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2015 ; 51
(3
): 449-54
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Progressive polyradiculoneuropathy due to intraneural oxalate deposition in type
1 primary hyperoxaluria
#MMPMID25363903
Berini SE
; Tracy JA
; Engelstad JK
; Lorenz EC
; Milliner DS
; Dyck PJ
Muscle Nerve
2015[Mar]; 51
(3
): 449-54
PMID25363903
show 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 and paraffin and epoxy
sections were done 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
polarized light, multiple bright hexagonal, rectangular, and starburst
inclusions, typical of calcium oxalate monohydrate crystals, were seen.
CONCLUSIONS: The 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.