| 释义 |
subacute sclerosing panencephalitis
subacute scle·ros·ing panencephalitis S0833050 (sklə-rō′sĭng)n. An often fatal degenerative disease of the central nervous system occurring chiefly in young people, caused by slow infection with a measles virus and characterized by progressive loss of mental and motor functions ending in dementia and paralysis.
[sclerosing, from sclerose, to harden, back-formation from sclerosed.]sub′acute scle•ros′ing pan′en•ceph•a•li′tis (sklɪˈroʊ sɪŋ ˈpæn ɛnˌsɛf əˈlaɪ tɪs, ˌpæn-)
n. an infection of the central nervous system caused by the measles virus, occurring in children and adolescents several years after a measles attack and characterized by progressive personality changes, seizures, and muscular incoordination. [1950] Thesaurus| Noun | 1. | subacute sclerosing panencephalitis - a rare chronic progressive encephalitis caused by the measles virus and occurring primarily in children and young adults; death usually occurs within three years; characterized by primary measles infection before the age of two yearsBosin's disease, Dawson's encephalitis, inclusion body encephalitis, sclerosing leukoencephalitis, SSPE, subacute inclusion body encephalitis, subacute sclerosing leukoencephalitis, Van Bogaert encephalitispanencephalitis - diffuse inflammation of the entire brain |
Translationssubacute sclerosing panencephalitis
Subacute Sclerosing Panencephalitis DefinitionSubacute sclerosing panencephalitis is a rare, progressive brain disorder caused by an abnormal immune response to the measles virus.DescriptionThis fatal condition is a complication of measles, and affects children and young adults before the age of 20. It usually occurs in boys more often than in girls, but is extremely rare, appearing in only one out of a million cases of measles.Causes and symptomsExperts believe this condition is a form of measles encephalitis (swelling of the brain), caused by an improper response by the immune system to the measles virus.The condition begins with behavioral changes, memory loss, irritability, and problems with school work. As the neurological damage increases, the child experiences seizures, involuntary movements, and further neurological deterioration. Eventually, the child starts suffering from progressive dementia. The optic nerve begins to shrink and weaken (atrophy) and subsequently the child becomes blind.DiagnosisBlood tests and spinal fluid reveal high levels of antibodies to measles virus, and there is a characteristically abnormal electroencephalogram (EEG), or brain wave test. Typically, there is a history of measles infection two to ten years before symptoms begin.TreatmentThere is no standard treatment, and a number of antiviral drugs have been tested with little success. Treatment of symptoms, including the use of anticonvulsant drugs, can be helpful.PrognosisWhile there may be periodic remissions during the course of this disease, it is usually fatal (often from pneumonia) within one to three years after onset.Key termsMeasles encephalitis — A serious complication of measles occurring in about one out of every 1,000 cases, causing headache, drowsiness, and vomiting seven to ten days after the rash appears. Seizures and coma can follow, which may lead to retardation and death.ResourcesOrganizationsNational Institute of Allergy and Infectious Disease. Building 31, Room 7A-50, 31 Center Drive MSC 2520, Bethesda, MD 20892-2520. (301) 496-5717. http://www.niaid.nih.gov/default.htm.National Organization for Rare Disorders. P.O. Box 8923, New Fairfield, CT 06812-8923. (800) 999-6673. http://www.rarediseases.org.sub·a·cute scle·ros·ing pan·en·ceph·a·li·tis (SSPE), [MIM*260470] a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by the measles virus. Characterized by a history of primary measles infection before the age of 2 years, followed by several asymptomatic years, and then gradual, progressive psychoneurologic deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma. Characteristic periodic activity is seen on EEG; pathologically, the white matter of both the hemispheres and brainstem is affected, as well as the cerebral cortex, and eosinophilic inclusion bodies are present in the cytoplasm nuclei of neurons and glial cells. Death usually occurs within 3 years. Synonym(s): Bosin disease, Dawson encephalitis, inclusion body encephalitis, sclerosing leukoencephalitis, subacute inclusion body encephalitis, subacute sclerosing leukoencephalitis, van Bogaert encephalitissubacute sclerosing panencephalitis (sklə-rō′sĭng)n. An often fatal degenerative disease of the central nervous system occurring chiefly in young people, caused by slow infection with a measles virus and characterized by progressive loss of mental and motor functions ending in dementia and paralysis.subacute sclerosing panencephalitis Dawson's encephalitis, SSPE Neurology A slow virus-induced inflammation evoked by the measles virus or its vaccines Clinical Onset in childhood, with mental dysfunction, dyskinesia, myoclonus, hypotonia, emotional lability; it is usually fatal in 1-3 yrs Lab Paretic gold curve with ↑ CSF Igs Management None. Cf Prion. sub·a·cute scle·ros·ing pan·en·ceph·a·li·tis (SSPE) (sŭb'ă-kyūt' skler-ōs'ing pan'en-sef'ă-lī'tis) A rare chronic, progressive encephalitis that affects primarily children and young adults, caused by the measles virus. Characterized by a history of primary measles infection before the age of 2 years, followed by several asymptomatic years, then gradual, progressive psychoneurologic deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma. Characteristic periodic activity is seen on electroencephalogram; pathologically, the white matter of both the hemispheres and brainstem is affected, as well as the cerebral cortex, and eosinophilic inclusion bodies are present in the cytoplasm nuclei of neurons and glial cells. Death usually occurs within 3 years.
Synonym(s): Bosin disease, Dawson encephalitis. Dawson, James R., U.S. pathologist, 1908–. Dawson encephalitis - a rare chronic, progressive encephalitis caused by the measles virus. Synonym(s): subacute sclerosing panencephalitis
van Bogaert, Ludo, Belgian neurologist, 1897–. Canavan-van Bogaert-Bertrand disease - Synonym(s): Canavan diseaseDivry-van Bogaert disease - see under DivryNyssen-van Bogaert-Meyer syndrome - see under Nyssenvan Bogaert diseasevan Bogaert encephalitis - a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by the measles virus. Synonym(s): subacute sclerosing panencephalitisSee SSPE
See SSPEsubacute sclerosing panencephalitis
Synonyms for subacute sclerosing panencephalitisnoun a rare chronic progressive encephalitis caused by the measles virus and occurring primarily in children and young adultsSynonyms- Bosin's disease
- Dawson's encephalitis
- inclusion body encephalitis
- sclerosing leukoencephalitis
- SSPE
- subacute inclusion body encephalitis
- subacute sclerosing leukoencephalitis
- Van Bogaert encephalitis
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