title: Correlation of clinical function and muscle CT scan images in limb-girdle muscular dystrophy.
abstract: Knowledge of selective muscle-involvement is useful to the clinician. Therefore we investigated in sarcoglycanopathy and dysferlinopathy: (i) the correlation between clinical scale, MRC scale and CT findings and (ii) the muscles involved. Patients with a definite diagnosis of dysferlinopathy and sarcoglycanopathy were tested for their clinical functions and muscle strength and assigned a functional grade. Nineteen muscles were evaluated by CT. In dysferlinopathy, distal lower-limb muscles are involved, while in sarcoglycanopathy proximal muscles are more affected. In both groups, muscles with MRC < 3 often had severe abnormalities on CT imaging, while muscles with MRC > 4 could be either normal or abnormal. Dysferlinopathy and sarcoglycanopathy have different and selective muscle involvement. This may affect certain types of functional activity. There is a possible relationship between CT findings and MRC grade in muscles with a low (< 3) MRC grade. Abnormality on CT scan may precede the clinical symptoms.
authors.affiliation: Neuromuscular Centre, Department of Neurology, University of Padua, Via Giustiniani 5, I-35128 Padua, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Vlak
authors.forename: E
authors.initials: E
authors.lastname: van der Kooi
authors.forename: C
authors.initials: C
authors.lastname: Angelini
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D008297:Male; D018908:Muscle Weakness / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology*; D009136:Muscular Dystrophies / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology*; D014057:Tomography, X-Ray Computed