National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Primary angiitis of the central nervous system


Other Names:
PACNS; Primary central nervous system vasculitis; Primary CNS vasculitis; PACNS; Primary central nervous system vasculitis; Primary CNS vasculitis; Granulomatous angiitis of the central nervous system See More
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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.[1][2]
Last updated: 10/31/2012

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Cerebral vasculitis 0005318
30%-79% of people have these symptoms
Abnormal CSF protein level 0025456
Aphasia
Difficulty finding words
Losing words
Loss of words
[ more ] 		0002381
CSF pleocytosis 0012229
Headache
Headaches
0002315
Hemiparesis
Weakness of one side of body
0001269
Multifocal cerebral white matter abnormalities 0007052
Nausea and vomiting 0002017
Paralysis
Inability to move
0003470
Recurrent subcortical infarcts 0007236
Transient ischemic attack
Mini stroke
0002326
5%-29% of people have these symptoms
Abnormal visual field test 0030588
Ataxia 0001251
Blurred vision 0000622
Diplopia
Double vision
0000651
Dysarthria
Difficulty articulating speech
0001260
Fever 0001945
Intracranial hemorrhage
Bleeding within the skull
0002170
Paraparesis
Partial paralysis of legs
0002385
Reduced visual acuity
Decreased clarity of vision
0007663
Seizure 0001250
Tetraparesis 0002273
Transient global amnesia 0010534
1%-4% of people have these symptoms
Amaurosis fugax 0100576
Constitutional symptom 0025142
Parkinsonism 0001300
Pseudopapilledema 0000538
Vertigo
Dizzy spell
0002321
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Last updated: 7/1/2020
The current treatment recommendation is to start with oral prednisone at a dose of 1 mg/kg per day and cyclophosphamide at a dose of 2 mg/kg per day. Most centers use prednisone and cyclophosphamide for 4-6 months to induce clinical remission, and then taper prednisone off.[1] Patients generally stay on cyclophosphamide therapy between three and six months, depending on when remission occurs and if there are any potential side effects from cyclophosphamide. Once cyclophosphamide is discontinued, it should be replaced with a less toxic medication for an additional six to twelve months of maintenance therapy. Some doctors switch from cyclophosphamide to azathioprine (2 mg/kg) or mycophenolate mofetil. Methotrexate can also be used, but may be limited by its difficulty to cross the blood brain barrier. There is limited data on how long the maintenance therapy lasts so the decision on the duration of the therapy should be individualized, based upon how the patient responds to therapy. [3]
Last updated: 10/31/2012

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

  • If your physician would like to review your case with a vasculitis specialist, the Vasculitis Foundation lists vasculitis medical consultants that may be able to speak with your healthcare provider. Click on the link above to learn more.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Primary angiitis of the central nervous system. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Noufal M. Primary Angiitis of the CNS. Medscape Reference. April 2012; http://emedicine.medscape.com/article/1484146-treatment. Accessed 10/29/2012.
  2. NINDS. Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet. February 7, 2012; http://www.ninds.nih.gov/disorders/vasculitis/detail_vasculitis.htm. Accessed 10/31/2012.
  3. Hajj-Ali, R., Calabrese L. Primary angiitis of the centeral nervous system. UpToDate. April 18, 2012; http://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system#H30. Accessed 10/29/2012.