Radiological correlates of episodes of acute decline in the leukodystrophy vanishing white matter

Purpose Patients with vanishing white matter (VWM) experience unremitting chronic neurological decline and stress-provoked episodes of rapid, partially reversible decline. Cerebral white matter abnormalities are progressive, without improvement, and are therefore unlikely to be related to the episod...

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Bibliographic Details
Published in:Neuroradiology 2023-04, Vol.65 (4), p.855-863
Main Authors: Stellingwerff, Menno D., van de Wiel, Mark A., van der Knaap, Marjo S.
Format: Article
Language:English
Subjects:
Abnormalities
Adolescent
Adult
Basal ganglia
Brain stem
Caudate nucleus
Child
Child, Preschool
Clinical trials
Disease
Female
Ganglia
Globus pallidus
Humans
Imaging
Infant
Infant, Newborn
Leukodystrophy
Leukoencephalopathies - diagnostic imaging
Leukoencephalopathies - pathology
Magnetic Resonance Imaging
Male
Medical records
Medicine
Medicine & Public Health
Medulla oblongata
Mesencephalon
Middle Aged
Neurology
Neuroradiology
Neurosciences
Neurosurgery
Paediatric Neuroradiology
Pons
Putamen
Questionnaires
Radiology
Substantia alba
Thalamus
White Matter - diagnostic imaging
White Matter - pathology
Young Adult
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Description
Summary:Purpose Patients with vanishing white matter (VWM) experience unremitting chronic neurological decline and stress-provoked episodes of rapid, partially reversible decline. Cerebral white matter abnormalities are progressive, without improvement, and are therefore unlikely to be related to the episodes. We determined which radiological findings are related to episodic decline. Methods MRI scans of VWM patients were retrospectively analyzed. Patients were grouped into A (never episodes) and B (episodes). Signal abnormalities outside the cerebral white matter were rated as absent, mild, or severe. A sum score was developed with abnormalities only seen in group B. The temporal relationship between signal abnormalities and episodes was determined by subdividing scans into those made before, less than 3 months after, and more than 3 months after onset of an episode. Results Five hundred forty-three examinations of 298 patients were analyzed. Mild and severe signal abnormalities in the caudate nucleus, putamen, globus pallidus, thalamus, midbrain, medulla oblongata, and severe signal abnormalities in the pons were only seen in group B. The sum score, constructed with these abnormalities, depended on the timing of the scan ( χ 2 (2, 400) = 22.8; p 
ISSN:0028-3940
1432-1920
DOI:10.1007/s00234-022-03097-3