Schilder's disease; myelinoclastic diffuse sclerosis; balo concentric sclerosis

Introduction

Diffuse sclerosis or encephalitis. A variant of multiple sclerosis.

Epidemiology

• sporadic disorder
• affects predominantly children & young adults
• more common in Chinese & Filipino population than Caucasians
• 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
  • lesions can be distinguished by alternating hypodense & hyperdense layers

Management

• treat as multiple sclerosis
  • corticosteroids
  • interferon-beta
  • immunosuppressive therapy
• prognosis
  • unpredictable course
  • may be slowly progressive
  • remissions may occur
  • may be fatal within a few years of onset

More general terms

• multiple sclerosis (MS); includes clinically isolated syndrome

Patient information

Schilder's disease patient information