rhabdomyolysis in retrospect - The International Journal of
Transcription
rhabdomyolysis in retrospect - The International Journal of
INT’L. J. PSYCHIATRY IN MEDICINE, Vol. 17(2), 1987 RHABDOMYOLYSIS IN RETROSPECT: ARE PSYCHIATRIC PATIENTS PREDISPOSED TO THIS LITTLE-KNOWN SYNDROME? STEVEN B. JOHNSON, M.D. Department of Psychiatry and Human Behavior University of California lrvine Medical Center WILLIAM A. ALVAREZ, M.D. Department of Psychiatry and Human Behavior California College of Medicine- University of California, lrvine JACK P. FREINHAR, M.D. Department of Psychiatry and Human Behavior University of California lrvine Medical Center ABSTRACT Rhabdomyolysis is a potentially lethal syndrome that follows skeletal muscle injury, both traumatic and nontraumatic. The literature on this syndrome remains sketchy, and rhabdomyolysis may often go unrecognized. The history, clinical presentation, laboratory findings, etiology, and treatment of rhabdomyolysis axe reviewed. Factors which predispose psychiatric patients in particular to this syndrome are discussed. Recommendations to reduce morbidity and mortality axe offered. Rhabdomyolysis is a potentially lethal syndrome characterized by muscle pain, weakness, and dark red or brown urine following skeletal muscle injury [ I ] . Acute renal failure, severe hemorrhage, and disseminated intravascular coagulation are common complications [ l ] . Involved muscles may be tender and feel “doughy” with hemorrhagic discoloration of overlying skin [ 11 . However, edema can develop, especially after intravenous fluid administration, and muscles may therefore feel firm and swollen instead [ l , 21. Edematous 163 0 1987,Baywood Publishing Co., Inc. doi: 10.2190/PW4M-BQ2X-0FT2-J6ML http://baywood.com 164 / S. 0. JOHNSON, W. A. ALVAREZ AND J. P. FREINHAR compartmental compression of nerves and arteries can cause weakened foot pulses, paresthesias, foot drop, and absent deep tendon reflexes [ 1, 31 . Information on rhabdomyolysis remains incomplete and confined mostly to scattered case reports. Rhabdomyolysis ma.y go unrecognized, at least in psychiatric patients, on a regular basis [4]. No specific numerical values for its incidence are available. Rhabdomyolysis has been variously called “rare” [5J , “not an uncommon disorder” [2] , and “exceptionally common” [ 11 . The incidence of mortality associated with rhabdomyolysis is likewise not known, but among seventy-seven patients involved in eighty-seven episodes of rhabdomyolysis studied by Gabow et al., eight died [2]. Injury can trigger the liberation of skeletal muscle intracellular contents like creatine phosphokinase (CPK), myoglobin, aldolase, potassium, phosphate, purines, lactate dehydrogenase, and glutamic oxaloacetic transaminase [2] . Their release into the circulation is reflected by subsequent elevated serum CPK, myoglobinuria, hyperkalemia, and hyperphosphatemia [ 1 J . Acid-base derangements (e.g., lactic acidosis), proteinuria, fibrin split products in serum and/or urine, hypofibrinogenemia, thrombocytopenia, and prolongation of prothrombin time are other laboratory features seen in rhabdomyolysis [ 1 , 2 ] . In addition, the conversion of muscle creatine to creatinine in the circulation may cause the blood urea nitrogen to creatinine ratio to fall to less than 10: 1 [l , 3 , 6 ] . Hyperuricemia due to the catabolism of escaped muscle purines may be observed, too 131. By the second to fourth day after the onset of rhabdomyolysis, the deposition of calcium salts in damaged muscle tissue may lead to hypocalcemia [ 11 . Hypoalbuminemia can also develop a few days after severe rhabdomyolysis, evidently because capillary injury allows the escape of albumin from plasma [ 11. Myoglobin is cleared from plasma in one to six hours by metabolism to bilirubin and, if plasma levels exceed 1.5 mg/dl, by renal excretion [2]. Myoglobin in the urine can be confirmed by a positive orthotolidine dipstick test, in the absence of hematuria (this “occult blood” test merely detects heme and thus does not distinguish between hemoglobin and myoglobin) [ 1, 21 . Urine myoglobin concentrations above 100 mg/dl lead to red or brown discoloration of the urine [2] . The MM, or type 111, isoenzyme of CPK is the most sensitive marker of skeletal muscle injury severity [2]. CPK levels peak about