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Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement
Fiona Costello1, Andrew G Lee, Adel K Afifi
1Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242, USA.
Abstract:
A 17-year-old male presented with chronic diplopia and generalized motor weakness. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and acute disseminated encephalomyelitis in childhood. Cranial magnetic resonance imaging (MRI) revealed a rarely reported finding of thickening and enhancement of multiple cranial nerves. Nerve conduction studies and electromyography showed peripheral nerve demyelination with axonal involvement. There was improvement in the clinical examination, MRI, and electrophysiologic studies after combined corticosteroid and plasma exchange therapy. We review the clinical presentation, course, and response to therapy among children with chronic inflammatory demyelinating polyradiculoneuropathy, with specific emphasis on the frequency and pattern of cranial nerve involvement.
Insights
This study highlights a rare case of cranial nerve involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a pediatric patient. Prompt treatment with corticosteroids and plasma exchange led to significant clinical and imaging improvements.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimmunology
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Cranial nerve involvement in pediatric CIDP is infrequently reported, presenting diagnostic challenges.
Observation:
- A 17-year-old male with a history of demyelinating disorders presented with chronic diplopia and motor weakness.
- Cranial MRI revealed rare thickening and enhancement of multiple cranial nerves.
- Electrophysiologic studies confirmed peripheral demyelination with axonal damage.
Findings:
- The patient experienced significant improvement in clinical symptoms, MRI findings, and electrophysiologic parameters after receiving combined corticosteroid and plasma exchange therapy.
- This case underscores the potential for cranial nerve abnormalities in pediatric CIDP.
Implications:
- Early recognition and aggressive treatment of cranial nerve involvement in pediatric CIDP can lead to favorable outcomes.
- Further research is warranted to understand the frequency and specific patterns of cranial nerve involvement in pediatric CIDP.