PACNS
MCID: PRM039
MIFTS: 33

Primary Angiitis of the Central Nervous System (PACNS)

Categories: Bone diseases, Cardiovascular diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Primary Angiitis of the Central Nervous System

MalaCards integrated aliases for Primary Angiitis of the Central Nervous System:

Name: Primary Angiitis of the Central Nervous System 52 58 71
Primary Central Nervous System Vasculitis 52 58
Primary Cns Vasculitis 52 71
Pacns 52 58
Granulomatous Angiitis of the Central Nervous System 52
Primary Vasculitis of the Central Nervous System 58
Isolated Angiitis of the Central Nervous System 58
Pcnsv 58

Characteristics:

Orphanet epidemiological data:

58
primary angiitis of the central nervous system
Inheritance: Not applicable; Age of onset: All ages; Age of death: adult;

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare circulatory system diseases
Rare systemic and rhumatological diseases


External Ids:

MESH via Orphanet 44 C535276 D020293
ICD10 via Orphanet 33 I67.7
UMLS via Orphanet 72 C0751881 C2930862
Orphanet 58 ORPHA140989
UMLS 71 C0751881 C2930862

Summaries for Primary Angiitis of the Central Nervous System

NIH Rare Diseases : 52 Primary angiitis of the central nervous system is a rare form of vasculitis (inflammation of blood vessels) affecting the blood vessels that nourish the brain, spinal cord and peripheral nerves. This condition can lead to narrowing and blockage of the blood vessels of the central nervous system which can eventually cause aneurysms , ischemia and/or hemmorrhage . The cause of this condition is unknown. Signs and symptoms of this condition may begin suddenly or develop over time. Some of the symptoms may incude headaches that do not go away, fever, rapid weight loss, confusion or forgetfulness, and general malaise. Treatment for this condition involves a course of immunosuppresive steroids .

MalaCards based summary : Primary Angiitis of the Central Nervous System, also known as primary central nervous system vasculitis, is related to vasculitis and reversible cerebral vasoconstriction syndrome. Affiliated tissues include brain, spinal cord and testes, and related phenotypes are cerebral vasculitis and nausea and vomiting

Related Diseases for Primary Angiitis of the Central Nervous System

Diseases in the Benign Angiitis of the Central Nervous System family:

Primary Angiitis of the Central Nervous System

Diseases related to Primary Angiitis of the Central Nervous System via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 120)
# Related Disease Score Top Affiliating Genes
1 vasculitis 10.9
2 reversible cerebral vasoconstriction syndrome 10.6
3 encephalopathy 10.5
4 headache 10.5
5 cerebral amyloid angiopathy, cst3-related 10.4
6 vasculitis, lymphocytic, nodular 10.4
7 ataxia and polyneuropathy, adult-onset 10.4
8 thunderclap headache 10.4
9 sarcoidosis 1 10.4
10 meningoencephalitis 10.3
11 granulomatous angiitis 10.3
12 central nervous system vasculitis 10.3
13 tremor 10.3
14 cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy, type 1 10.2
15 moyamoya disease 1 10.2
16 ocular motor apraxia 10.2
17 autoimmune vasculitis 10.2
18 hemiplegia 10.2
19 visual epilepsy 10.2
20 dementia 10.2
21 uveitis 10.2
22 status epilepticus 10.2
23 demyelinating disease 10.2
24 47,xyy 10.2
25 hypersomnia 10.2
26 seizure disorder 10.2
27 specific language disorder 10.2
28 lymphoma, hodgkin, classic 10.1
29 leukemia, chronic myeloid 10.1
30 viral meningitis 10.1
31 gastritis 10.1
32 gerstmann syndrome 10.1
33 chickenpox 10.1
34 myeloid leukemia 10.1
35 herpes simplex encephalitis 10.1
36 herpes zoster ophthalmicus 10.1
37 lymphoma 10.1
38 transient cerebral ischemia 10.1
39 myelodysplastic syndrome 10.1
40 toxoplasmosis 10.1
41 asymptomatic neurosyphilis 10.1
42 atypical depressive disorder 10.1
43 binswanger's disease 10.1
44 neurosyphilis 10.1
45 anisocoria 10.1
46 atherosclerosis susceptibility 10.1
47 creutzfeldt-jakob disease 10.1
48 hypercholesterolemia, familial, 1 10.1
49 migraine with or without aura 1 10.1
50 temporal arteritis 10.1

Graphical network of the top 20 diseases related to Primary Angiitis of the Central Nervous System:



Diseases related to Primary Angiitis of the Central Nervous System

Symptoms & Phenotypes for Primary Angiitis of the Central Nervous System

Human phenotypes related to Primary Angiitis of the Central Nervous System:

