Journal article
Myelinoclastic Diffuse Sclerosis (Schilder's Disease): Report of a Case and Review of the Literature
Journal of Child Neurology, Vol.9(4), pp.398-403
10/1994
DOI: 10.1177/088307389400900412
PMID: 7822732
Abstract
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. (J Child Neurol 1994;9:398-403).
Details
- Title: Subtitle
- Myelinoclastic Diffuse Sclerosis (Schilder's Disease): Report of a Case and Review of the Literature
- Creators
- Adel K Afifi - Department of Pediatrics and Neurology, University of Iowa Hospitals and Clinics, Iowa City, IAWilliam E Bell - Department of Pediatrics and Neurology, University of Iowa Hospitals and Clinics, Iowa City, IA, Department of Anatomy, University of Iowa Hsopitals and Clinics, Iowa City, IAArnold H Menezes - Department of Surgery, University of Iowa Hospital and Clinics, Iowa, City, IASteven A Moore - Department of Pathology University of Iowa Hospitals and Clinics, Iowa City, IA
- Resource Type
- Journal article
- Publication Details
- Journal of Child Neurology, Vol.9(4), pp.398-403
- Publisher
- Sage Publications; Thousand Oaks, CA
- DOI
- 10.1177/088307389400900412
- PMID
- 7822732
- ISSN
- 0883-0738
- eISSN
- 1708-8283
- Language
- English
- Date published
- 10/1994
- Academic Unit
- Neurology; Stead Family Department of Pediatrics; Pathology; Neurosurgery; Otolaryngology
- Record Identifier
- 9984040239902771
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