Abstract Details

Title MRI Shows Unique Symmetrical Centrifugal Migration of a Low Diffusion Front from the Ventricular Horns in HDLS

Topic Neural Repair/Rehabilitation

Presentation(s) P04 - (-)

Poster/Presentation
Number 041

Objective

Background BACKGROUND: HDLS is a devastating adult onset white matter disorder with symptom onset in the 4th decade with a multisystem progressive course, leading to death within a few years.

Design/Methods DESIGN/METHODS: One HDLS patient from the original Swedish family with a moderately fast course, dominated by frontal neuropsychiatric symptoms, was evaluated and followed prospectively with MRI as well as DWI/DTI and MRS for 22 months after symptom onset.

Results RESULTS: In 5 consecutive MRI examinations we found centrifugally expanding regions of diffusion abnormalities around the ventricular horns. These changes had a peripheral border of markedly increased DWI-a signal, demonstrating restriction on mean diffusivity. Behind this rim was an enlarging center of increasing diffusion. These signal abnormalities were most prominent in anterior-WM, but eventually similar changes developed in posterior-WM. The DTI eigenvalues demonstrated changes that suggested early myelin disturbance in normal appearing white matter (NAWM), and complete destruction of axons and myelin in the later stages. These findings were supported by temporal changes on MRS.

Conclusions CONCLUSIONS: Our study supports progressive damage of myelin and axons with a characteristic pattern of centrifugal progression of WM lesions where the expanding front shows restricted diffusion. This pattern may be characteristic of HDLS. Increasing knowledge about histology and DTI/MRS parameters in HDLS will give insights into the mechanisms underlying neurodegeneration, and may be used to monitor potential future treatments.

Authors/Disclosures
Christina Sundal, MD (NeuroClinic Norway) PRESENTER	Dr. Sundal has nothing to disclose.
Paul C. Van Ness, MD, FAAN	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Thomas Lilnden	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file

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