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The mild benefits in muscle strength experienced by HIBM patients after IVIG …


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- Intravenous immune globulin in hereditary inclusion body myopathy: a pilot study

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Figure 1 Serum IgG levels in patients during the course of treatment with IVIG. Arrows indicate loading doses (1 g/kg) and maintenance doses (0.4 g/kg).

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Figure 2 Quadriceps and shoulder abduction testing in four patients with HIBM. On average, quadriceps strength increased 22% after IVIG loading and 35% at the end of the study. Shoulder abduction strength increased 44% after IVIG loading and 46% at the end of the study.

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Figure 3 Expression of α-dystroglycan in quadriceps muscle from HIBM patients. A-F. Immunohistochemistry using antibodies to IIH6. No consistent difference in staining was apparent before (A,C,E) compared with after (B,D,F) IVIG treatment. The specimen shown in A was sampled from a deteriorating area. G. Immunoblots of muscle from a normal individual (N) and HIBM patients 1, 2, and 4, labeled with antibodies to α-dystroglycan. No consistent difference in IIH6 staining (reflecting the sialylation status of α-dystroglycan) was apparent before (Pre) compared with after (Post) IVIG treatment. β-Actin bands provide an indication of the level of protein loading.

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Figure 4 Expression of sialylated NCAM in muscle of HIBM patients. A. Muscle from two control individuals exhibit NCAM mobility consistent with a higher molecular weight, indicative of sialylation. N1 is normal and N2 has sporadic IBM; note increased NCAM in this person's muscle. The muscle of patient 1 also has an increased amount of NCAM; patients 2 and 4 have moderate amounts of NCAM. The NCAM of the HIBM patients migrates with greater mobility than normal, indicating lack of sialylation. The amount of muscle NCAM decreased after (Post) compared with before (Pre) IVIG treatment in patients 1 and 2, but increased in patient 4. B. Sialidase treatment increased the mobility of control muscle (N1 and N2). The mobility of muscle NCAM was not altered by sialidase treatment in patient 2, but it was increased by sialidase treatment in patient 1.

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