Abstract
Introduction
Patients with Chiari I malformation (CM1) may have chronic symptoms of syringomyelia,
including numbness and weakness of the upper limbs, typically during young adulthood.
Acute or subacute presentation of unilateral foot drop has been rarely reported as
a first symptom of CM1-associated syringomyelia exclusively in childhood or adolescence.
Why these patients do not show any symptoms of the upper limbs although holocord syringomyelia
is always observed on magnetic resonance imaging (MRI) is unclear.
Case presentation
A four-year-old girl presented rapidly with isolated left foot drop. Conventional
MRI revealed holocord syringomyelia associated with CM1. Three-dimensional constructive
interference in steady state (3D-CISS) imaging further demonstrated that the syringomyelia
was comprised of two differential cavities that communicated with each other via a
small pore: a centrally positioned upper cavity and a left-deviated lower one. Surgical
decompression of the foramen magnum resolved the symptom with radiological improvement
of the two cavities.
Conclusion
In contrast to a centrally enlarged syrinx that is often asymptomatic, a paracentrally
extended syrinx usually produces segmental signs related to its levels. Thus, the
left foot drop in this case would have been due to the ipsilaterally deviated lower
cavity that was distinguished from the central upper cavity by 3D-CISS imaging. Further
reports using this imaging technique are needed to verify the hypothetic pathology.
Keywords
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Article info
Publication history
Published online: December 16, 2022
Accepted:
November 30,
2022
Received in revised form:
November 21,
2022
Received:
October 8,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2023 Published by Elsevier B.V. on behalf of The Japanese Society of Child Neurology. All rights reserved.
