Search
Schilder's disease; myelinoclastic diffuse sclerosis; balo concentric sclerosis
Diffuse sclerosis or encephalitis. A variant of multiple sclerosis.
Epidemiology:
1) sporadic disorder
2) affects predominantly children & young adults
3) more common in Chinese & Filipino population than Caucasians
4) rare
Pathology:
- large asymmetric area of myelin destruction
- may involve an entire cerebral hemisphere
- generally extends across the corpus callosum
- demyelinated tissues form concentric layers
- distal oligodendrogliopathy
Clinical manifestations:
- similar to multiple sclerosis
- primary progressive or relapsing-remitting
- progressive dementia
- visual impairment
- deafness
- aphasia
- pseudobulbar palsy
- hemiplegia or quadriplegia
- seizures
- personality changes
- poor attention
- tremors
- balance instability
- incontinence
- muscle weakness
- headache
- vomiting
Radiology:
- MRI neuroimaging [3]
- lesions can be distinguished by alternating hypodense & hyperdense layers
Management:
1) treat as multiple sclerosis
a) corticosteroids
b) interferon-beta
c) immunosuppressive therapy
2) prognosis
a) unpredictable course
b) may be slowly progressive
c) remissions may occur
b) may be fatal within a few years of onset
General
multiple sclerosis (MS); includes clinically isolated syndrome
References
- Saunders Manual of Medical Practice, Rakel (ed), WB Saunders,
Philadelphia, 1996, pg 76
- Gaillard F and Weerakkody Y
Radiopaedia.org: Balo concentric sclerosis
http://radiopaedia.org/articles/balo-concentric-sclerosis-2
- Wengert O and Siebert E
Balo's Concentric Sclerosis
N Engl J Med 2011; 365:742August 25, 2011
http://www.nejm.org/doi/full/10.1056/NEJMicm1008401
- Wikipedia: Balo concentric sclerosis
http://en.wikipedia.org/wiki/Balo_concentric_sclerosis
- NINDS Schilder's Disease Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Schilders-Disease-Information-Page