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: M Vlak, E van der Kooi, C Angelini
• Journal: Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology (Neurol Sci) Vol. 21 Issue 5 Suppl Pg. S975-7 ( 2000) ISSN: 1590-1874 [Print] Italy
• PMID: 11382199 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adult
• Diagnosis, Differential
• Disability Evaluation
• Female
• Humans
• Male
• Muscle Weakness (diagnostic imaging, pathology, physiopathology)
• Muscle, Skeletal (diagnostic imaging, pathology, physiopathology)
• Muscular Dystrophies (diagnostic imaging, pathology, physiopathology)
• Tomography, X-Ray Computed