Distinguishing primary angiitis of the central nervous system from cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: The importance of family history

Eric E. Williamson, Felix E. Chukwudelunzu, James F Meschia, Robert J. Witte, Dennis W Dickson, Marc D. Cohen

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetically linked neurologic disease characterized by recurrent strokes and progressive or stepwise dementia, with or without migraine-like headaches, seizures, and pseudobulbar palsy. We describe a patient referred with a diagnosis of treatment-refractory primary angiitis of the central nervous system. Meningocortical and skin biopsies confirmed that the patient had CADASIL. Clinical and radiographic differences in these disorders may be subtle, but awareness of them is crucial if the patient is to avoid unnecessary exposure to potentially deleterious immunosuppressive therapy.

Original languageEnglish (US)
Pages (from-to)2243-2248
Number of pages6
JournalArthritis and Rheumatism
Volume42
Issue number10
DOIs
StatePublished - Oct 1999

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CADASIL
Pseudobulbar Palsy
Immunosuppressive Agents
Nervous System Diseases
Migraine Disorders
Dementia
Seizures
Stroke
Biopsy
Skin
Therapeutics
Primary angiitis of the central nervous system

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

Cite this

Distinguishing primary angiitis of the central nervous system from cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy : The importance of family history. / Williamson, Eric E.; Chukwudelunzu, Felix E.; Meschia, James F; Witte, Robert J.; Dickson, Dennis W; Cohen, Marc D.

In: Arthritis and Rheumatism, Vol. 42, No. 10, 10.1999, p. 2243-2248.

Research output: Contribution to journalArticle

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