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Defective neutrophil function in myotonic dystrophy.

A R Seay, F A Ziter, H R Hill

    Journal of the Neurological Sciences
    |January 1, 1978
    PubMed
    Summary
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    Polymorphonuclear leukocytes (PMNs) from myotonic dystrophy patients show impaired chemotaxis and chemiluminescence. These immune cell defects suggest PMNs are affected by the disease's underlying membrane abnormalities.

    Area of Science:

    • Immunology
    • Neurology
    • Genetics

    Background:

    • Myotonic dystrophy is a multisystem disorder characterized by progressive muscle wasting.
    • Generalized membrane abnormalities are implicated in the pathophysiology of myotonic dystrophy.
    • Polymorphonuclear leukocytes (PMNs) play a crucial role in innate immunity.

    Purpose of the Study:

    • To investigate the functional status of PMNs in patients with myotonic dystrophy.
    • To assess PMN chemotaxis, chemiluminescence (CL), and phagocytosis in myotonic dystrophy.
    • To determine if serum factors in myotonic dystrophy patients affect PMN function.

    Main Methods:

    • PMNs were isolated from 10 myotonic dystrophy patients and 39 age-matched controls.
    • Chemotactic activity, CL, and phagocytosis assays were performed on isolated PMNs.

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  • Control PMNs were incubated in patient serum to evaluate serum-mediated effects.

    • Main Results:

    • PMNs from 80% of myotonic dystrophy patients exhibited significantly depressed chemotactic indexes compared to controls (P < 0.0003).
    • Reduced peak chemiluminescence was observed in PMNs from 60% of patients.
    • Phagocytosis and bactericidal activity in PMNs were found to be normal in the evaluated patients.
    • Incubation of control PMNs in patient serum did not alter their chemotactic activity or CL response.

    Conclusions:

    • Myotonic dystrophy is associated with defects in PMN chemotaxis and chemiluminescence.
    • These findings indicate abnormalities in PMN membrane-associated functions in myotonic dystrophy.
    • The results suggest that PMNs are affected by the generalized membrane defect underlying myotonic dystrophy.