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Sensorimotor neuropathy in hemophagocytosis syndrome.
L S Honig1, G J Snipes, H Vogel
1Department of Neurology, Stanford University School of Medicine, California.
Acta Neurologica Scandinavica
|October 1, 1991
Summary
Hemophagocytosis syndromes involve histiocyte proliferation and organ failure. This case reveals histiocyte infiltration in peripheral nerves, a novel finding potentially causing polyneuropathy in these rare disorders.
Area of Science:
- Neurology
- Pathology
- Hematology
Background:
- Hemophagocytosis syndromes (HPS) are rare, life-threatening conditions characterized by excessive histiocyte proliferation and multi-organ dysfunction.
- While HPS affects multiple organs, its impact on the peripheral nervous system is not well-established.
Observation:
- A 24-year-old woman presented with severe systemic symptoms including fever, liver failure, anemia, respiratory distress, cardiomyopathy, and kidney failure.
- She subsequently developed a sensorimotor polyneuropathy, despite normal cerebrospinal fluid analysis.
Findings:
- Sural nerve biopsy revealed mild to moderate axonal damage with signs of regeneration and some demyelination/remyelination.
- Crucially, the biopsy showed hemorrhage within the perineurium and significant infiltration by foamy histiocytes (macrophages) in the subperineural space, likely causing Wallerian degeneration.
Implications:
- This is the first documentation of peripheral nerve histiocyte infiltration in hemophagocytosis syndrome.
- These findings suggest that neuro-histiocytic infiltration may be a previously unrecognized mechanism contributing to polyneuropathy in HPS.