Qualitative and quantitative skeletal muscle ultrasound in late-onset acid maltase deficiency.
Muscle Nerve
; 44(3): 418-23, 2011 Sep.
Article
em En
| MEDLINE
| ID: mdl-21755514
ABSTRACT
INTRODUCTION:
Acid maltase deficiency (AMD, or Pompe disease) is an inherited myopathic disorder of glycogen degradation. Diagnosis is often delayed. Muscle ultrasound could improve diagnosis.METHODS:
We compared skeletal muscle ultrasound images from adults with AMD (n = 10) to other myopathies (n = 81) and, in AMD, compared qualitative (Heckmatt) and quantitative (backscatter) ultrasound measurements with strength and function.RESULTS:
Qualitative ultrasound was abnormal in at least one muscle in all AMD subjects. Ultrasound patterns specific for AMD were normal triceps brachii despite abnormalities in elbow flexors (89% vs. 17%, P < 0.0001); focal abnormalities affecting deep more than superficial biceps brachii (40% vs. 4%, P = 0.002); and more severe involvement of vastus intermedius than rectus femoris (40 vs. 11%, P = 0.03). In AMD, both qualitative (Heckmatt) and quantitative (backscatter) ultrasound measures increased with decreasing strength and function.CONCLUSIONS:
Muscle ultrasound identifies the presence and specific patterns of AMD pathology, measures disease severity, and can help in the diagnosis of AMD.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doença de Depósito de Glicogênio Tipo II
/
Músculo Esquelético
Tipo de estudo:
Diagnostic_studies
/
Qualitative_research
Limite:
Adolescent
/
Adult
/
Aged
/
Female
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Humans
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Male
/
Middle aged
Idioma:
En
Revista:
Muscle Nerve
Ano de publicação:
2011
Tipo de documento:
Article
País de afiliação:
Estados Unidos