58 31 (show all 31)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 cerebral vasculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0005318
2 nausea and vomiting 58 31 frequent (33%) Frequent (79-30%) HP:0002017
3 transient ischemic attack 58 31 frequent (33%) Frequent (79-30%) HP:0002326
4 headache 58 31 frequent (33%) Frequent (79-30%) HP:0002315
5 aphasia 58 31 frequent (33%) Frequent (79-30%) HP:0002381
6 hemiparesis 58 31 frequent (33%) Frequent (79-30%) HP:0001269
7 paralysis 58 31 frequent (33%) Frequent (79-30%) HP:0003470
8 multifocal cerebral white matter abnormalities 58 31 frequent (33%) Frequent (79-30%) HP:0007052
9 csf pleocytosis 58 31 frequent (33%) Frequent (79-30%) HP:0012229
10 abnormal csf protein level 58 31 frequent (33%) Frequent (79-30%) HP:0025456
11 recurrent subcortical infarcts 58 31 frequent (33%) Frequent (79-30%) HP:0007236
12 fever 58 31 occasional (7.5%) Occasional (29-5%) HP:0001945
13 ataxia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001251
14 dysarthria 58 31 occasional (7.5%) Occasional (29-5%) HP:0001260
15 reduced visual acuity 58 31 occasional (7.5%) Occasional (29-5%) HP:0007663
16 intracranial hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0002170
17 diplopia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000651
18 tetraparesis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002273
19 paraparesis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002385
20 blurred vision 58 31 occasional (7.5%) Occasional (29-5%) HP:0000622
21 transient global amnesia 58 31 occasional (7.5%) Occasional (29-5%) HP:0010534
22 abnormal visual field test 58 31 occasional (7.5%) Occasional (29-5%) HP:0030588
23 seizure 31 occasional (7.5%) HP:0001250
24 vertigo 58 31 very rare (1%) Very rare (<4-1%) HP:0002321
25 amaurosis fugax 58 31 very rare (1%) Very rare (<4-1%) HP:0100576
26 pseudopapilledema 58 31 very rare (1%) Very rare (<4-1%) HP:0000538
27 parkinsonism 58 31 very rare (1%) Very rare (<4-1%) HP:0001300
28 constitutional symptom 58 31 very rare (1%) Very rare (<4-1%) HP:0025142
29 seizures 58 Occasional (29-5%)
30 cognitive impairment 58 Frequent (79-30%)
31 stroke 58 Frequent (79-30%)

Drugs & Therapeutics for Primary Angiitis of the Central Nervous System

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Chronic Childhood Vasculitis: Characterizing the Individual Rare Diseases to Improve Patient Outcomes Unknown status NCT02006134
2 The Utility of Intracranial Vessel Wall Imaging for Suspected CNS Vasculitis Diagnosis and Assessment of Disease Activity. Active, not recruiting NCT03166319

Search NIH Clinical Center for Primary Angiitis of the Central Nervous System

Genetic Tests for Primary Angiitis of the Central Nervous System

Anatomical Context for Primary Angiitis of the Central Nervous System

MalaCards organs/tissues related to Primary Angiitis of the Central Nervous System:

40
Brain, Spinal Cord, Testes, Bone, Heart, Lung, B Cells

Publications for Primary Angiitis of the Central Nervous System

Articles related to Primary Angiitis of the Central Nervous System:

