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;
Origin ID : 816172167;
Automatic exact mappings (from CISMeF team)
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.
