MCID: ANG030
MIFTS: 18

Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

MalaCards integrated aliases for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert:

Name: Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert 56 52 71
Corticomeningeal Angiomatosis, Myelination of the White Substance of the Centrum Ovale, Hemianopsia and Marbled Skin 52
Divry-Van Bogaert Syndrome 56

Characteristics:

OMIM:

56
Inheritance:
autosomal recessive
isolated cases

Miscellaneous:
onset in second decade
death about 20 years after symptom onset


HPO:

31
angiomatosis, diffuse corticomeningeal, of divry and van bogaert:
Inheritance autosomal recessive inheritance sporadic


Classifications:



Summaries for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

MalaCards based summary : Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert, also known as corticomeningeal angiomatosis, myelination of the white substance of the centrum ovale, hemianopsia and marbled skin, is related to angiomatosis, and has symptoms including seizures, ataxia and pseudobulbar signs. Affiliated tissues include skin and brain, and related phenotypes are hypertension and ataxia

More information from OMIM: 206570

Related Diseases for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Diseases related to Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 angiomatosis 10.7

Symptoms & Phenotypes for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Human phenotypes related to Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert:

31 (show all 16)
# Description HPO Frequency HPO Source Accession
1 hypertension 31 occasional (7.5%) HP:0000822
2 ataxia 31 HP:0001251
3 dysarthria 31 HP:0001260
4 ventriculomegaly 31 HP:0002119
5 migraine 31 HP:0002076
6 broad-based gait 31 HP:0002136
7 cutis marmorata 31 HP:0000965
8 emotional lability 31 HP:0000712
9 hemianopia 31 HP:0012377
10 dementia 31 HP:0000726
11 visual field defect 31 HP:0001123
12 brain atrophy 31 HP:0012444
13 pseudobulbar signs 31 HP:0002200
14 seizure 31 HP:0001250
15 aplasia/hypoplasia involving the central nervous system 31 HP:0002977
16 telangiectases producing 'marbled' skin 31 HP:0007586

Symptoms via clinical synopsis from OMIM:

56
Neurologic Central Nervous System:
seizures
ataxia
dysarthria
migraine
broad-based gait
more
Neurologic Behavioral Psychiatric Manifestations:
emotional lability

Cardiovascular Vascular:
hypertension (reported in 1 patient)
leptomeningeal angiomatosis
distal tapering of the carotid, cerebellar, and meningeal arteries
prominent venous pattern

Skin Nails Hair Skin:
cutis marmorata
prominent venous pattern
marbled skin due to telangiectasia

Head And Neck Eyes:
visual field defects
hemianopsia

Clinical features from OMIM:

206570

UMLS symptoms related to Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert:


seizures, ataxia, pseudobulbar signs

Drugs & Therapeutics for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Search Clinical Trials , NIH Clinical Center for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Genetic Tests for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Anatomical Context for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

MalaCards organs/tissues related to Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert:

40
Skin, Brain

Publications for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Articles related to Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert:

# Title Authors PMID Year
1
Divry-Van Bogaert syndrome. Clinical and ultrastructural findings. 61 56
6721724 1984
2
Familial capillaro-venous leptomeningeal angiomatosis. 56
4698574 1973
3
[Not Available]. 56
20274395 1946
4
The diagnostic challenge of Divry van Bogaert and Sneddon Syndrome: Report of three cases and literature review. 61
27084221 2016
5
The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa. A literature review. 61
16133722 2005
6
Intracerebral hemorrhage associated with Sneddon's syndrome: is ischemia-related angiogenesis the cause? Case report and review of the literature. 61
12750866 2003
7
Divry-Van Bogaert syndrome in a female: relationship to Sneddon's syndrome and radiographic appearances. 61
11512587 2001

Variations for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Expression for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Search GEO for disease gene expression data for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert.

Pathways for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

GO Terms for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

Sources for Angiomatosis, Diffuse Corticomeningeal, of Divry and Van Bogaert

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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