Document Detail

Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature.
MedLine Citation:
PMID:  7822732     Owner:  NLM     Status:  MEDLINE    
The clinical, neuroimaging, and neuropathologic features of Schilder's disease in a 17-year-old girl are presented and compared to 11 well-documented cases reported since 1912. The evolution of knowledge about Schilder's disease and the confusion in nomenclature are reviewed. Signs and symptoms in this case and others reported in the literature are nonspecific and may mimic mass lesions. Neuroimaging studies also may mimic brain tumor or abscess; however, the absence of significant edema, the irregular and incomplete ring enhancement, the discrepancy between size of the lesions and the associated mass effect, and the absence of other lesions elsewhere in the brain may help differentiate Schilder's disease from neoplasm, infection, and other demyelinating lesions. Although frozen sections of these lesions are often interpreted as astrocytoma, the inflammatory, primarily histiocytic, nature of Schilder's disease is more easily recognized in paraffin-embedded material. Unique features of this case include multiple unilateral lesions and the cyst-like degeneration present in both lesions. Multiple lesions in Schilder's disease are characteristically bilateral. The examination of aspirated fluid is the first such report in Schilder's disease. The limitation of multiple lesions in our case to one hemisphere calls for reexamination of the restrictive 1985 criteria of Poser for the diagnosis of Schilder's disease.
A K Afifi; W E Bell; A H Menezes; S A Moore
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Journal of child neurology     Volume:  9     ISSN:  0883-0738     ISO Abbreviation:  J. Child Neurol.     Publication Date:  1994 Oct 
Date Detail:
Created Date:  1995-02-10     Completed Date:  1995-02-10     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  8606714     Medline TA:  J Child Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  398-403     Citation Subset:  IM    
Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City.
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MeSH Terms
Astrocytoma / diagnosis,  pathology,  surgery
Brain Diseases / diagnosis
Brain Neoplasms / diagnosis,  pathology,  surgery
Cysts / diagnosis
Diagnosis, Differential
Diffuse Cerebral Sclerosis of Schilder / diagnosis*,  pathology,  surgery
Follow-Up Studies
Frontal Lobe / pathology
Magnetic Resonance Imaging
Parietal Lobe / pathology
Postoperative Complications / diagnosis
Tomography, X-Ray Computed

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