EM Guidemap - Myopathy and Myoglobulinuria
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myopathy EM guidemap - Myopathy and myoglobulinuria Click on any of the headings or subheadings to rapidly navigate to the relevant section of the guidemap Introduction General principles ● endocrine myopathy ● toxic myopathy ● periodic paralyses ● myoglobinuria Introduction - this short guidemap supplements the neuromuscular weakness guidemap and offers the reader supplementary information on myopathies, and a short section on myoglobulinuria - this guidemap only consists of a few brief checklists of "causes of the different types of myopathy" that an emergency physician may encounter in clinical practice when dealing with a patient with acute/subacute muscular weakness General principles - a myopathy is suggested when generalized muscle weakness involves large proximal muscle groups, especially around the shoulder and proximal girdle, and when the diffuse muscle weakness is associated with normal tendon reflexes and no sensory findings - a simple classification of myopathy:- Hereditary ● muscular dystrophies ● congenital myopathies http://www.homestead.com/emguidemaps/files/myopathy.html (1 of 13)8/20/2004 5:14:27 PM myopathy ● myotonias ● channelopathies (periodic paralysis syndromes) ● metabolic myopathies ● mitochondrial myopathies Acquired ● inflammatory myopathy ● endocrine myopathies ● drug-induced/toxic myopathies ● myopathy associated with systemic illness - a myopathy can present with fixed weakness (muscular dystrophy, inflammatory myopathy) or episodic weakness (periodic paralysis due to a channelopathy, metabolic myopathy due to certain glycolytic pathway disorders) - a myopathy can be acute (< 4 weeks), subacute (4 - 8 weeks) or chronic (> 8 weeks) - channelopathies and certain metabolic myopathies can produce recurrent episodes of episodic weakness, while certain toxins (eg. cocaine) can produce a single episode of episodic weakness - abnormal weakness after exercise suggests a channelopathy, or a metabolic or mitochondrial myopathy (mimics myasthenia gravis) - muscle pain (myalgia) is surprisingly infrequent in many myopathies, and the presence of episodic muscle pain suggests a metabolic myopathy, while constant muscle pain suggests an inflammatory myopathy - muscle cramps are not common in myopathic disorders, and they strongly suggest anterior horn cell disorders eg. amyotrophic lateral sclerosis, or many non-specific conditions eg. dehydration and hyponatremia, azotemia, hemodialysis, pregnancy - muscle contractures resemble cramps, but they last longer and are usually provoked by exercise in patients with glycogenolytic enzyme defects - myotonia is the phenomenon of impaired muscle relaxation after a forceful muscle contraction; patients complain of muscle stiffness and difficulty releasing their handgrip after a handshake - myotonia often improves after exercise (warm-up phenomenon), in contrast to paratonia where exercise makes the myotonia worse; both myotonia and paratonia are http://www.homestead.com/emguidemaps/files/myopathy.html (2 of 13)8/20/2004 5:14:27 PM myopathy usually worse when exposed to the cold (* paratonia suggests frontal lobe disease and is seen in association with other signs suggestive of frontal lobe disease eg. frontal gait ataxia, "gegenhalten") - certain myopathies may produce rhabdomyolysis and myoglobulinuria, and the combination of weakness + muscle pain + discolored red urine after mild exercise, or prolonged exercise, suggests a metabolic myopathy eg. glycolytic pathway defect or fatty acid oxidation defect (* strenuous anaerobic exercise can produce rhabdomyolysis and myoglobulinuria even in healthy people) - muscle atrophy is a late finding in chronic progressive myopathies, and the pattern of muscle atrophy can suggest a particular myopathy eg. atrophy of the peri-scapular muscles causing scapular winging suggests fascioscapulohumeral dystrophy, selective atrophy of the quadriceps and forearm muscles suggests inclusion body myopathy - the pattern of muscle weakness can suggest a particular myopathy ● limb girdle weakness (proximal muscle weakness) is non-specific and is seen in many hereditary and acquired myopathies ● scapuloperoneal weakness due to weakness of the peri-scapular muscles and muscles of the anterior compartment of the lower leg in association with facial muscle weakness suggests fascioscapulohumeral dystrophy, scapuloperoneal dystrophy, Emery-Dreifuss dystrophy, acid maltase deficiency and some congenital myopathies ● asymmetric weakness of of distal forearm muscles (wrist and finger flexors) + proximal lower extremities (quadriceps) is essentially pathognomonic of inclusion body myositis => produces weakness of hand grip and frequent falling ● prominent neck extensor muscle weakness (dropped