MCID: SPR070
MIFTS: 20

Sporadic Infantile Bilateral Striatal Necrosis

Categories: Genetic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Sporadic Infantile Bilateral Striatal Necrosis

MalaCards integrated aliases for Sporadic Infantile Bilateral Striatal Necrosis:

Name: Sporadic Infantile Bilateral Striatal Necrosis 60
Sporadic Infantile Striatonigral Degeneration 60
Sporadic Infantile Striatonigral Necrosis 60
Acute Bilateral Striatal Necrosis 60
Sporadic Ibsn 60
Absn 60

Characteristics:

Orphanet epidemiological data:

60
sporadic infantile bilateral striatal necrosis
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: All ages;

Classifications:

Orphanet: 60  
Rare neurological diseases


Summaries for Sporadic Infantile Bilateral Striatal Necrosis

MalaCards based summary : Sporadic Infantile Bilateral Striatal Necrosis, also known as sporadic infantile striatonigral degeneration, is related to striatonigral degeneration, infantile and encephalopathy, acute, infection-induced 4. Related phenotypes are dysarthria and dysphagia

Related Diseases for Sporadic Infantile Bilateral Striatal Necrosis

Diseases in the Familial Bilateral Striatal Necrosis family:

Familial Infantile Bilateral Striatal Necrosis Sporadic Infantile Bilateral Striatal Necrosis

Diseases related to Sporadic Infantile Bilateral Striatal Necrosis via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 striatonigral degeneration, infantile 11.3
2 encephalopathy, acute, infection-induced 4 10.0
3 gastroenteritis 10.0
4 acute necrotizing encephalopathy 10.0
5 encephalopathy 10.0

Graphical network of the top 20 diseases related to Sporadic Infantile Bilateral Striatal Necrosis:



Diseases related to Sporadic Infantile Bilateral Striatal Necrosis

Symptoms & Phenotypes for Sporadic Infantile Bilateral Striatal Necrosis

Human phenotypes related to Sporadic Infantile Bilateral Striatal Necrosis:

60 33 (show all 38)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dysarthria 60 33 frequent (33%) Frequent (79-30%) HP:0001260
2 dysphagia 60 33 frequent (33%) Frequent (79-30%) HP:0002015
3 recurrent upper respiratory tract infections 60 33 frequent (33%) Frequent (79-30%) HP:0002788
4 specific learning disability 60 33 frequent (33%) Frequent (79-30%) HP:0001328
5 abnormal posturing 60 33 frequent (33%) Frequent (79-30%) HP:0002533
6 facial grimacing 60 33 frequent (33%) Frequent (79-30%) HP:0000273
7 infantile axial hypotonia 60 33 frequent (33%) Frequent (79-30%) HP:0009062
8 inappropriate crying 60 33 frequent (33%) Frequent (79-30%) HP:0030215
9 hyperreflexia 60 33 occasional (7.5%) Occasional (29-5%) HP:0001347
10 chorea 60 33 occasional (7.5%) Occasional (29-5%) HP:0002072
11 gait ataxia 60 33 occasional (7.5%) Occasional (29-5%) HP:0002066
12 babinski sign 60 33 occasional (7.5%) Occasional (29-5%) HP:0003487
13 dystonia 60 33 occasional (7.5%) Occasional (29-5%) HP:0001332
14 mask-like facies 60 33 occasional (7.5%) Occasional (29-5%) HP:0000298
15 nasogastric tube feeding 60 33 occasional (7.5%) Occasional (29-5%) HP:0040288
16 bradykinesia 60 33 occasional (7.5%) Occasional (29-5%) HP:0002067
17 hemiplegia 60 33 occasional (7.5%) Occasional (29-5%) HP:0002301
18 urinary incontinence 60 33 occasional (7.5%) Occasional (29-5%) HP:0000020
19 poor suck 60 33 occasional (7.5%) Occasional (29-5%) HP:0002033
20 parkinsonism 60 33 occasional (7.5%) Occasional (29-5%) HP:0001300
21 drooling 60 33 occasional (7.5%) Occasional (29-5%) HP:0002307
22 hypomimic face 60 33 occasional (7.5%) Occasional (29-5%) HP:0000338
23 pharyngitis 60 33 occasional (7.5%) Occasional (29-5%) HP:0025439
24 focal seizures, afebril 60 33 occasional (7.5%) Occasional (29-5%) HP:0040168
25 loss of consciousness 60 33 occasional (7.5%) Occasional (29-5%) HP:0007185
26 poor speech 60 33 occasional (7.5%) Occasional (29-5%) HP:0002465
27 mutism 60 33 occasional (7.5%) Occasional (29-5%) HP:0002300
28 resting tremor 60 33 occasional (7.5%) Occasional (29-5%) HP:0002322
29 short attention span 60 33 occasional (7.5%) Occasional (29-5%) HP:0000736
30 recurrent streptococcus pneumoniae infections 60 33 occasional (7.5%) Occasional (29-5%) HP:0005366
31 progressive extrapyramidal muscular rigidity 60 33 occasional (7.5%) Occasional (29-5%) HP:0007158
32 obtundation status 60 33 occasional (7.5%) Occasional (29-5%) HP:0011151
33 titubation 60 33 occasional (7.5%) Occasional (29-5%) HP:0030187
34 seizures 60 Frequent (79-30%)
35 gait disturbance 60 Occasional (29-5%)
36 reduced consciousness/confusion 60 Occasional (29-5%)
37 abnormality of movement 60 Frequent (79-30%)
38 infantile muscular hypotonia 60 Occasional (29-5%)

Drugs & Therapeutics for Sporadic Infantile Bilateral Striatal Necrosis

Search Clinical Trials , NIH Clinical Center for Sporadic Infantile Bilateral Striatal Necrosis

Genetic Tests for Sporadic Infantile Bilateral Striatal Necrosis

Anatomical Context for Sporadic Infantile Bilateral Striatal Necrosis

Publications for Sporadic Infantile Bilateral Striatal Necrosis

Articles related to Sporadic Infantile Bilateral Striatal Necrosis:

# Title Authors Year
1
Acute bilateral striatal necrosis with rotavirus gastroenteritis and inborn metabolic predisposition. ( 15934490 )
2005
2
Must acute necrotizing encephalopathy of childhood and acute bilateral striatal necrosis be differentiated? ( 15087115 )
2004

Variations for Sporadic Infantile Bilateral Striatal Necrosis

Expression for Sporadic Infantile Bilateral Striatal Necrosis

Search GEO for disease gene expression data for Sporadic Infantile Bilateral Striatal Necrosis.

Pathways for Sporadic Infantile Bilateral Striatal Necrosis

GO Terms for Sporadic Infantile Bilateral Striatal Necrosis

Sources for Sporadic Infantile Bilateral Striatal Necrosis

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63 PubMed
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70 SNOMED-CT via HPO
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75 UMLS via Orphanet
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