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Schilder's disease: additional aspects and a therapeutic option
R J Konkol1, D Bousounis, K C Kuban
1Medical College of Wisconsin, Department of Neurology, Children's Hospital of Wisconsin, Milwaukee 53201.
Neuropediatrics
|August 1, 1987
Summary
This case study shows that ACTH and cyclophosphamide (cytoxin) effectively treated a rare form of Schilder's disease in an 8-year-old boy. The therapy led to significant clinical improvement and lesion shrinkage.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Immunology
Background:
- Schilder's disease, a rare demyelinating disorder, presents with extensive white matter lesions.
- Distinguishing Schilder's disease from other white matter pathologies like adrenoleukodystrophy is crucial for appropriate management.
Observation:
- An 8-year-old boy exhibited bifrontal and parietal white matter lesions with high intracranial pressure.
- Initial treatment with ACTH and cyclophosphamide (cytoxin) led to lesion shrinkage and clinical improvement.
Findings:
- A recurrence with a new frontal lesion and neurological deficits (hemiparesis, speech difficulty) was observed nine months later.
- The same immunosuppressive therapy again resulted in prompt CT scan improvement and clinical recovery.
- Investigations ruled out common markers for post-infectious encephalopathy and adrenoleukodystrophy, with CSF analysis showing no abnormalities.
Implications:
- This case highlights the potential efficacy of immunosuppressive therapy, specifically ACTH and cyclophosphamide, in managing the 1912 variety of Schilder's disease.
- The rapid clinical response to immunosuppression in this case, despite pathological similarities to multiple sclerosis, warrants further investigation into treatment strategies for this specific demyelinating condition.