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Diseases of Skeletal Muscle

R. H. T. Edwards, D. A. Jones

10.1002/cphy.cp100120

Source: Supplement 27: Handbook of Physiology, Skeletal Muscle

Originally published: 1983

Published online: January 2011

Full Article on Wiley Online Library



Abstract

The sections in this article are:

1 History
2 Clinical Presentation of Muscle Disease
2.1 Weakness
2.2 Fatigue
2.3 Myoglobinuria
2.4 Unusual Function
3 Investigation of Patients with Muscle Disease
3.1 Clinical History and Examination
3.2 Blood Biochemistry
3.3 Electromyography
3.4 Muscle Biopsy
3.5 Nuclear Magnetic Resonance
3.6 Measurement of Muscle Cross‐Sectional Area
3.7 Tests of Muscle Function
3.8 Isolated Human Muscle Preparations
3.9 Exercise and Fasting as Provocation Tests
3.10 Sudies of Protein Turnover
4 Analysis of Muscle Weakness
4.1 Dystrophies
4.2 Inflammatory Myopathies
4.3 Atrophy and Hypertrophy
4.4 Factors Affecting Protein Turnover in Muscle
4.5 Neuropathies
5 Analysis of Abnormal Fatigability
5.1 Muscle Fatigue in Normal Subjects
5.2 Myasthenia Gravis
5.3 Disorders of Energy Metabolism
5.4 Disorders of Mitochondrial Oxidative Metabolism
5.5 Disorders of Fat Metabolism
5.6 Changes in Muscle Function
6 Disordered Function
6.1 Myotonic Syndromes
6.2 Periodic Paralysis
6.3 Malignant Hyperpyrexia
6.4 Muscle Pain, Cramps, and Contractures
7 Drug‐Induced Myopathies
8 Mechanisms Underlying Muscle Disease
8.1 Vascular and Humoral Influences
8.2 Influence of Nerve on Muscle
8.3 Disorders of the Cell Membrane
8.4 Muscle Protease Activity
8.5 Inflammatory Disorders of Muscle
8.6 Overuse and Disuse as Causes of Muscle Damage
9 Prospects For Treatment of Muscle Disease
Figure 1. Figure 1.

Computerized tomography in a male West Indian (age 44 yr) with limb‐girdle dystrophy. Top: cross sections in midthigh region. There is partial replacement of quadriceps muscle and almost complete replacement of posterior thigh (hamstring) muscles with fat. Bottom: cross sections in region of maximum calf circumference. There is hypertrophy of anterior tibial muscles, preservation of gastrocnemius, and replacement of soleus muscle by fat.
Figure 2. Figure 2.

Abnormal response to exercise of a patient with defect of mitochondrial metabolism. ▪, Normal subject; work done = 196 W (70% max), □, Patient with a defect in mitochondrial pyruvate metabolism; work done = 65 W (59% max). Note that despite doing less absolute and relative work, patient had a much greater rise in plasma lactate, which was slow to recover. [From Edwards et al. 131]
Figure 3. Figure 3.

Dystrophic changes in muscle: cross sections of quadriceps muscle stained with hematoxylin‐eosin. A: normal (male, 22 yr). B: early dystrophy (male, 9 yr); note internal nuclei and variations in fiber size. The overall difference in fiber size between A and B reflects age difference between the 2 subjects and is not abnormal. C: advanced dystrophy (male, 17 yr); note loss of contractile material, which is replaced by fat and fibrous tissue. Scale bar = 50 μm.
Figure 4. Figure 4.

Cross section of quadriceps muscle in patient with polymyositis (female, 48 yr); note marked differences in fiber size, internal nuclei, and infiltration with macrophages. Scale bar = 50 μm.
Figure 5. Figure 5.

Atrophic changes. Cross sections of quadriceps muscle stained for myosin ATPase at pH 9.4. Type I fibers, light staining; type II fibers, dark staining. A: normal male. B: male patient showing type II fiber atrophy after steroid therapy. C: normal female. D: female with Cushing's syndrome showing severe type II fiber atrophy. Scale bar = 50 μm.
Figure 6. Figure 6.

