Magnetic Resonance of Myelination and Myelin Disorders

Knaap, Marjo S. van der; Valk, Jacob

doi:10.1007/3-540-27660-2

Cited by 290 publications

(247 citation statements)

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“…5,6 In some of our patients, as in another similar study, 11 foci of hypointensity were observed within the hyperintense white matter on T2-weighted images. This probably reflects the histopathologic "tigroid leukodystrophy," which corresponds to areas of preserved perivascular myelin within a deficient myelin area.…”

Section: Neuroimaging Diagnosis Of Cssupporting

confidence: 84%

“…In patients with classic CS, symmetric, attenuated, and homogeneous putaminal calcifications are usually predominant but can sometimes appear punctuate and slightly asymmetric. 11 In patients with severe CS II and COFS, diffuse cortical calcifications at the depths of the sulci are associated with the putaminal calcifications. This particular cortical topography has been reported previously in a neuropathologic study, along with calcifications of neighboring arteries.…”

Section: Neuroimaging Diagnosis Of Csmentioning

confidence: 99%

“…In congenital cytomegalovirus infections, brain calcifications typically show a periventricular subependymal distribution and may be associated with cortical gyral anomalies. 21 In Aicardi-Goutières syndrome, calcifications are more frequently small and punctuate in the basal ganglia and the deep and periventricular white matter. 22 Other hypomyelinating disorders, in particular Pelizaeus-Merzbacher disease, may show similar MR imaging aspects in the early stages.…”

Section: Neuroimaging Diagnosis Of Csmentioning

confidence: 99%

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Neuroimaging In Cockayne Syndrome

Koob¹,

Laugel²,

Durand³

et al. 2010

AJNR Am J Neuroradiol

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SUMMARY:CS is an autosomal recessive multisystem disorder, which is mainly characterized by neurologic and sensory impairment, cachectic dwarfism, and photosensitivity. We describe the neuroimaging features (MR imaging, 1 H-MR spectroscopy, and CT) in the various clinical subtypes of CS from a cohort of genetically and biochemically proved cases. Hypomyelination, calcifications, and brain atrophy were the main imaging features. Calcifications were typically found in the putamen and less often in the cortex and dentate nuclei. Severe progressive atrophy was seen in the supratentorial white matter, the cerebellum, the corpus callosum, and the brain stem. Patients with early-onset disease displayed more severe hypomyelination and prominent calcifications in the sulcal depth of the cerebral cortex, but atrophy was less severe in late-onset patients. On proton MR spectroscopy, lactate was detected and Cho and NAA values were decreased. These combined neuroradiologic findings can help in the differential diagnosis of CS, distinguishing it from other leukoencephalopathies and/or cerebral calcifications in childhood., ABBREVIATIONS: Cho ϭ choline; Cr ϭ creatine; CS ϭ Cockayne syndrome; COFS ϭ cerebro-oculofacio-skeletal syndrome; FLAIR ϭ fluid-attenuated inversion recovery; NAA ϭ N-acetylaspartate

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Section: Neuroimaging Diagnosis Of Cssupporting

confidence: 84%

Section: Neuroimaging Diagnosis Of Csmentioning

confidence: 99%

Section: Neuroimaging Diagnosis Of Csmentioning

confidence: 99%

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Neuroimaging In Cockayne Syndrome

Koob¹,

Laugel²,

Durand³

et al. 2010

AJNR Am J Neuroradiol

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“…The characteristic periventricular rim of low signal intensity on T2-weighted and high signal intensity on T1-weighted images has been reported to be related to an extreme attenuation of Rosenthal fibers in the periventricular white matter. 2,7 The neonatal MR imaging examinations performed in our patient fulfilled all the diagnostic MR imaging criteria.…”

Section: Discussionmentioning

confidence: 85%

“…2 On histopathologic study, astrocytes containing Rosenthal fibers are seen to surround and disrupt the ependyma of the aqueduct, resulting in obstructive hydrocephalus. The cerebellum, fornix, optic nerves, chiasm, and optic tracts may also contain these fibers, a nonspecific finding believed to represent a reaction to metabolic stress.…”

Section: Discussionmentioning

confidence: 99%