Original article
Acute flaccid weakness with myelopathy and
peripheral nerve involvement in 2 children: Recent
characterization of a previously observed
phenomenon
Gemma Olive
a
, Antonia Clarke
b
, Katja Doerholt
c
, Vasantha Gowda
a
,
Atta Siddiqui
d
, Ming J. Lim
a,*
a
Children's Neurosciences, Evelina London Children's Hospital, Kings Health Partners Academic Health Science
Centre, UK
b
Paediatric Neurology Department, St Georges Healthcare NHS Trust, UK
c
Paediatric Infectious Diseases Unit, St Georges Healthcare NHS Trust, UK
d
Department of Neuroradiology, Guy's and St Thomas' NHS Foundation Trust, King's Health Partners Academic
Health Science Centre, UK
article info
Article history:
Received 18 April 2016
Received in revised form
29 June 2016
Accepted 4 July 2016
Keywords:
Acute flaccid weakness
Myelopathy
Peripheral nerve disease
Infectious etiology
abstract
Background: Acute flaccid weakness may be the first presentation of acute transverse
myelitis (ATM), an immune-mediated central nervous system disorder or may be the first
presentation of anterior horn cell syndrome or peripheral nervous system disease.
Case reports: We describe two previously healthy female infants who presented with acute
flaccid paralysis and encephalopathy. Neuroimaging revealed central cord signal changes
in both cases and surprisingly electrophysiological studies performed revealed a general-
ized axonal motor neuropathy as well.
Conclusion: Clinical, radiological and neurophysiological assessment are important to aid in
the diagnosis and subsequent management of children with overlapping inflammatory
peripheral and central nervous system syndromes.
© 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights
reserved.
1. Introduction
Childhood acute transverse myelitis (ATM) is an immune-
mediated central nervous system (CNS) disorder of potentially
devastating outcome.
1
It may occur as an isolated inflamma-
tory process or as part of a widespread demyelinating disorder
such as in acute disseminated encephalomyelitis (ADEM). Pain,
motor disorders and/or autonomic dysfunction may be the first
symptoms of spinal inflammation,
2
usually evolves over 2e4
* Corresponding author. Children's Neurosciences, Evelina Children's Hospital at Guys and St Thomas' NHS Foundation Trust, Kings
Health Partners Academic Health Science Centre, London, UK Lambeth Palace Road, London SE1 7EH, UK. Fax: þ44 0 20 71880851.
E-mail address: Ming.Lim@gstt.nhs.uk (M.J. Lim).
Official Journal of the European Paediatric Neurology Society
european journal of paediatric neurology xxx (2016) 1 e5
http://dx.doi.org/10.1016/j.ejpn.2016.07.008
1090-3798/© 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Olive G, et al., Acute flaccid weakness with myelopathy and peripheral nerve involvement in 2
children: Recent characterization of a previously observed phenomenon, European Journal of Paediatric Neurology (2016), http://
dx.doi.org/10.1016/j.ejpn.2016.07.008