Consequences of muscle denervation. Low‐power views of cross sections of quadriceps muscle, stained for myosin ATPase at pH 9.4. A: normal muscle; note the even distribution of type I and II fibers. B: muscle from a patient with a neuropathy. A number of small atrophied fibers can be seen; these are a consequence of denervation. Main feature is pronounced fiber grouping, which is due to reinnervation of fibers by sprouting of nearby surviving motor nerve axons. Scale bar = 500 μm.
Figure 7. Figure 7.

Force generated by percutaneous stimulation of quadriceps muscle at different frequencies. Muscle was stimulated with 5 impulses at 1 Hz and then for 2 s successively at 10, 20, 50, and 100 Hz. A: fresh, rested muscle. B: same muscle tested after 10‐min rest after a 20‐min step test. Note that although maximum tetanic force is nearly fully recovered, force generated at low frequencies is much reduced.


Figure 1.

Computerized tomography in a male West Indian (age 44 yr) with limb‐girdle dystrophy. Top: cross sections in midthigh region. There is partial replacement of quadriceps muscle and almost complete replacement of posterior thigh (hamstring) muscles with fat. Bottom: cross sections in region of maximum calf circumference. There is hypertrophy of anterior tibial muscles, preservation of gastrocnemius, and replacement of soleus muscle by fat.



Figure 2.

Abnormal response to exercise of a patient with defect of mitochondrial metabolism. ▪, Normal subject; work done = 196 W (70% max), □, Patient with a defect in mitochondrial pyruvate metabolism; work done = 65 W (59% max). Note that despite doing less absolute and relative work, patient had a much greater rise in plasma lactate, which was slow to recover. [From Edwards et al. 131]



Figure 3.

Dystrophic changes in muscle: cross sections of quadriceps muscle stained with hematoxylin‐eosin. A: normal (male, 22 yr). B: early dystrophy (male, 9 yr); note internal nuclei and variations in fiber size. The overall difference in fiber size between A and B reflects age difference between the 2 subjects and is not abnormal. C: advanced dystrophy (male, 17 yr); note loss of contractile material, which is replaced by fat and fibrous tissue. Scale bar = 50 μm.



Figure 4.

Cross section of quadriceps muscle in patient with polymyositis (female, 48 yr); note marked differences in fiber size, internal nuclei, and infiltration with macrophages. Scale bar = 50 μm.



Figure 5.

Atrophic changes. Cross sections of quadriceps muscle stained for myosin ATPase at pH 9.4. Type I fibers, light staining; type II fibers, dark staining. A: normal male. B: male patient showing type II fiber atrophy after steroid therapy. C: normal female. D: female with Cushing's syndrome showing severe type II fiber atrophy. Scale bar = 50 μm.



Figure 6.

Consequences of muscle denervation. Low‐power views of cross sections of quadriceps muscle, stained for myosin ATPase at pH 9.4. A: normal muscle; note the even distribution of type I and II fibers. B: muscle from a patient with a neuropathy. A number of small atrophied fibers can be seen; these are a consequence of denervation. Main feature is pronounced fiber grouping, which is due to reinnervation of fibers by sprouting of nearby surviving motor nerve axons. Scale bar = 500 μm.



Figure 7.

Force generated by percutaneous stimulation of quadriceps muscle at different frequencies. Muscle was stimulated with 5 impulses at 1 Hz and then for 2 s successively at 10, 20, 50, and 100 Hz. A: fresh, rested muscle. B: same muscle tested after 10‐min rest after a 20‐min step test. Note that although maximum tetanic force is nearly fully recovered, force generated at low frequencies is much reduced.

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Article Title: Diseases of Skeletal Muscle
 

How to Cite

R. H. T. Edwards, D. A. Jones. Diseases of Skeletal Muscle. Compr Physiol 2011, Supplement 27: Handbook of Physiology, Skeletal Muscle: 633-672. First published in print 1983. doi: 10.1002/cphy.cp100120