P957: Mixed-type polyneuropathy in Wilson's disease

Abstract

Wilson’s Disease (WD) is an autosomal recessive disorder of abnormal copper metabolism witch predominantly affects the central nervous system and the liver. Peripheral nervous system involvement is rarely reported in the context of WD and not well characterized. Here we describe a WD patient with peripheral neuropathy. A 58-year-old male suffering from WD on therapy with trientine referred to our center because of mild distal limb weakness with hypoesthesia. Clinical evaluation showed mild leg weakness with foot drop and hyporreflexia. A nerve conduction study was performed using standard techniques (Medtronic Keypoint v5.09). Distal motor latency, F-wave latency, motor nerve conduction velocity and CMAP were evaluated in the deep peroneal, posterior tibial, median and ulnar nerves; sensory nerve conduction velocity and SAP were measured in the sural, superficial peroneal, median, ulnar and radial nerve. No other cause of polyneuropathy was found. Electroneurographyc findings suggested an asymmetric length-dependent sensory-motor neuropathy of mixed type. We confirmed peripheral nervous system involvement in WD. According to the literature, also our findings showed both myelin and axonal damage. Polyneuropathy may appear despite therapy, although a iatrogenic contribution cannot be excluded.