Introduction
A 24 year old man with primary hyperoxaluria type 1 (PH1) presented with a rapidly progressive axonal and demyelinating sensorimotor polyradiculoneuropathy shortly after the onset of end stage renal disease. His plasma oxalate level was markedly elevated at 107 µmol/L (normal: <1.8 µmol/L).
Methods
A sural nerve biopsy was performed. Teased fiber, paraffin and epoxy sections, and morphometric procedures were performed on this sample and on an archived sample from a 22 year old man as an age- and gender-matched control. Embedded teased fiber electron microscopy was also performed.
Results
The biopsy revealed secondary demyelination and axonal degeneration. Under polarizing light, multiple bright hexagonal, rectangular, and starburst inclusions typical of calcium oxalate monohydrate crystals were seen1,2,3.
Discussion
Proposed mechanisms of nerve damage include disruption of axonal transport due to crystal deposition, toxic effect of oxalate, or nerve ischemia related to vessel occlusion from oxalate crystal deposition.