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Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids: novel imaging findings
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Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids: novel imaging findings
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Journal Article
Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids: novel imaging findings
2010
Overview
Introduction Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is a paediatric autosomal dominant dysmyelinating disease. 1 Rare adult onset cases manifest with personality change, cognitive decline and gait impairment. 1-3 Diagnosis is often made following brain biopsy with the presence of giant neuroaxonal swellings known as spheroids. 4 Characteristic brain MRI abnormalities of HDLS include decreased T1-weighted signal intensity of the frontoparietal white matter and corpus callosum. 2 T2-weighted images reveal patchy increased signal intensity in the subcortical white matter with corticospinal tract involvement. 1-4 To our knowledge, this is the first report of an abnormal apparent diffusion coefficient (ADC) map in HDLS. The brain biopsy specimen was significant for areas of demyelination, axonal dropout and unpigmented activated macrophages, and the presence of large neuroaxonal spheroids ( figure 1F , arrows, haematoxylin-eosin stain) diagnostic of HDLS.
Publisher
BMJ Publishing Group Ltd,BMJ Publishing Group,BMJ Publishing Group LTD
