Muscle computed tomography in adult-onset acid maltase deficiency.

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.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

de Jager AE,van der Vliet TM,van der Ree TC,Oosterink BJ,Loonen MC

doi

10.1002/(sici)1097-4598(199803)21:3<398::aid-mus15

subject

Has Abstract

pub_date

1998-03-01 00:00:00

pages

398-400

issue

3

eissn

0148-639X

issn

1097-4598

pii

10.1002/(SICI)1097-4598(199803)21:3<398::AID-MUS15

journal_volume

21

pub_type

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