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Cranial polyneuropathy with elevated serum antiganglioside antibody
K Matsubara1, H Nigami, H Harigaya
1Department of Pediatrics, Nishi-Kobe Medical Center, Kobe, Japan.
Pediatric Neurology
|February 1, 1997
Summary
This study details a rare pediatric case of cranial polyneuropathy associated with antiganglioside antibodies, potentially linked to Campylobacter jejuni infection. The patient experienced cranial nerve palsies that resolved over time.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Miller Fisher syndrome (MFS) and Bickerstaff's brainstem encephalitis (BBE) are rare neurological disorders.
- Antiganglioside antibodies are implicated in various autoimmune neuropathies.
- Campylobacter jejuni infection is a known trigger for Guillain-Barré syndrome and its variants.
Observation:
- A 6-year-old girl presented with cranial polyneuropathy, including palsies of the 3rd, 6th, 7th, and 12th cranial nerves, following herpetic vesicles.
- No limb weakness, ataxia, or areflexia was noted; cerebrospinal fluid and brain MRI were normal.
- Elevated serum antibodies to gangliosides GQ1b and GT1b, and Campylobacter jejuni were detected.
Findings:
- The patient's presentation suggests a variant form of Miller Fisher syndrome or Bickerstaff's brainstem encephalitis.
- The neurological deficits were associated with specific antiganglioside antibodies (GQ1b, GT1b) and Campylobacter jejuni infection.
- Treatment with methylprednisolone and acyclovir showed minimal initial effect, but spontaneous resolution of cranial nerve palsies occurred within 4 months.
Implications:
- This case highlights a potential association between cranial polyneuropathy, antiganglioside antibodies, and asymptomatic Campylobacter jejuni infection in a pediatric patient.
- It expands the spectrum of clinical presentations for anti-ganglioside antibody-associated neurological disorders in children.
- The findings contribute to understanding the pathogenesis of rare neurological syndromes and may inform diagnostic and therapeutic approaches.