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Motor variant of chronic inflammatory demyelinating polyneuropathy in a child
Durriyah D Sinno1, Basil T Darras, Bassem I Yamout
1Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
Insights
This case study details a rare pediatric motor variant of chronic demyelinating inflammatory polyneuropathy in a 5-year-old girl. The patient showed improvement with immunotherapy, highlighting treatment responses in this uncommon condition.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Chronic demyelinating inflammatory polyneuropathy (CDIP) is rare in children.
- Pure motor and motor variant CDIP are exceptionally uncommon pediatric presentations.
Observation:
- A 5-year-old girl presented with 12 months of progressive motor weakness and no sensory deficits.
- The patient was diagnosed with a motor variant of CDIP associated with anti-ganglioside antibodies.
Findings:
- Initial partial response to intravenous immunoglobulin (IVIg) therapy.
- Sustained, albeit incomplete, improvement with chronic prednisone treatment.
Implications:
- This case expands the understanding of pediatric CDIP variants.
- It suggests potential therapeutic avenues for similar rare pediatric neuropathies.
- Highlights the importance of considering anti-ganglioside antibodies in pediatric motor neuropathies.
Abstract:
Only 2 cases of pure motor chronic demyelinating inflammatory polyneuropathy in the pediatric age group have been reported in the literature. We report on a motor variant of chronic demyelinating inflammatory polyneuropathy with anti-ganglioside antibodies, diagnosed in a 5-year-old girl who presented with progressive motor weakness over a period of 12 months with no sensory involvement. She initially responded partially to intravenous immunoglobulin therapy (1 gm/kg/month for 6 months), and then demonstrated sustained but incomplete improvement on chronic prednisone therapy (1-2 mg/kg/day), on which she has continued since 1 year and 4 months after her initial presentation 3 years ago.
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