Abstract Details

Title Bilateral, Symmetrical, Linear Enhancement of the Deep White Matter in a Radial Distribution on Brain MRI Is an Identifiable Signature of Primary Angiitis of the Central Nervous System (PACNS)

Topic MS and Related Diseases

Presentation(s) P02 - (-)

Poster/Presentation
Number 108

Objective

Background BACKGROUND: PACNS is defined as vasculitis affecting the CNS without systemic disease. It is a rare disease of unclear etiology. It manifests with headache, altered mentation and focal neurological deficits. MRI brain abnormalities are present in 95% of histologically confirmed cases including focal signal changes, bilateral cerebral infarcts, leptomeningeal enhancement or hemorrhagic lesions, although they are nonspecific. Brain biopsy is still the gold standard for diagnosis of PACNS, which is invasive and has a significant false negative rate.

Design/Methods DESIGN/METHODS: Case report and comprehensive literature search was performed for articles published up to September 2012. Relevant articles were retrieved, prioritized and their references checked for related material.

Results RESULTS: A 47yo African American male developed progressive bilateral hand tremor, difficulty walking, cognitive slowing and headache. Physical exam showed bilateral hand tremor, diffuse hyperreflexia, bilateral dysmetria and frontal release signs. Brain MRI showed T2 hyper intensities throughout the white matter in centrum semiovale and corona radiata with diffuse linear enhancement on gadolinium-contrasted study in a radiating fashion. Comprehensive work-up was negative. Brain biopsy revealed granulomatous PACNS. He was treated with steroids and cyclophosphamide and the MRI and clinical exam improved. The literature search revealed five other biopsy-proven cases that described MRI Brain findings similar to our patient. These linear, radial enhancing signal abnormalities follow the path of blood vessels, which is likely to be specific for vasculitis. This configuration is not seen in the MRI scans of patients with multiple sclerosis or post-viral demyelination. This is one of a few cases,but we now observe a pattern that is of diagnostic importance.

Conclusions CONCLUSIONS: The presence of diffuse linear enhancement on gadolinium-contrasted study in a radiating fashion on brain MRI may be a diagnostic signature of PACNS.

Authors/Disclosures
Kartheek Ganta, MD (Cumberland Neurology) PRESENTER	No disclosure on file
Aisha M. Malik, MD (Synapse Neurology)	No disclosure on file
Michael C. Levin, MD, FAAN (University of Saskatchewan)	Dr. Levin has received personal compensation for serving as an employee of Merck. Dr. Levin has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. The institution of Dr. Levin has received research support from CIHR.
	No disclosure on file
Olivier F. Gout, MD (Hôpital Fondation A de Rothschild)	Dr. Gout has nothing to disclose.

��ɫ��

��ɫ��