Myelinoclastic Diffuse Sclerosis Presenting as a Mass Lesion in a Child with Turner’s Syndrome

Stachniak, Joseph; Mickle, J. Parker; Ellis, Thomas L.; Quisling, Ronald G.; Rojiani, Amyn M.

doi:10.1159/000120912

Cited by 8 publications

(3 citation statements)

References 7 publications

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“…Other cases have been described as cases of acute disseminated encephalomyelitis (ADEM) 9,12,16 . However, these lesions, as well as those in the present cases, clearly did not correspond to the de‐scription of inflammatory myelinoclastic diffuse sclerosis, 23 or myelinoclastic diffuse sclerosis 24 . The biological and clinical behavior of these lesions including those of our present cases, appears to be distinct from the usually progressive childhood diffuse sclerosis of Schilder, or the acutely progressive monophasic malignant form of MS known as Marburg's disease 25 .…”

Section: Discussioncontrasting

confidence: 71%

Acute focal demyelinating disease simulating brain tumors: Histopathologic guidelines for an accurate diagnosis

Sugita¹,

Terasaki

Shigemori

et al. 2001

Neuropathology

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The object of the present study was to determine the histopathological guidelines for accurate diagnosis of cases of acute focal demyelinating disease that simulates brain tumors. The surgical pathology of three such cases is assessed. Histopathological keys to the diagnosis of such cases are as follows. First, a pattern of sheets of atypical gemistocytic astrocytes in the white matter that show well‐formed processes and that are adequately distanced from each other argues against a diagnosis of neoplasm. Second, uniform distribution of foamy macrophages aligned along axons, with occasional focal collections surrounding blood vessels and in the absence of any associated coagulative necrosis argues against the presence of a tumor. Third, perivascular chronic inflammatory infiltration, especially a mixture of lymphocytes and macrophages, favors the diagnosis of demyelination plaque. In such cases the lymphocytes will be predominantly T cells. Fourth, pleomorphic astrocytic proliferation with a lack of vascular endothelial proliferation should raise the suspicion that the lesion may not be a brain tumor. These diagnostic keys should be followed when diagnosing cases that are suspected to be demyelination processes rather than brain tumors. The presence of demyelination plaque should then be confirmed by imaging modalities such as staining with myelin‐and axon‐specific stains.

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Section: Discussioncontrasting

confidence: 71%

Acute focal demyelinating disease simulating brain tumors: Histopathologic guidelines for an accurate diagnosis

Sugita¹,

Terasaki

Shigemori

et al. 2001

Neuropathology

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“…Myelinoclastic diffuse sclerosis (MDS), also known as inflammatory diffuse sclerosis and Schilder's disease, is a rare demyelination disorder of the central nervous system that usually presents as an intracranial mass lesion. 7,12,17,18) MDS is more common in children than adults. 1,4,5,[8][9][10] MDS has variable presentations and imaging findings, and can involve an area large enough to simulate the radiological appearance and clinical course of a brain tumor.…”

Section: Introductionmentioning

confidence: 99%

Tumefactive Myelinoclastic Diffuse Sclerosis-Case Report-

Obara

Takeshima

Awa

et al. 2003

Neurol. Med. Chir.(Tokyo)

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A 6-year-old boy presented with mental disturbance and progressive left hemiparesis. Magnetic resonance imaging demonstrated large intracranial mass lesions with ring-like enhancement. His neurological condition deteriorated rapidly. Open biopsy via craniotomy was performed under the suspicion of tumor. Histological examination showed massive demyelination and axon preservation, but no tumor cells. The diagnosis was myelinoclastic diffuse sclerosis (MDS). He was treated with high-dose methylprednisolone and improved dramatically. MDS is a rare demyelinating disorder of the central nervous system that affects mainly children and may mimic a brain tumor. MDS must be included in the differential diagnosis in young patients with a brain tumor with atypical radiological appearance.

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“…It generally presents as an intracranial mass lesion which is confirmed by neuroimaging. It prompts the differential diagnosis of tumor or abscess, and rarely demyelination [2]. Since its original description by Paul Schilder [3] in 1912, there have been sporadic case reports and reviews in the literature.…”

Section: Introductionmentioning

confidence: 99%

Presentation, Management and Follow-Up of Schilder’s Disease

Garell

Menezes²,

Baumbach³

et al. 1998

Pediatr Neurosurg

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Schilder’s diffuse myelinoclastic sclerosis is a rare demyelinating disease which often mimics intracranial neoplasm or abscess. We have treated 3 patients with this disorder in the past 5 years and followed their postoperative course. Certain distinct features of this disease will allow neurosurgeons to preoperatively entertain this diagnosis. We discuss postoperative treatment and propose a new hypothesis regarding the variable prognoses of this disorder. Schilder’s disease constitutes an important diagnosis for any neurosurgeon to be aware of (especially those treating the pediatric age group) which has not received adequate coverage in the neurosurgical literature.

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Myelinoclastic Diffuse Sclerosis Presenting as a Mass Lesion in a Child with Turner’s Syndrome

Cited by 8 publications

References 7 publications

Acute focal demyelinating disease simulating brain tumors: Histopathologic guidelines for an accurate diagnosis

Acute focal demyelinating disease simulating brain tumors: Histopathologic guidelines for an accurate diagnosis

Tumefactive Myelinoclastic Diffuse Sclerosis-Case Report-

Presentation, Management and Follow-Up of Schilder’s Disease

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