head) suggests isolated neck extensor myopathy, polymyositis, dermatomyositis, inclusion body myositis, carnitine deficiency, myotonic dystrophy, or a congenital myopathy ● predominant weakness of ocular and pharyngeal muscles suggests oculopharyngeal dystrophy, while ptosis and ophthalmoplegia without prominent pharyngeal involvement suggests a motochondrial myopathy ● ptosis and facial weakness without ophthalmoplegia and pharyngeal weakness suggests myotonic dystrophy Endocrine myopathy Hypothyroidism http://www.homestead.com/emguidemaps/files/myopathy.html (3 of 13)8/20/2004 5:14:27 PM myopathy - neuromuscular findings in hypothyroidism include proximal muscle weakness, muscle stiffness and cramping, muscle hypertrophy, slow reflexes and myoedema (* myoedema is the phenomenon of rounding up of muscle tissue after light percussion) - lipid-lowering drugs may exacerbate hypothyroidic myopathy - the CK may be elevated up to 10X in hypothyroidic myopathy Hyperthyroidism - neuromuscular findings include proximal muscle weakness with atrophy, brisk reflexes, and bulbar weakness - respiratory muscle weakness and respiratory failure is rare - there may be some clinical overlap with myasthenia gravis, and medical treatement of the thyroid disease may exacerbate the myasthenia - thyrotoxic hypokalemic paralysis is a rare phenomenon in Asian patients in their third decade Hypoparathyroidism - usually produces muscle tetany due to hypocalemia - a myopathy rarely occurs Hyperparathyroidism - a proximal muscle weakness associated with easy fatigueability, atrophy and hyperrflexia may occur Cushings disease - usually produces an insidious onset of proximal muscle weakness and atrophy and myalgia - iatrogenic steroid administration may produce a similar picture, and high-dose steroids may rarely produce quadriplegia and respiratory failure http://www.homestead.com/emguidemaps/files/myopathy.html (4 of 13)8/20/2004 5:14:27 PM myopathy Toxic myopathy - there are many causes of a toxic myopathy, and one system of classifying toxic myopathy is as follows:- Painless myopathies ● alcohol (chronic) ● steroids ● myoglobulinuria (CNS depressants, CNS stimulants, carbon monoxide, cyanide, arsenic, snake venoms) ● hypokalemia (licorice, carbenoxolone, amphotericin B, toluene, diuretics, alcohol) ● others (chloroquine, quinacrine, pancuronium, vecuronium, amiodarone, perhexiline, colchicine, vincristine) Painful myopathies ● D penicillamine ● procainamide ● phenytoin ● levodopa ● cimetidine ● leuprolide ● propylthiouracil ● zidovudine ● germanium ● others (alcohol, clofibrate, gemfibrizol, lovastatin, simvastatin, epsilon aminocaproic acid, etretinate, isotretinion, hypervitaminosis E, ipecac, emetine, organophosphates, toxic oil syndrome) Drugs of abuse ● alcohol ● amphetamines ● cocaine ● heroin ● phencyclidine ● toluene and gasoline - the CNS sedatives produce a painless myoglobulinuric myopathy due to over-sedation and immmobilization resulting in pressure necrosis http://www.homestead.com/emguidemaps/files/myopathy.html (5 of 13)8/20/2004 5:14:27 PM myopathy Periodic paralysis - there are many different types based on varying genetic abnormalities of the sodium, calcium or potassium channel - this basic outline is a summary of some of the major types, which are most likely to produce severe episodes of muscle weakness Hypokalemic periodic paralysis - due to a disorder of voltage-gated calcium (Ca2+) channel gene, CACNL1A3, chromosome 1q - often runs in families (2/3 of cases) - develops in early childhood and always < 30 years of age (otherwise consider thyrotoxic hypokalemic periodic paralysis or secondary hypokalemia-induced paralysis) - attacks often occur during sleep in the early morning hours => on awakening the patient may have profound weakness and be unable to get out of bed - attacks may be precipitated by a "trigger" on the preceding day - triggers include cold, strenuous exercise followed by rest, large carbohydrate meal - proximal limbs and truncal musculature weak, cranial nerves and respiratory muscles usually spared - attack lasts hours-days; usually 3 - 6 hours - the serum potassium is usually low during attacks; however, the serum potassium may be normal - over the years, in the absence of treatment, weakness may develop between attacks - treatment in the ED should be oral potassium; avoiding IV potassium or IV glucose solutions Thyrotoxic hypokalemic paralysis - male predominance (80 - 95%) http://www.homestead.com/emguidemaps/files/myopathy.html (6 of 13)8/20/2004 5:14:27 PM myopathy - onset 20 - 40 years - proximal weakness > distal weakness, legs > arms; weakness may selectively involve exercised muscles - severe attacks may involve respiratory and bulbar muscles - attacks last hours-days - attacks often occur randomly without an obvious stimulus; attacks