DiseaseID 26440

脊髓小脑性共济失调1型

Spinocerebellar Ataxia 1

SNOMEDCT_US_2016_09_01:Main features described as dysarthria, writing difficulties, limb ataxia, and commonly nystagmus and saccadic abnormalities. The disease typically presents in the fourth decade. Ataxia gradually pr

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Disease: 1Symptom: 1Target: 12Links: 13
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Record Fields

Scalar fields from the final disease record.

Disease Id
26440
Core Entity Id
119008
Source Entity Count
1
Preferred Name
Spinocerebellar Ataxia 1
Name Cn
脊髓小脑性共济失调1型
Name Pinyin
Ji Sui Xiao Nao Xing Gong Ji Shi Tiao 1 Xing
Name En
Spinocerebellar Ataxia 1
Name Latin
Bilingual Status
complete
Disease Type
Umls Disease Type
Disgenet Type
Mesh Class
Do Class
Hpo Class
Mesh Class Name
Hpo Class Name
Do Class Name
Disease Definition
SNOMEDCT_US_2016_09_01:Main features described as dysarthria, writing difficulties, limb ataxia, and commonly nystagmus and saccadic abnormalities. The disease typically presents in the fourth decade. Ataxia gradually progresses and additional features may emerge including proprioceptive loss, hypoactive reflexes, ophthalmoparesis, and mild optic neuropathy. Initial presentation with blepharospasm, oromandibular dystonia, and retrocollis preceding ataxia has been reported. Caused by CAG repeat expansions in the ATXN1 gene region on chromosome 6p23.
Version
v1,v2
Suppressed
No

Names

Preferred names, aliases, and source labels retained in the final schema.

Name
Spinocerebellar Ataxia 1
Role
preferred
Source
SymMap_v2
Preferred
Yes

Cross References

Trusted external identifiers retained for this final record.

Umls
C0752120
Sym Map
SMDE03466

Attributes

Merged source attributes and domain-specific metadata.

Version
v1,v2
Suppress
0
Disease Definition
SNOMEDCT_US_2016_09_01:Main features described as dysarthria, writing difficulties, limb ataxia, and commonly nystagmus and saccadic abnormalities. The disease typically presents in the fourth decade. Ataxia gradually progresses and additional features may emerge including proprioceptive loss, hypoactive reflexes, ophthalmoparesis, and mild optic neuropathy. Initial presentation with blepharospasm, oromandibular dystonia, and retrocollis preceding ataxia has been reported. Caused by CAG repeat expansions in the ATXN1 gene region on chromosome 6p23.