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P85-T Diagnostic value of conduction block in demyelinating polyneuropathies

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Background Conduction block (CB) is described as a complete or partial failure in the propagation of the nervous impulse within the axon. It is diagnostic of focal demyelination, and suggestive… Click to show full abstract

Background Conduction block (CB) is described as a complete or partial failure in the propagation of the nervous impulse within the axon. It is diagnostic of focal demyelination, and suggestive of acute demyelinating neuropathies (typically inflammatory). Patients and methods We report three cases of demyelinating polyneuropathy with CBs in which their presence led to the erroneous diagnosis of inflammatory polyneuropathy. Results Case 1: 32-year-old woman with a history of slowly progressive gait disturbance and cavus foot, muscle weakness and musculoskeletal pain. In two repeated EMG studies presented a demyelinating polyneuropathy with CBs, and was diagnosed of a chronic inflammatory demyelinating polyneuropathy. Immunosuppressant treatment did not modify the symptoms neither did its withdrawal, finally being diagnosed with autosomal recessive form of Charcot-Marie-Tooth disease (CMT). Case 2: 60-year-old man with lower limb atrophy and absence of deep tendon reflexes. The first genetic test resulted negative for CMT. Later, genetic test presented a new variant of genes PMP22 and ARHGEF10. Case 3: 50-year-old woman with acute myeloid refractory leukemia, who presented a subacute generalized weakness. EMG demonstrated a demyelinating polyneuropathy, predominantly motor, involving proximal segments and with partial CBs. Cerebrospinal fluid studies were normal. Treatment with immunoglobulins was initiated without improvement. Finally, polyneuropathy was attributed to the chemotherapy treatment. Conclusions Although conduction blocks are typical of autoimmune polyneuropathies, their presence has been described in polyneuropathies of another causes. This must be taken into account when making the differential diagnosis.

Keywords: demyelinating polyneuropathy; conduction block; case year; year old; conduction

Journal Title: Clinical Neurophysiology
Year Published: 2019

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