Clinical characteristics of children with cerebral white matter abnormalities

The rapidly expanding use of magnetic resonance imaging (MRI) in children with neurological impairments of unknown aetiology has revealed a large number of children with abnormalities of the cerebral white matter, some with leukodystrophy-like white matter abnormalities on MRI, but non-progressive i...

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Bibliographic Details
Published in:	European journal of paediatric neurology Vol. 4; no. 1; pp. 17 - 26
Main Authors:	Kristjnsdóttir, R, Uvebrant, P, Wiklund, L-M
Format:	Journal Article
Language:	English
Published:	England Elsevier Ltd 01-01-2000
Subjects:	Adolescent Brain Brain - pathology Brain Damage Brain Damage, Chronic - diagnosis Brain Damage, Chronic - genetics Brain Edema Brain Edema - diagnosis Brain Edema - genetics Cerebral Child Child, Preschool Chronic Consanguinity diagnosis Diagnosis, Differential Differential Disease Progression Dominance Dominance, Cerebral - physiology Female Follow-Up Studies genetics Hereditary Central Nervous System Demyelinating Diseases Hereditary Central Nervous System Demyelinating Diseases - diagnosis Hereditary Central Nervous System Demyelinating Diseases - genetics Humans Infant Infant, Newborn Intellectual Disability - diagnosis Intellectual Disability - genetics leukodystropy,leukoencephalopathy,metabolism,white matter disorders,Neuroimpairments Magnetic Resonance Imaging Male MEDICAL AND HEALTH SCIENCES MEDICIN OCH HÄLSOVETENSKAP Mental Retardation Neurologic Examination Newborn pathology physiology Preschool leukodystropy,leukoencephalopathy,metabolism,white matter disorders,Neuroimpairments
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Summary:	The rapidly expanding use of magnetic resonance imaging (MRI) in children with neurological impairments of unknown aetiology has revealed a large number of children with abnormalities of the cerebral white matter, some with leukodystrophy-like white matter abnormalities on MRI, but non-progressive in clinical presentation and course. The aim of this study was to investigate the clinical and neuroradiological characteristics of 26 children with white matter abnormalities of unknown origin and to find diagnostic clues or indicators of progressive versus non-progressive disease. The typical child with white matter abnormalities was characterized by onset of symptoms within the first year of life, most often presenting as general developmental delay and hypotonia. Later-appearing signs were spasticity and ataxia and as a rule severe learning and motor disabilities. Serious ophthalmological signs were frequently seen. Perinatal adverse events were rare, infectious aetiologies not indicated but prenatal stigmata relatively common. The clinical course was progressive in 11 children and non-progressive in 15. Late onset presentation was associated with a progressive course whereas prenatal stigmata and asymmetrical white matter lesions only were found in children with a non-progressive disorder. The MRI showed three main patterns: a) a generalized increase of the T2 signal of the white matter in 12 children, b) a bilateral, symmetric but not generalized abnormality in nine and c) asymmetric, focal or multifocal pathology in five. Useful information as to clinical entities and course was obtained from the combined clinical and radiological assessment. A precise nosological diagnosis could be made in six cases. The study showed that white matter abnormalities in children constitute a heterogeneous group of rare and `anonymous' conditions, motivating collaborative studies for further clarification of background and management.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1090-3798 1532-2130
DOI:	10.1053/ejpn.1999.0255