Article

Duchenne muscular dystrophy (DMD), an inherited recessive X
chromosome-linked disease, is the most severe childhood form of muscular
dystrophy. Boys with DMD experience muscle loss, with infiltration of
intramuscular fat into muscles. This case series describes the
progression of DMD in boys using magnetic resonance imaging (MRI) and magnetic
resonance spectroscopy (MRS). Magnetic resonance results are then compared with
an established functional timed test. METHODS: Four boys with DMD and 4 healthy
age-matched controls were chosen from a larger cohort. Boys with DMD were
assessed at 4 time points over 2 years, with controls assessed at baseline only.
Progression of the disease was documented by assessing the plantar flexors using
MRI and MRS techniques and by assessing ambulation using the 30-Foot Fast Walk
Test. RESULTS: Transverse relaxation time (T2) values were elevated in all boys
with DMD at baseline. The lipid ratio increased rapidly as the disease progressed
in 2 boys. Discrete changes in T2 in the other 2 boys with DMD indicated a slower
disease progression. Magnetic resonance imaging and MRS allowed monitoring of the
disease over all time periods regardless of ambulation status. LIMITATIONS: The
magnetic resonance data were collected with 2 different magnets at 2 different
field strengths (1.5 and 3.0 T). Although we corrected for this difference, care
must be taken in interpreting data when different image collection systems are
used. This was a case series of 4 boys with DMD taken from a larger cohort study.
CONCLUSIONS: Magnetic resonance imaging and MRS are objective, noninvasive
techniques for measuring muscle pathology and can be used to detect discrete
changes in both people who are ambulatory and those who are nonambulatory. These
techniques should be considered when monitoring DMD progression and assessing
efficacy of therapeutic interventions.
CI - (c) 2015 American Physical Therapy Association.

Langue : ANGLAIS