington disease. As in other trinucleotide repeat disorders, the phenomenom of anticipation has been observed, in this case expressed as earlier age of onset in offspring, particularly with paternal transmission. A further complication is the presence of two neighboring trinucleotide repeats (both CCG) which can expand independently without causing the disease. Early PCR primer sets encompassed these adjacent repeats, potentially yielding false positive test results, newer primers hone in more closely on the CAG repeat sequence. Careful attention must be paid to the psychosocial support structure of prospective test subjects in Huntington disease genetic counseling. Established protocols require systematic neuropsychiatric assessment and informed consent prior to DNA testing. Inheritance: autosomal dominant. (29 Dec 1997) |
| Huntington disease | <neurology> An inherited adult-onset disease of the central nervous system. It is characterised by dementia and bizarre involuntary movements. The disease is progressive and there is currently no known cure. The identification of the gene (huntingtin) on chromosome 4p now allows for direct mutation analysis. The gene contains a trinucleotide repeat (CAG) that is found to be expanded in length in affected patients. The normal allele size ranges from 11 to 34 triplet repeat units, while 42 repeats or greater is considered diagnostic of Huntington disease. As in other trinucleotide repeat disorders, the phenomenom of anticipation has been observed, in this case expressed as earlier age of onset in offspring, particularly with paternal transmission. A further complication is the presence of two neighboring trinucleotide repeats (both CCG) which can expand independently without causing the disease. Early PCR primer sets encompassed these adjacent repeats, potentially yielding false positive test results, newer primers hone in more closely on the CAG repeat sequence. Careful attention must be paid to the psychosocial support structure of prospective test subjects in Huntington disease genetic counseling. Established protocols require systematic neuropsychiatric assessment and informed consent prior to DNA testing. Inheritance: autosomal dominant. (29 Dec 1997) |
| Huntington's chorea | A progressive disorder usually beginning in young to middle age, consisting of a triad of choreoathetosis, dementia, and autosomal dominant inheritance with complete penetrance. Bilateral marked wasting of the putamen and the head of the caudate nucleus is characteristic. Synonym: chronic progressive chorea, degenerative chorea, hereditary chorea, Huntington's disease. (05 Mar 2000) |
| huntington's disease | An hereditary disorder with mental and physical deterioration leading to death. Although characterised as an adult-onset disease (as is usually the case), we have seen children with full-blown huntington's disease. (12 Dec 1998) |
| disease, huntington's | An hereditary disorder with mental and physical deterioration leading to death. Although characterised as an adult-onset disease (as is usually the case), we have seen children with full-blown Huntington's disease. (12 Dec 1998) |
| Huntington's s. |
the patient lies supine, with lower limbs hanging over the edge of a table, and is told to cough. If the coughing produces flexion of the thigh and extension of a paralyzed lower limb, it indicates that the paralysis is due to an upper motor neuron lesion.
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