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Published on: March 24, 2023
Biopsy-Supported Tumefactive Demyelination of the Central Nervous System in Children
Seema Bangalore Hanumanthe1, Carla Francisco2,3, Janace Hart2
1Department of Neurology, University of California, San Francisco, San Francisco, CA, USA seem.bh@gmail.com.
Insights
Pediatric tumefactive demyelination in children is difficult to diagnose and treat. Cyclophosphamide showed significant improvement in cases unresponsive to other therapies, with many later meeting multiple sclerosis criteria.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Pediatric tumefactive lesions present diagnostic and therapeutic challenges.
- Central nervous system tumefactive demyelination requires careful evaluation in children.
Purpose of the Study:
- To describe clinical and radiological findings in children with central nervous system tumefactive demyelination.
- To evaluate treatment responses and long-term outcomes, including conversion to multiple sclerosis.
Main Methods:
- Retrospective review of 11 children with biopsy-proven central nervous system tumefactive demyelination.
- Analysis of clinical presentation, radiological data, cerebrospinal fluid oligoclonal bands, and treatment outcomes.
- Assessment for conversion to multiple sclerosis using the 2010 McDonald criteria.
Main Results:
- Diagnosis was aided by clinical, radiological, and biopsy data.
- Six of 11 patients showed poor response to initial treatments (glucocorticosteroids, IVIg, plasmapheresis) but improved with cyclophosphamide.
- Eight of nine patients received multiple sclerosis preventative therapy; five relapsed during treatment.
- Seven patients met 2010 McDonald criteria for multiple sclerosis at follow-up.
Conclusions:
- Central nervous system tumefactive demyelination in children poses diagnostic challenges.
- Cyclophosphamide can be effective in refractory cases.
- A significant proportion of pediatric tumefactive demyelination cases may evolve into multiple sclerosis.
Abstract:
Pediatric tumefactive lesions remains challenging to clinicians in terms of diagnosis and treatment. The authors describe 11 children with biopsy-proven central nervous system tumefactive demyelination. The mean age of onset was 11 years. Clinical and radiological data coupled with biopsy aided in the diagnosis of tumefactive demyelination. Of the 6 cases in which oligoclonal band data were available, only 3 showed oligoclonal band in the cerebrospinal fluid. Due to poor recovery despite treatment with high-dose glucocorticosteroids, intravenous immunoglobulin, and/or plasmapheresis, 6 cases went on to receive cyclophosphamide with marked improvement. Long-term data were available on 9 cases. Eight of 9 cases were started on preventative multiple sclerosis therapy after initial presentation; 1 is pending discussion with family. Five of the 8 cases had clinical relapse during treatment. Seven cases met 2010 McDonald criteria for multiple sclerosis at follow-up, (1 developed secondary progressive multiple sclerosis), and 2 cases remained as clinically isolated syndrome on treatment.
