Volume 21, Issue 3 pp. 398-400
Short Report

Muscle computed tomography in adult-onset acid maltase deficiency

Aeiko E.J. de Jager PhD

Corresponding Author

Aeiko E.J. de Jager PhD

Department of Neurology, University Hospital Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands

Department of Neurology, University Hospital Groningen, P.O. Box 30.001, 9700 RB Groningen, The NetherlandsSearch for more papers by this author
Ton M. van der Vliet MD

Ton M. van der Vliet MD

Department of Neuroradiology, University Hospital Groningen, Groningen, The Netherlands

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Taco C. van der Ree MD

Taco C. van der Ree MD

Department of Neurology, University Hospital Rotterdam, Rotterdam, The Netherlands

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Bart J. Oosterink MD

Bart J. Oosterink MD

Department of Neurology, University Hospital Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands

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M. Christa B. Loonen PhD

M. Christa B. Loonen PhD

Department of Neurology, University Hospital Rotterdam, Rotterdam, The Netherlands

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Abstract

Nine patients with adult-onset acid maltase deficiency (Pompe's disease) were examined clinically and with computed tomography (CT). The CT scan showed early and severe involvement of the muscles of trunk and thighs, with selective sparing of the tensor fasciae latae, short head of biceps femoris, gracilis, and sartorius muscles. Shoulder and leg muscles were less affected. The disease spread over the years from trunk to extremities. Muscle strength and CT findings were positively correlated. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21:398–400, 1998.

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