(show top 50) (show all 298)
# Title Authors PMID Year
1
Subacute cognitive impairment as clinical presentation of primary angiitis of the central nervous system. 61
31564426 2020
2
Intracranial Vessel Wall MRI in Cryptogenic Stroke and Intracranial Vasculitis. 61
32151477 2020
3
Recurrent intracranial hemorrhage and cerebral venous sinus thrombosis: an atypical case of Neuro-Behçet's Syndrome. 61
31981998 2020
4
The diagnosis of primary central nervous system vasculitis. 61
31649101 2020
5
An ischemic stroke as the presenting manifestation of rapidly progressive primary angiitis of central nervous system in a 17-year-old boy. 61
32088635 2020
6
Intracranial vessel wall imaging: applications, interpretation, and pitfalls. 61
32197916 2020
7
Primary CNS lymphoma initially diagnosed as vasculitis. 61
32190424 2020
8
Circulating Endothelial Cells as Promising Biomarkers in the Differential Diagnosis of Primary Angiitis of the Central Nervous System. 61
32296382 2020
9
Initial experience with [18F]DPA-714 TSPO-PET to image inflammation in primary angiitis of the central nervous system. 61
31960097 2020
10
Reversible Cerebral Vasoconstriction Syndrome: A Severe Neurological Complication in Postpartum Period. 61
32129278 2020
11
Childhood Small Vessel Primary Angiitis of the Central Nervous System: A Treatable Cause of Super-refractory Status Epilepticus. 61
31530228 2020
12
Concordance of angiography and cerebral biopsy results for suspected primary central nervous system vasculitis: A multi-center retrospective review. 61
31421586 2019
13
Ataxia as the main manifestation of tumor-like primary angiitis of the central nervous system: a case report and literature review. 61
31615440 2019
14
Primary Angiitis of the Central Nervous System Presenting with Microhemorrhages on Gradient Echo Imaging. 61
31744984 2019
15
[A Case of Cerebral Amyloid Angiopathy-related Subarachnoid Hemorrhage with Parkinson Disease Dementia]. 61
31477632 2019
16
Strategies for treatment of childhood primary angiitis of the central nervous system. 61
31355303 2019
17
Exploratory proteomic analysis implicates the alternative complement cascade in primary CNS vasculitis. 61
31270218 2019
18
CSF Neurofilament light chain level predicts axonal damage in cerebral vasculitis. 61
31211179 2019
19
Relapse rates and long-term outcome in primary angiitis of the central nervous system. 61
30904954 2019
20
Biopsy-proven primary angiitis of the central nervous system mimicking leukodystrophy: A case report and review of the literature. 61
30910547 2019
21
MOG antibody disease manifesting as progressive cognitive deterioration and behavioral changes with primary central nervous system vasculitis. 61
30738279 2019
22
Leading symptoms in cerebrovascular diseases: what about headache? 61
30891639 2019
23
Cognitive outcomes of childhood primary CNS vasculitis. 61
30762375 2019
24
Primary angiitis of the central nervous system (PACNS) - a rare and serious, but treatable entity. 61
31347602 2019
25
Atypical presentation of fulminant primary central nervous system angiitis. 61
30716674 2019
26
[Primary angiitis of the central nervous system with cerebral infarction and spinal hemorrhage]. 61
31061305 2019
27
Study on the Clinical, Imaging, and Pathological Characteristics of 18 Cases with Primary Central Nervous System Vasculitis. 61
30635219 2019
28
Anti-MOG encephalitis mimicking small vessel CNS vasculitis. 61
30800721 2019
29
Long-term outcomes of patients with primary angiitis of the central nervous system. 61
30789149 2019
30
Neuroradiologic Characteristics of Primary Angiitis of the Central Nervous System According to the Affected Vessel Size. 61
28875326 2019
31
A case of primary angiitis of the central nervous system presenting with diffuse cerebral microbleeds and recurrent intracranial hemorrhage. 61
30284656 2019
32
Primary Angiitis of the Central Nervous System: New Potential Imaging Techniques and Biomarkers in Blood and Cerebrospinal Fluid. 61
31244749 2019
33
Primary angiitis of the central nervous system: Clinical profiles and outcomes of 45 patients. 61
30860105 2019
34
Biomarkers in Vasculitides of the Nervous System. 61
31244756 2019
35
Primary angiitis of the central nervous system - An Indian experience. 61
30860106 2019
36
Primary Angiitis of the Central Nervous System: From Psychiatry to Neurology. 61
31781439 2019
37
Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review. 61
31798527 2019
38
Recurrent arterial ischemic stroke with good response to mycophenolate mofetil. 61
30528093 2019
39
Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system. 61
30273791 2018
40
Clinical, imaging, and histological presentations and outcomes of stroke related to sarcoidosis. 61
30109479 2018
41
Primary Angiitis of the Central Nervous System Presenting With Headache and Ataxia. 61
30079846 2018
42
Cerebrospinal fluid findings in reversible cerebral vasoconstriction syndrome: a way to differentiate from cerebral vasculitis? 61
29722896 2018
43
The first case of bacillus Calmette-Guérin-induced small-vessel central nervous system vasculitis. 61
29740727 2018
44
Immune cell profiling in the cerebrospinal fluid of patients with primary angiitis of the central nervous system reflects the heterogeneity of the disease. 61
29957380 2018
45
Primary Angiitis of the Central Nervous System Mimicking Sporadic Creutzfeldt-Jakob Disease: A Case Study. 61
29369829 2018
46
Intracranial Atherosclerosis Versus Primary Angiitis of the Central Nervous System: a Case Report. 61
30254820 2018
47
Rituximab treatment in primary angiitis of the central nervous system. 61
29898271 2018
48
Primary angiitis of the central nervous system mimicking a cerebellar tumor. 61
29656658 2018
49
Primary central nervous system vasculitis associated with lymphoma. 61
29429967 2018
50
[Small vessel-childhood primary angiitis of the central nervous system: a case report and literature review]. 61
29429204 2018

Variations for Primary Angiitis of the Central Nervous System

Expression for Primary Angiitis of the Central Nervous System

Search GEO for disease gene expression data for Primary Angiitis of the Central Nervous System.

Pathways for Primary Angiitis of the Central Nervous System

GO Terms for Primary Angiitis of the Central Nervous System

Sources for Primary Angiitis of the Central Nervous System

3 CDC
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32 ICD10
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43 MeSH
44 MESH via Orphanet
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48 NCI
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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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