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Schilder's disease: case study with serial neuroimaging.

Semra Kurul1, Handan Cakmakçi, Eray Dirik

  • 1Department of Pediatric Neurology, Dokuz Eylül University, Faculty of Medicine, Izmir, Turkey. skurul@hotmail.com

Journal of Child Neurology
|March 29, 2003
PubMed
Summary

Schilder's disease, a rare demyelinating condition, can mimic brain abscesses in children. Prompt diagnosis and corticosteroid treatment led to a full recovery in a 9-year-old girl.

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Area of Science:

  • Neuroscience
  • Pediatric Neurology
  • Demyelinating Diseases

Background:

  • Schilder's myelinoclastic diffuse sclerosis is a rare, sporadic demyelinating disease primarily affecting children aged 5-14.
  • It frequently presents with symptoms mimicking intracranial neoplasms or abscesses, posing diagnostic challenges.

Observation:

  • A 9-year-old girl presented with left hemiparesis, and imaging revealed large subcortical white matter lesions resembling an abscess.
  • Cerebrospinal fluid analysis showed positive oligoclonal bands, leading to a suspicion of acute disseminated encephalomyelitis.
  • Serial MRI demonstrated lesion regression but also new corpus callosum involvement, followed by optic nerve lesion and visual loss.

Findings:

  • The patient exhibited a significant clinical response to corticosteroid therapy.

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  • Subsequent MRI scans confirmed the regression of both parenchymal and optic nerve lesions.
  • At 24 months, the patient remained asymptomatic with no neurological deficits.

    • Implications:

    • This case highlights the importance of considering Schilder's disease in the differential diagnosis of pediatric brain lesions that mimic abscesses.
    • Early diagnosis and prompt corticosteroid treatment can lead to favorable outcomes and prevent long-term sequelae.
    • The study underscores the variable presentation and potential for recovery in Schilder's disease with appropriate management.