CHARCOT-MARIE-TOOTH MUSCULAR-ATROPHY (HER EDITARY MOTOR AND SENSORY NEUROPATHY TYPE-I) AND PROGRESSIVE RENAL-INSUFFICIENCY WITH FOCAL AND SEGMENTAL GLOMERULOSCLEROSIS

The peroneal muscular atrophy Charcot-Marie-Tooth (hereditary motor an d sensory neuropathy type I) is a familiar neuropathy which begins in early childhood presenting with stepdance movements, 13 patients are k nown from the literature presenting with signs of a nephropathy in add ition to the underlying disease, Focal and seg mental glomeruloscleros is is the predominant histo-pathologic diagnosis leading progressively to endstage renal failure. We report about a 13 year old girl known t o have had peroneal muscular atrophy Charcot-Marie-Tooth for years, Sh e presented with acute cerebral seizures and consequently advanced ren al failure was diagnosed, Regular peritoneal dialysis had to be initia ted two years later. In all known cases of nephropathy associated with Charcot-Marie-Tooth muscular atrophy, proteinuria is one of the first symptoms. We therefore conclude that an urinary examination should re gularly be performed in all patients with hereditary motor and sensory neuropathy type I.