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Gait & Posture
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Short communication
O 050 - Gait and muscle characteristics in a girl with hereditary spastic
paraplegia: A clinical case study
H. Adams
a,
⁎
, N. De Beukelaer
b
, C. Huenaerts
a
, B. Hanssen
b
, L. Bar-on
b
, S.H. Schless
b
,
A. Van Campenhout
c
, E. Ortibus
d
, N. Peeters
b
, K. Desloovere
a,b
a
Clinical Motion Analysis Laboratory, University Hospitals Leuven, Department of Orthopedics, Leuven, Belgium
b
KU Leuven, University of Leuven, Department of Rehabilitation Sciences, Neuromotor Rehabilitation Research Group, Leuven, Belgium
c
UZ Leuven, Department of Orthopedic Surgery, Leuven, Belgium
d
Development and Regeneration, KU Leuven, University of Leuven, Leuven, Belgium
ARTICLE INFO
Keywords:
Hereditary spastic paraplegia
Muscle characteristics
Gait
Cerebral palsy
Spasticity
1. Introduction
While the etiology of hereditary spastic paraplegia (HSP) clearly
differs from spastic cerebral palsy (SCP), the phenotype of 3SPGA-HSP
and bilateral SCP children show much similarity. Hence, HSP children
are commonly treated as SCP children. However, the HSP gait pattern
and its relation to muscle impairment (spasticity, weakness and mor-
phology) has only rarely been studied. A detailed assessment of pa-
thological gait and muscle impairment of the HSP child, compared to
typically developing (TD) and bilateral SCP children, may help to de-
fine HSP-specific characteristics that can support the clinical decision-
making. The gait and muscle impairment of an 11-year old girl, with
HSP (3SPGA gene mutation, age of onset: 5.5 years) was compared to
TD children as well as to children with bilateral SCP, matched for age
and gross motor function classification system (GMFCS1).
2. Research question
Do 3D gait analysis (3DGA), muscle morphology and instrumented
spasticity assessment (ISA) provide sufficient information for pa-
thology-specific characteristics and treatment, distinguishing this HSP-
patient from a SCP-cohort?
3. Methods
The HSP-patient and five age/GMFCS-matched SCP children re-
ceived 3DGA, including kinematics and kinetics (Vicon, AMTI-for-
ceplates). Apart from the standard clinical exam (Modified Ashworth
Scale (MAS), range of motion (ROM) and muscle strength) spasticity
was quantified with ISA, integrating dynamometry (torque), kinematics
and EMG while stretching the medial gastrocnemius (MG) at low ve-
locity (LV) and high velocity (HV) [1]. 3D freehand Ultrasound mea-
surement (3DfUS) was used to acquire muscle length normalized to
muscle-tendon-unit length (nML), normalized muscle volume (nMV)
and mean echo intensity (EI) [2].
4. Results
This HSP gait pattern is characterized by increased knee flexion at
initial contact, pathological first and second ankle rocker and increased
plantar flexion in swing phase, which is a frequently observed pattern
of SCP children (Fig. 1). Lower limb spasticity is confirmed by both
objective (ISA) and subjective (MAS) spasticity assessment. MAS is
slightly higher (MAS = 3) compared to the GMFCS I-matched SCP-
group (median-MAS = 2). ISA reveals high torque when stretching MG
at high velocity, indicating a large neural component. The HSP-patient
and the SCP-group showed similar results for muscle structure, where
EI was increased and nML and nMV were decreased compared to TD
https://doi.org/10.1016/j.gaitpost.2018.06.077
⁎
Corresponding author.
E-mail address: heleen.1.adams@uzleuven.be (H. Adams).
Gait & Posture xxx (xxxx) xxx–xxx
0966-6362/ © 2018 Elsevier B.V. All rights reserved.
Please cite this article as: Adams, H., Gait & Posture (2018), https://doi.org/10.1016/j.gaitpost.2018.06.077