Related Experiment Videos
Recurrent encephalomyelitis associated with incontinentia pigmenti
1Department of Pediatrics, Rhode Island Hospital, Providence.
Pediatric Neurology
|May 1, 1987
Summary
Recurrent encephalomyelitis, a rare neurological disorder, can occur in children with incontinentia pigmenti. This immune-mediated condition involves the central nervous system and may be linked to transient suppressor T cell loss.
Area of Science:
- Pediatric Neurology
- Immunology
- Dermatology
Background:
- Incontinentia pigmenti is a rare genetic disorder affecting skin, hair, nails, and teeth.
- Neurological complications, including encephalomyelitis, can occur in patients with incontinentia pigmenti.
Observation:
- A 4-year-old girl presented with progressive obtundation after an upper respiratory infection.
- Clinical and diagnostic findings suggested disseminated encephalomyelitis.
- Skin biopsy confirmed incontinentia pigmenti.
Findings:
- The patient experienced a similar episode of central nervous system deterioration at 6 months of age.
- Transient loss of suppressor T cells was noted during the encephalomyelitis episode.
- Recurrent encephalomyelitis is a recognized complication of incontinentia pigmenti.
Implications:
- The findings suggest that recurrent encephalomyelitis in incontinentia pigmenti is an immune-mediated process.
- Transient suppressor T cell depletion may play a role in the pathogenesis.
- This case highlights the importance of considering neurological evaluations in children with incontinentia pigmenti.