{Reference Type}: Journal Article
{Title}: Electrophysiological demyelinating features in hereditary ATTR amyloidosis.
{Author}: Ohashi N;Kodaira M;Morita H;Sekijima Y;
{Journal}: Amyloid
{Volume}: 26
{Issue}: 1
{Year}: Mar 2019
{Factor}: 6.571
{DOI}: 10.1080/13506129.2018.1564903
{Abstract}: <B>OBJECTIVE: </B>To elucidate the electrophysiological demyelinating features in patients with hereditary ATTR amyloidosis that may lead to a misdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).<BR><B>METHODS: </B>In 102 patients with hereditary ATTR amyloidosis (85 Val30Met and 17 non-Val30Met; 37 and 65 from endemic and non-endemic areas, respectively), results of motor nerve conduction studies (MNCSs) with a 2-Hz low-cut filter in the unilateral ulnar and tibial nerves were retrospectively investigated to assess whether each MNCS parameter demonstrated demyelinating features that fulfil the European Federation of Neurological Societies/Peripheral Nerve Society electrodiagnostic (EFNS/PNS EDX) criteria for CIDP.<BR><B>RESULTS: </B>Thirteen patients with low compound muscle action potential (CMAP) amplitude in the tibial nerve (0.7 ± 0.7 mV) and prolonged distal CMAP duration in the ulnar nerve satisfied the definite EFNS/PNS EDX criteria for CIDP. Abnormal temporal dispersion and prolongation of distal latency in the tibial nerve were observed in 5 of 13 patients. However, only one of the 13 patients presented with the reduction of motor conduction velocity in each nerve. No patient exhibited conduction block in any nerve.<BR><B>CONCLUSIONS: </B>Patients with hereditary ATTR amyloidosis occasionally show electrophysiological demyelinating features without conduction block following severe axonal degeneration.