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Muscle Contracture, Pseudomyotonia, Tetanus, and Related States
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Physiologic Contracture Due to Phosphorylase De ciency (McArdle) and Phosphofructokinase De ciency (Tarui) These are examples of an entirely different type of painful shortening and hardness of muscle In both these diseases, an otherwise healthy child, adolescent, or adult begins to complain of weakness and stiffness and sometimes pain on using the limbs Muscle contraction and relaxation are normal when the patient is in repose, but strenuous activity, especially under conditions of ischemia, causes the muscles to shorten gradually, due to a failure of relaxation The contracted muscles in these disorders unlike muscles in cramp,
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DISORDERS OF MUSCLE
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cramps and spasms and then a painful rigidity of abdominal, trunk, and leg muscles The spasms are followed by weakness There is also vasoconstriction, hypertension, and autonomic hyperactivity If death does not occur in the rst 24 to 48 h, recovery is complete The spider venom has a presynaptic localization and rapidly releases quanta of ACh The vesicles are depleted There is some evidence that the venom prevents endocytosis of the vesicles by inserting itself into the presynaptic membranes, causing a disturbance of ionic conductance channels (Swift) Treatment, which is more or less empiric, consists of calcium gluconate infusions Intravenous magnesium sulfate also helps to reduce the release of ACh and control the convulsions that sometimes occur There is a reconstituted antiserum that is available in regions where such envenomation is frequent Malignant Hyperthermia (See page 1272) This condition is also characterized by an acute onset of generalized muscular rigidity accompanied by a rapid rise in body temperature, metabolic acidosis, and myoglobinuria It is invariably induced by anesthetic agents and other drugs
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this is dif cult to interpret, since it may be found in otherwise healthy individuals The sedimentation rate is greatly elevated in the majority of patients, and a 48-h trial of prednisone, by alleviating muscle pain, con rms the diagnosis In the context of muscle pain, systemic symptoms such as weight loss, headache, and fatigue, as well as mild anemia, are particularly suggestive of polymyalgia rheumatica Fibromyalgia This would appear, by de nition, to represent an in ammation of the brous tissues of the muscles, fascia, aponeuroses, and possibly nerves as well Unfortunately, the pathologic basis of this state remains obscure Only some clinical facts are at hand During the rst movements after a period of inactivity, a muscle or group of muscles may become painful and tender, particularly after exposure to cold, dampness, or minor trauma, but often for no reason that can be discerned One looks in vain for signs of tendinous or arthritic disease The neck and shoulders are the most common sites Sometimes rm, tender areas, up to several centimeters in diameter, can be palpated within the muscles ( brositic nodules ), and active contraction or passive stretching of the involved muscles increases the pain points said to be of diagnostic value, but still disputed Often, symptoms such as mental and physical fatigue, insomnia, and headache are associated and raise the suspicion of an anxiety state or depression In a few instances the condition clears up in a few weeks; local heat and massage and local injections of anesthetics or steroids are found to give comfort while symptoms are present, but most often it becomes a chronic condition The chronic form of bromyalgia presents far greater problems, usually disabling the patient and causing a change in accustomed habits and employment It has become one of the prime diagnoses made by rheumatologists and physiatrists, but the patient may rst come to the attention of a neurologist Muscle pain is the primary symptom, with fatigue and the other aforementioned complaints added as an afterthought by the patient, or the muscular symptoms are elicited in exploring a case of the equally vexing condition now called chronic fatigue syndrome (pages 190 and 435) There is a great preponderance of women among these patients, and in the majority the illness is not predated by any particular factor, although a few have a viral prodrome or recently preceding trauma Most de nitions of the syndrome have been circular or somewhat arbitrary Those now in general use are similar to the one proposed by a committee of the American College of Rheumatology The basis for diagnosis is the presence of widespread pain including focal areas of pain (trigger points) that can be produced by 4 kg of digital pressure in 11 of 18 typical locations over muscles, tendons, or bone these are concentrated around the shoulders and paraspinal regions and there is no requirement for the presence of the several common systemic complaints that accompany the illness in most patients (fatigue, dif culty concentrating, sleeping dif culty, or anxiety) There is said to be a knot or brous band at the site of tenderness, but this is dif cult to verify There is, to say the least, controversy regarding the nature of these pressure points and the validity of methods used to elicit them, since mechanical dolorimetry often suggests that these patients have a reduced tolerance to pain at all sites But there is also uncertainty regarding the relationship of the symptoms to underlying depression In the past, similar pains were associated with cases of irritable bowel or irritable bladder syndromes, dysmenorrhea, chronic headache, and cold intolerance Depending upon
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