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Related Experiment Videos

Myopathy associated with linear scleroderma. A histochemical and electron microscopic study.

L Z Stern, C M Payne, J T Alvarez

    Neurology
    |February 1, 1975
    PubMed
    Summary

    Linear scleroderma can affect underlying rectus femoris muscle, causing weakness and atrophy. Ultrastructural analysis revealed thickened capillary basal laminae and unique mitochondrial inclusions in affected muscle fibers.

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    Area of Science:

    • Muscle pathology
    • Dermatology
    • Electron microscopy

    Background:

    • Linear scleroderma is a connective tissue disease that can affect underlying muscle.
    • Muscle involvement can lead to weakness and atrophy, impacting patient mobility.
    • Understanding the pathologic changes is crucial for diagnosis and management.

    Purpose of the Study:

    • To describe the pathologic findings in rectus femoris muscle affected by linear scleroderma.
    • To correlate electromyography findings with muscle pathology.
    • To investigate ultrastructural changes in muscle capillaries and mitochondria.

    Main Methods:

    • Biopsy of rectus femoris muscle from a patient with linear scleroderma.
    • Light microscopy and histochemical analysis of muscle fibers.

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  • Electromyography (EMG) to assess muscle electrical activity.
  • Transmission electron microscopy (TEM) for ultrastructural examination.

    • Main Results:

    • Affected muscle showed weakness and atrophy.
    • EMG revealed motor unit potentials with decreased amplitude and duration.
    • Light microscopy showed atrophy of type I muscle fibers.
    • Ultrastructural analysis revealed thickened and reduplicated capillary basal laminae.
    • Distinct electron-dense inclusions were observed within mitochondria.

    Conclusions:

    • Linear scleroderma can cause significant muscle pathology, including fiber atrophy and capillary changes.
    • Ultrastructural findings, particularly mitochondrial inclusions, may represent a specific marker of the disease.
    • These findings highlight the importance of integrating clinical, electrophysiologic, and ultrastructural data for comprehensive diagnosis.