O A Oyinlade1, I A Lagunju1, B E Adebayo1. 1. Department of Paediatrics, College of Medicine, University of Ibadan/University College Hospital, Ibadan.
Abstract
BACKGROUND: Nemaline rod disease is a congenital myopathy, presentation of which may mimic myasthenia gravis. METHOD: We report a suspected case of nemaline rod disease in a female adolescent who presented with features similar to myasthenia gravis but failed to respond effectively to its conventional management. She had features of respiratory failure and cardiomyopathy. RESULTS: Patient had a turbulent clinical course and finally succumbed to illness on the fifth day of admission. CONCLUSION: This report is meant to sensitize child neurologists and general paediatricians on the need to have a broad spectrum of considerations in the management of suspected myasthenia gravis, especially when response to anticholinesterase is poor.
BACKGROUND:Nemaline rod disease is a congenital myopathy, presentation of which may mimic myasthenia gravis. METHOD: We report a suspected case of nemaline rod disease in a female adolescent who presented with features similar to myasthenia gravis but failed to respond effectively to its conventional management. She had features of respiratory failure and cardiomyopathy. RESULTS:Patient had a turbulent clinical course and finally succumbed to illness on the fifth day of admission. CONCLUSION: This report is meant to sensitize child neurologists and general paediatricians on the need to have a broad spectrum of considerations in the management of suspected myasthenia gravis, especially when response to anticholinesterase is poor.
Authors: Edoardo Malfatti; Vilma-Lotta Lehtokari; Johann Böhm; Josine M De Winter; Ursula Schäffer; Brigitte Estournet; Susana Quijano-Roy; Soledad Monges; Fabiana Lubieniecki; Remi Bellance; Mai Thao Viou; Angéline Madelaine; Bin Wu; Ana Lía Taratuto; Bruno Eymard; Katarina Pelin; Michel Fardeau; Coen A C Ottenheijm; Carina Wallgren-Pettersson; Jocelyn Laporte; Norma B Romero Journal: Acta Neuropathol Commun Date: 2014-04-12 Impact factor: 7.801