" /> Myelinoclastic diffuse sclerosis - CISMeF





Preferred Label : Myelinoclastic diffuse sclerosis;

ICD-11 definition : This is a demyelinating disorder characterized by inflammation and a large lesion, or lesions, of myelin destruction. Diffuse sclerosis is a rare disorder characterised by an inflammatory white matter plaque of demyelination, which often mimics intracranial neoplasm or abscess. It usually affects children between 5 and 14 years old . Characterised by a single or two symmetrically arranged lesions measuring at least 2 x 3cm with involvement of the centrum semiovale in the setting of symptoms unusual for multiple sclerosis.;

ICD-11 synonym : myelinoclastic diffuse sclerosis; Encephalitis periaxialis diffusa; Schilder diffuse myelinoclastic sclerosis; Schilder disease;

ICD-11 inclusion : centrolobar progressive encephalopathy;

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This is a demyelinating disorder characterized by inflammation and a large lesion, or lesions, of myelin destruction. Diffuse sclerosis is a rare disorder characterised by an inflammatory white matter plaque of demyelination, which often mimics intracranial neoplasm or abscess. It usually affects children between 5 and 14 years old . Characterised by a single or two symmetrically arranged lesions measuring at least 2 x 3cm with involvement of the centrum semiovale in the setting of symptoms unusual for multiple sclerosis.

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27/07/2025


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