Schilder's disease

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Schilder's disease
ICD-9 341.1
OMIM 272100
DiseasesDB 11849
MeSH D002549

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]


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,[1][2] and for a hundred years the term was used to describe also the other white matter pathologies described by him[3]. 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.[4][5]

Natrual History, Complication and Prognosis

The prognosis of this disease is very variable and can take three different courses: a monofasic, not remitting; [6] [7], remitting;[8] [9] [10] and finally, progressive, with increase in deficits.[11]

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[12].



Symptoms are similar to those in multiple sclerosis and may include dementia, aphasia, seizures, personality changes, poor attention, tremors, instability, incontinence, muscle weakness, headache, vomiting, and vision and speech impairment.[13]

Diagnostic Criteria

The Poser criteria for diagnosis are:[14]

  • 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.


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


  1. Template:WhoNamedIt
  2. P. F. Schilder, Zur Kenntnis der sogenannten diffusen Sklerose (über Encephalitis periaxialis diffusa). Zeitschrift für die gesamte Neurologie und Psychiatrie, 1912, 10 Orig.: 1-60.
  3. Martin JJ, Guazzi GC (1991). "Schilder's diffuse sclerosis". Dev. Neurosci. 13 (4–5): 267–73. PMID 1817032.
  4. Garrido C, Levy-Gomes A, Teixeira J, Temudo T (2004). "[Schilder's disease: two new cases and a review of the literature]". Revista de neurologia (in Spanish). 39 (8): 734–8. PMID 15514902.
  5. Afifi AK, Bell WE, Menezes AH, Moore SA (1994). "Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature". J. Child Neurol. 9 (4): 398–403. PMID 7822732.
  6. Afifi AK, Bell WE, Menezes AH, Moore SA (1994). "Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature". J. Child Neurol. 9 (4): 398–403. PMID 7822732.
  7. Pretorius ML, Loock DB, Ravenscroft A, Schoeman JF (1998). "Demyelinating disease of Schilder type in three young South African children: dramatic response to corticosteroids". J. Child Neurol. 13 (5): 197–201. PMID 9620009.
  8. de Lacour A, Guisado F, Zambrano A, Argente J, Acosta J, Ramos C (1998). "[Pseudotumor forms of demyelinating diseases. Report of three cases and review of the literature]". Revista de neurologia (in Spanish; Castilian). 27 (160): 966–70. PMID 9951014.
  9. Leuzzi V, Lyon G, Cilio MR, Pedespan JM, Fontan D, Chateil JF, Vital A (1999). "Childhood demyelinating diseases with a prolonged remitting course and their relation to Schilder's disease: report of two cases". J. Neurol. Neurosurg. Psychiatr. 66 (3): 407–8. PMID 10084548.
  10. Brunot E, Marcus JC (1999). "Multiple sclerosis presenting as a single mass lesion". Pediatr. Neurol. 20 (5): 383–6. PMID 10371386.
  11. Garell PC, Menezes AH, Baumbach G, Moore SA, Nelson G, Mathews K, Afifi AK (1998). "Presentation, management and follow-up of Schilder's disease". Pediatric neurosurgery. 29 (2): 86–91. PMID 9792962.
  12. Fontaine B (2001). "[Borderline forms of multiple sclerosis]". Rev. Neurol. (Paris) (in French). 157 (8-9 Pt 2): 929–34. PMID 11787357.
  13. NINDS Schilder's Disease Information Page [1]
  14. Poser CM, Goutières F, Carpentier MA, Aicardi J (1986). "Schilder's myelinoclastic diffuse sclerosis". Pediatrics. 77 (1): 107–12. PMID 3940347.

See also

Multiple sclerosis Microchapters


Patient Information


Historical Perspective




Differentiating Multiple sclerosis from other Diseases

Epidemiology and Demographics

Risk Factors


Natural History, Complications and Prognosis


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Template:Diseases of the nervous system Template:WH Template:WS