Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature

  title={Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature},
  author={Kadir Kotil and Murat Kalaycı and Tankut K{\"o}seoḡlu and Armağan Tuğrul},
  journal={British Journal of Neurosurgery},
  pages={516 - 519}
Schilder's myelinoclastic diffuse sclerosis is a rare demyelinating disease, which often mimics intracranial neoplasm or abscess. This disease is of importance to the neurosurgeon in that the clinical manifestations and course may closely simulate brain tumour, including the development of papilloedema and other signs of increased intracranial pressure. We have treated a 29-year-old right-handed young man presenting with left hemiplegia, aphasia and behavioural changes. Magnetic resonance… 

Tumefactive myelinoclastic diffuse sclerosis--case report.

A 6-year-old boy presented with mental disturbance and progressive left hemiparesis and was treated with high-dose methylprednisolone and improved dramatically and must be included in the differential diagnosis in young patients with a brain tumor with atypical radiological appearance.

Schilder’s disease: non-invasive diagnosis?

A 29-year-old female patient is presented, where a careful evaluation of both the clinical and radiological features, which might have been at a first glance misleadingly suggestive for a brain tumour, allowed non-invasive diagnosis of Schilder’s disease.

Myelinoclastic diffuse sclerosis (Schilder’s disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures

In the majority of patients diagnosed with MDS, CSF features differ significantly from those typically found in MS and are more similar to those previously reported in patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (IGG)-positive encephalomyelitis, aquaporin-4-IgG-positive neuromyelitic spectrum disorders or Baló’s concentric sclerosis.

Tumefactive demyelinating lesions

No case strongly suggestive of variants or related diseases, such as Schilder's disease or Balo's concentric sclerosis, were found and there was one case suggestive of acute disseminated encephalomyelitis.

A Pathology-proven Case of Schilder's Disease

This report reports a 66-year-old woman with progressive motor weakness and diffuse white matter degeneration on MRI, which satisfies the Poser’s restrictive criteria of MDS.

Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis

Although lesion size >5 cm was associated with a slightly higher EDSS at last follow-up, long-term prognosis in patients with disease duration >10 years was better compared with a population-based multiple sclerosis cohort matched for disease duration (EDSS 3.5; P < 0.001).

Variants of multiple sclerosis.


The recently recommended McDonald criteria for the diagnosis of MS are reviewed, which delineate the specific use of magnetic resonance imaging findings, and Variants of MS, or closely related conditions, including Balós concentric sclerosis, Marburg variant, Devic's neuromyelitis optica, and acute disseminated encephalomyelitas are discussed.

Acquired Demyelinating Disorders of the CNS in Children

This chapter will cover the aetiologies, consensus definitions, clinical presentation, neuroimaging, evolution and therapeutic advances in acquired demyelinating disorders in children, and the pivotal role of neuro-imaging in unraveling the pathology, aetiology and diagnosis of these disorders.

Uncertainty of leukoencephalopathies: a case report

Adult-onset leukoencephalopathy can become a diagnostic challenge and certain approaches need to be developed to explore the uncertainty of such conditions and to improve diagnostic yield.



Inflammatory myelinoclastic diffuse sclerosis (Schilder's disease): neuroradiologic findings.

Because of the rapid and dramatic fluctuations in the patient's functional status, it has been possible to obtain sequential CT and comparative MR scans that graph­ ically document the interplay of cavitary and diffuse white­ matter lesions, which has allowed cliniconeuroradiologic assessment of this rare but distinctive inflammatory myelino­ clastic condition.

Schilder's myelinoclastic diffuse sclerosis.

A case of Schilder's myelinoclastic diffuse sclerosis in a 14-year-old girl with sudden bilateral visual loss is reported, with almost complete visual recovery and shrinkage of the lesions.

Schilder's myelinoclastic diffuse sclerosis.

Schilder's myelinoclastic diffuse sclerosis, a variant of multiple sclerosis, is a very rare disease that occurs in children and adults of both sexes and appears to respond to vigorous treatment with corticosteroids and/or corticotropin.

Clinical recovery from Schilder disease

A 7-year-old girl presented with progressive dementia with a large, bilateral frontal lesion that crossed the corpus callosum, and the histologic diagnosis was Schilder disease.

Cranial MRI in acute disseminated encephalomyelitis

The clinical features of the case along with the significantly elevated CSF level of MBP-like material and the marked white matter abnormality on cranial MRI are consistent with ADEM.

The pathogenesis of acute viral encephalitis and postinfectious encephalomyelitis.

  • R. Johnson
  • Medicine, Biology
    The Journal of infectious diseases
  • 1987
In Japanese encephalitis there is direct invasion of the virus into the nervous system, selective infection and destruction of neurons, and evidence that both humoral and cellular immune responses attenuate the infection, while in measles encephalomyelitisthere is little evidence that the virus invades the nervousSystem.

Demyelinating disease.

  • K. Noad
  • Medicine
    The Medical journal of Australia
  • 1960