title: Temporalis muscle hypertrophy and reduced skull eccentricity in Duchenne muscular dystrophy.
abstract: Muscle hypertrophy and muscle weakness are well known in Duchenne muscular dystrophy. Decreased muscle force can have secondary effects on skeletal growth and development such as facial and dental morphology changes. In this study, we quantified temporal muscle thickness, circumference, and eccentricity of the skull and the head on T1-weighted magnetic resonance imaging (MRI) scans of the head of 15 Duchenne muscular dystrophy patients and 15 controls. Average temporal muscle thickness was significantly increased in patients (12.9 ± 5.2 mm) compared to controls (6.8 ± 1.4 mm) (P < .0001), whereas the shape of the skull was significantly rounder compared to controls. Temporal muscle thickness and skull eccentricity were significantly negatively correlated in patients, and positively in controls. Hypertrophy of the temporal muscles and changes in skull eccentricity appear to occur early in the course of Duchenne muscular dystrophy. Further studies in younger patients are needed to confirm a causal relationship.
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands c.s.m.straathof@lumc.nl.
authors.forename: C S M
authors.initials: CS
authors.lastname: Straathof
authors.affiliation: Department of Radiology, C.J. Gorter Center, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: N
authors.initials: N
authors.lastname: Doorenweerd
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: B H A
authors.initials: BH
authors.lastname: Wokke
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: E M
authors.initials: EM
authors.lastname: Dumas
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: J C
authors.initials: JC
authors.lastname: van den Bergen
authors.affiliation: Department of Radiology, C.J. Gorter Center, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: M A
authors.initials: MA
authors.lastname: van Buchem
authors.affiliation: Center of Neurological Learning Disabilities, Epilepsy Center Kempenhaeghe, Heeze, the Netherlands.
authors.forename: J G M
authors.initials: JG
authors.lastname: Hendriksen
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: J J G M
authors.initials: JJ
authors.lastname: Verschuuren
authors.affiliation: Department of Radiology, C.J. Gorter Center, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: H E
authors.initials: HE
authors.lastname: Kan
mesh_terms: D000293:Adolescent; D002648:Child; D006801:Humans; D006984:Hypertrophy; D008279:Magnetic Resonance Imaging; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000473:pathology*; D009929:Organ Size; D012886:Skull / Q000473:pathology*; D013703:Temporal Muscle / Q000473:pathology*; D044465:White People
keywords: MRI; muscle disease; muscular dystrophy; pediatric