ABSN
MCID: SPR070
MIFTS: 17

Sporadic Infantile Bilateral Striatal Necrosis (ABSN)

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 59
Sporadic Infantile Striatonigral Degeneration 59
Sporadic Infantile Striatonigral Necrosis 59
Acute Bilateral Striatal Necrosis 59
Sporadic Ibsn 59
Absn 59

Characteristics:

Orphanet epidemiological data:

59
sporadic infantile bilateral striatal necrosis
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

ICD10 via Orphanet 34 G23.2
Orphanet 59 ORPHA225147

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. 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 11.4
2 encephalopathy 10.1
3 gastroenteritis 10.0
4 encephalitis 10.0
5 acute necrotizing 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:

59 32 (show all 38)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dysarthria 59 32 frequent (33%) Frequent (79-30%) HP:0001260
2 dysphagia 59 32 frequent (33%) Frequent (79-30%) HP:0002015
3 recurrent upper respiratory tract infections 59 32 frequent (33%) Frequent (79-30%) HP:0002788
4 specific learning disability 59 32 frequent (33%) Frequent (79-30%) HP:0001328
5 inappropriate crying 59 32 frequent (33%) Frequent (79-30%) HP:0030215
6 infantile axial hypotonia 59 32 frequent (33%) Frequent (79-30%) HP:0009062
7 abnormal posturing 59 32 frequent (33%) Frequent (79-30%) HP:0002533
8 facial grimacing 59 32 frequent (33%) Frequent (79-30%) HP:0000273
9 hyperreflexia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001347
10 chorea 59 32 occasional (7.5%) Occasional (29-5%) HP:0002072
11 hypomimic face 59 32 occasional (7.5%) Occasional (29-5%) HP:0000338
12 nasogastric tube feeding 59 32 occasional (7.5%) Occasional (29-5%) HP:0040288
13 gait ataxia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002066
14 babinski sign 59 32 occasional (7.5%) Occasional (29-5%) HP:0003487
15 dystonia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001332
16 mask-like facies 59 32 occasional (7.5%) Occasional (29-5%) HP:0000298
17 bradykinesia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002067
18 hemiplegia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002301
19 urinary incontinence 59 32 occasional (7.5%) Occasional (29-5%) HP:0000020
20 poor suck 59 32 occasional (7.5%) Occasional (29-5%) HP:0002033
21 parkinsonism 59 32 occasional (7.5%) Occasional (29-5%) HP:0001300
22 drooling 59 32 occasional (7.5%) Occasional (29-5%) HP:0002307
23 pharyngitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0025439
24 poor speech 59 32 occasional (7.5%) Occasional (29-5%) HP:0002465
25 focal seizures, afebril 59 32 occasional (7.5%) Occasional (29-5%) HP:0040168
26 loss of consciousness 59 32 occasional (7.5%) Occasional (29-5%) HP:0007185
27 mutism 59 32 occasional (7.5%) Occasional (29-5%) HP:0002300
28 resting tremor 59 32 occasional (7.5%) Occasional (29-5%) HP:0002322
29 short attention span 59 32 occasional (7.5%) Occasional (29-5%) HP:0000736
30 recurrent streptococcus pneumoniae infections 59 32 occasional (7.5%) Occasional (29-5%) HP:0005366
31 progressive extrapyramidal muscular rigidity 59 32 occasional (7.5%) Occasional (29-5%) HP:0007158
32 obtundation status 59 32 occasional (7.5%) Occasional (29-5%) HP:0011151
33 titubation 59 32 occasional (7.5%) Occasional (29-5%) HP:0030187
34 seizures 59 Frequent (79-30%)
35 gait disturbance 59 Occasional (29-5%)
36 reduced consciousness/confusion 59 Occasional (29-5%)
37 abnormality of movement 59 Frequent (79-30%)
38 infantile muscular hypotonia 59 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 PMID Year
1
Infantile bilateral striatal necrosis associated with human herpes virus-6 (HHV-6) infection. 38
16198210 2005
2
Acute bilateral striatal necrosis with rotavirus gastroenteritis and inborn metabolic predisposition. 38
15934490 2005
3
Must acute necrotizing encephalopathy of childhood and acute bilateral striatal necrosis be differentiated? 38
15087115 2004
4
Acute bilateral striatal necrosis associated with Mycoplasma pneumoniae infection. 38
8970225 1996
5
[Acute basal ganglia necrosis with favorable course during Mycoplasma encepahlitis]. 38
8952776 1996
6
Acute bilateral striatal necrosis in an infant: CT and MRI. 38
8041453 1994

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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