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Results for “Diffuse Cerebral Sclerosis of Schilder”

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At least 19 recordsLinked to original sources
PubMed1997

Differentiation of dys- and demyelination using diffusional anisotropy.

We attempted differential diagnosis of dysmyelination and demyelination in childhood using magnetic resonance diffusion weighted imaging. Pelizaeus-Merzbacher disease, one of the dysmyelination disorders, demonstrated diffuse high intensity of the cerebral white matter on T2-weighted images, which demonstrated diffusional anisotropy on diffusion weighted images. On the other hand, high intensity lesions on T2-weighted images in Krabbe disease, one of the demyelination disorders, lost diffusional anisotropy. Another demyelination disorder, Alexander disease-related disorder, also lost its diffusional anisotropy. In contrast to relatively high signal of the lesions on diffusion-weighted images in Krabbe disease (high signal type), the lesions in Alexander disease-related disorder showed low signal on diffusion-weighted images (low signal type). These results suggest that diffusion-weighted images will be clinically useful to differentiate dysmyelination from demyelination; both of them demonstrate similar high intensity lesions of the white matter on T2-weighted images.

Adult↗
PubMedDate not supplied

Detection of acute cytotoxic changes in progressive neuronal degeneration of childhood with liver disease (Alpers-Huttenlocher syndrome) using diffusion-weighted MRI and MR spectroscopy.

Alpers-Huttenlocher syndrome (AHS) is a rare mitochondrial disorder of childhood onset that is characterized by progressive encephalopathy and hepatopathy. MRI studies are rare and have not added substantial information to the pathogenesis of the encephalopathy. Diffusion-weighted MRI (DWI) and MR spectroscopy (MRS) were used in a patient with AHS during acute clinical deterioration and after improvement. DWI detected signal hyperintensity in several brain areas not restricted to any vascular territory. MRS revealed an unequivocal lactate peak and a reduced N-acetyl-aspartate-creatinine (NAA/Crea) ratio. DWI signal hyperintensity was correlated with neurologic symptoms and decreased after clinical improvement. Potentially reversible neuronal cytotoxic edema resulting from acute impairment of mitochondrial function is strongly suggested to be an important pathogenetic mechanism in AHS encephalopathy.

Aspartic Acid↗
PubMed1950

[Schilder disease].

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Diffuse Cerebral Sclerosis of Schilder↗
PubMed2002

Decompressive aspiration in myelinoclastic diffuse sclerosis or Schilder disease. Case report.

This 9-year-old girl with rapidly progressive cerebral demyelinating disease presented with hemiplegia and intracranial hypertension. Brain images revealed four lesions with mass effect in the subcortical white matter of both hemispheres. Demyelination was found on pathological studies of these lesions. The patient experienced some recovery with corticosteroid treatment but improved completely with decompressive aspiration of the largest lesion.

Adrenal Cortex Hormones↗