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Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Muscle+Nerve 2015 ; 51 (3): 449-54 Nephropedia Template TP
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Progressive polyradiculoneuropathy due to intraneural oxalate deposition in type 1 primary hyperoxaluria #MMPMID25363903
Muscle Nerve 2015[Mar]; 51 (3): 449-54 PMID25363903show ga
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.