Renal complications in two patients with dentatorubral-pallidoluysian atrophy.

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Dentatorubral-pallidoluysian atrophy (DRPLA ) is an autosomal dominant neurodegenerative disorder characterized by various combinations of myoclonus epilepsy , ataxia , choreoathetosis and dementia . No specific therapy has been established and renal complication is rare . We report two cases of DRPLA with renal complications . Hematuria and proteinuria had gradually progressed for 2 and 13 years in these patients . Renal biopsy findings revealed focal glomerulosclerosis in one case and end-stage kidney disease in the other case . Angiotensin-converting enzyme inhibitor and angiotensin receptor II antagonist were administered to both patients , resulting in improved proteinuria and preserved renal function in one patient , while renal function continued to deteriorate in the other patient . Although renal complication is rare in patients with DRPLA , the presence of renal disease has to be suspected in patients with persistent proteinuria .