Schilder's disease

Overview
Schilder's disease or diffuse myelinoclastic sclerosis is a a very infrequent neurodegenerative disease that presents clinically as pseudotumoural demyelinating lesions, that make its diagnosis difficult. The disease presents with both adrenal atrophy and diffuse cerebral demyelination.

Historical Perspective
It was first described by Paul Ferdinand Schilder in 1912, and for a hundred years the term was used to describe also the other white matter pathologies described by him. In 1986 Poser restricted its use to the disease described here.

Epidemiology and Demographics
The disorder usually begins in childhood with an age of onset between 5 and 14 years old.

Natrual History, Complication and Prognosis
The prognosis of this disease is very variable and can take three different courses: a monofasic, not remitting; , remitting; and finally, progressive, with increase in deficits.

Disorders to Differentiate Shilder's Disease From
This disease is considered one of the borderline forms of multiple sclerosis because some authors consider them different diseases and others MS variants. Other diseases in this group are neuromyelitis optica (NMO), Balo concentric sclerosis and Marburg multiple sclerosis.

Symptoms
Symptoms are similar to those in multiple sclerosis and may include dementia, aphasia, seizures, personality changes, poor attention, tremors, balance instability, incontinence, muscle weakness, headache, vomiting, and vision and speech impairment.

Diagnostic Criteria
The Poser criteria for diagnosis are:
 * 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.

Treatment
Management Corticosteroids may be effective in some patients. Otherwise management in supportive and includes physiotherapy, occupational therapy and nutritional support in the later stages