Related Experiment Videos
Focal upper limb neuropathy in a child
D G McDonald1, M A Farrell, J B McMenamin
1Department of Neurology, Our Lady's Hospital for Sick Children, Dublin, Ireland.
Summary
A child initially presented with focal brachial plexitis, later developing chronic inflammatory demyelinating polyneuropathy. Intravenous immunoglobulin treatment led to sustained functional recovery, highlighting a unique disease progression.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Clinical Neuroscience
Background:
- Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Pediatric CIDP cases often present with diffuse, symmetrical weakness, unlike the focal onset described here.
- Understanding atypical presentations is crucial for timely diagnosis and effective management in children.
Observation:
- A 9-year-old girl experienced initial right upper limb weakness, diagnosed as atypical brachial plexitis.
- She later developed progressive, diffuse muscle weakness affecting both lower limbs and the left upper limb.
- No improvement was noted in the initial right upper limb deficit over 3.5 years.
Findings:
- Clinical, neurophysiological, and pathological findings confirmed a diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).
- The patient demonstrated significant functional recovery after a single course of intravenous immunoglobulin (IVIg).
- Long-term follow-up exceeding 6 years showed sustained normal function, indicating long-term IVIg efficacy.
Implications:
- This case describes a previously unreported focal neuropathy preceding diffuse CIDP in a pediatric patient.
- It underscores the importance of considering CIDP in children with seemingly focal neurological deficits that progress.
- The findings suggest that IVIg may be a highly effective long-term treatment for this rare pediatric CIDP presentation.