Section 29. Disorders of the Nervous System >
Part 7. Developmental Delay and Regression >
Chapter 576. LeukoencephalopathiesMark P. Gorman
Topics Discussed: hematopoietic stem cell transplantation; leukodystrophy; leukoencephalopathies; neurology.
Sections: X-Linked Adrenoleukodystrophy, Aicardi-Goutieres Syndrome, Alexander Disease, Canavan Disease, Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy, Globoid Cell Leukodystrophy (Krabbe Disease), Hypomyelination with Atrophy of the Basal Ganglia and Cerebellum, Hypomyelination and Congenital Cataract, Megalencephalic Leukoencephalopathy with Subcortical Cysts (MLC), Metachromatic Leukodystrophy (MLD), Pelizaeus-Merzbacher Disease, Vanishing White Matter Disease, 18q Deletion Syndrome, References.
Excerpt:"Leukoencephalopathies comprise a
clinical and radiographic heterogeneous group of disorders. All these
disorders share the common features of neurologic dysfunction and
preferential involvement of CNS white matter. Although white matter
can be affected by many different processes, the term leukodystrophy is
generally reserved for those with an identified or presumed genetic
basis that is associated with a loss of previously formed myelin.
Acquired causes of white matter dysfunction include infectious (such
as encephalitis), inflammatory (such as acute disseminated encephalomyelitis and
multiple sclerosis; see Chapter 556, nutritional (such as vitamin
B12 deficiency), and neoplastic (such as astrocytoma) etiologies.
When evaluating a patient with a suspected leukoencephalopathy, these
acquired disorders should be considered and excluded with specific
testing when clinically indicated. ..."
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