Diffuse myelinoclastic sclerosis

Diffuse myelinoclastic sclerosis
Other names	Dms
Specialty	Neurology

Diffuse myelinoclastic sclerosis, sometimes referred to as Schilder's disease, is a very infrequent neurodegenerative disease that presents clinically as pseudotumoural demyelinating lesions, making its diagnosis difficult. It usually begins in childhood, affecting children between 5 and 14 years old,^[1][2] but cases in adults are also possible.^[3]

This disease is considered one of the

The Poser criteria for diagnosis are:[7]

• One or two roughly symmetrical large plaques. Plaques are greater than 2 cm diameter.
• No other lesions are present and there are no abnormalities of the peripheral nervous system.
• Results of adrenal function studies and serum very long chain fatty acids are normal.
• Pathological analysis is consistent with subacute or chronic myelinoclastic diffuse sclerosis.

Neuropathological examination

The typical demyelinating plaques in Schilder's sclerosis are usually found bilaterally in the centrum semiovale. Both hemispheres are almost completely occupied by large, well defined lesions. Although plaques of this kind are largely prevalent in Schilder's sclerosis, smaller lesions can also be observed.^{[citation needed]}

Immunology

It has been reported that DMS cases show no oligoclonal bands, being therefore distinct from standard MS.^[8]

Treatments

Management:

The prognosis of this disease is very variable and can take three different courses: a monophasic, not remitting;[2]^[9] remitting;^[10][11][12] and finally, progressive, with increase in deficits.^[13]

History

It was first described by Paul Ferdinand Schilder in 1912,^[14][15] and for nearly one hundred years the term "Schilder disease" was used to describe it, but the same name was also used for some other white matter pathologies described by him.^[16] In 1986 Poser tried to restrict the use of Schilder's disease name to the disease described here, but this name has still remained ambiguous.^{[citation needed]}

The name comes from a traditional classification of demyelinating diseases in two groups: demyelinating myelinoclastic diseases and demyelinating leukodystrophic diseases. In the first group, a normal and healthy myelin is destroyed by a toxic, chemical, or autoimmune substance. In the second group, myelin is abnormal and degenerates.^[17] The second group was denominated dysmyelinating diseases by Poser.^[18]

References