twenty-four hours after muscle injury and decline approximately 39 percent each day thereafter [2] .Peak CPK values in mild cases of rhabdomyolysis will be 500-5,000 IU/L (normal is less than 86) [l J . CPK levels in the vicinity of 100,000 IU/L are common in more severe cases [ 11 . Johnson et al. report a CPK value of 914,000 IU/L in a schizophrenic man who developed rhabdomyolysis following molindone administration [7 J . The causes of rhabdomyolysis are legion and may include all of the following: 1) excessive muscular contraction associated with strenuous exercise, seizures, RHABDOMYOLYSIS IN RETROSPECT I 165 delirium tremens, status asthmaticus, malignant hyperthermia, neuroleptic malignant syndrome (NMS), dystonias, psychotic posturing, or Wilson’s disease; 2) temperature extremes, i.e., heat stroke and hypothermia; 3) direct muscle trauma due to crush or compression injury, burns, electric shock, lightning, or intramuscular injection; 4) exposure to perphenazine, loxapine, molindone , amoxapine, amitriptyline, lorazepam, diazepam glutethimide, codeine, methadone, heroin, amphetamines, phencyclidine (PCP), lysergic acid diethylamide (LSD), barbiturates, toluene (paint sniffing), ethanol, isopropyl alcohol, ethylene glycol, salicylate, mercuric chloride, phenylpropranolamine, diphenhydramine, epsilon aminocaproic acid, succinylcholine, propoxyphene, carbenoxolone, clofibrate, carbon monoxide, licorice, snake or brown spider venom, hornet or wasp venom, fish flesh (Haff disease), or quail meat (hemlock?); 5) immunological disorders such as dermatomyositis and polymyositis; 6 ) genetic disorders, including muscular dystrophies and lipid or glycogen storage/metabolism enzyme deficiencies, e.g., carnitine palmityltransferase deficiency; 7) muscle ischemia arising from compression, shock, sickle cell trait, embolism, or immobilization in one position; 8) infections like tetanus, Legionnaire’s disease, pyomyositis, influenza, mononucleosis, Rocky Mountain Spotted Fever, gas gangrene, leptospirosis, coxsackie, and shigellosis; 9) metabolic disorders such as diabetes mellitus and myxedema; and 10) electrolyte imbalances, including hypokalemia, hypernatremia, hyponatremia, and hypophosphatemia [2,3,5-181. Figure 1 summarizes these causes of rhabdomyolysis. Probably the first reported cases of rhabdomyolysis were the ancient Israelites, as described in the Bible (Numbers, xi), who died after feasting on quail during their exodus from Egypt 1191. Quail consumption has led to myoglobinuric acute renal failure in several modern cases in Algeria and Greece [ 18-20] . The hemlock (Conium maculatum) seeds on which quail occasionally feed may be responsible for the rhabdomyolysis associated with quail poisoning [ 181 . The clinical triad of muscle pain, weakness, and dark urine was described by Meyer-Betz in 191 1 [21], and was formerly called Meyer-Betz disease [ l ] . The term “myoglobinuria” is sometimes used as if it were synonymous with this syndrome. However, myoglobinuria is only one feature of this syndrome. The term “rhabdomyolysis” on the other hand refers to the entirety of the described syndrome. Furthermore, while rhabdomyolysis can occur without myoglobinuria, myoglobinuria cannot occur in the absence of rhabdomyolysis [ 1 , 2 ] . “Rhabdomyolysis” is thus the more specific and the preferred term for this syndrome [ 1 , 2 ] . Beall and Bywaters reported acute renal failure among London air raid casualties who incurred crush injuries during the Battle of Britain in 1941 [22]. As many as 33 percent of the rhabdomyolysis victims may develop acute renal failure [2] . The pathogenesis of renal failure associated with rhabdomyolysis 166 I S. 6. JOHNSON, W. A. ALVAREZ AND .J. P. FREINHAR CXCESSIVE MUSCULAR ACTIVITY (EXERCISE, SEIZURES PSYCHOSIS) HY PERTHERMIA/ HEAT STROKE IMRAVASCULAR COAGULATION 1 PRIMARY MUSCLE INJURY DRUGSITOXINS IMMUNOLOGICAL DISORDERS RHABDOMYOLYSS LAC1Ic AC IDOSIS (DERM*TOMYOSITIS) HYPERURICEMIA GENETIC DISORDERS MUSCLE ISCHEMIA INFECTIONS MElABOLlC DERANGEMENTS I I- FALURE I J HYPERURICOSURI ELECTROLYTE DISORDERS Figure 1. Rhabdomyolysis and acute renal failure. remains unclear. Hypovolemia, myoglobinuria, intravascular coagulation, acidosis, and hyperuricosuria appear to be causative, either singly or in some combination [23]. Hypovolemia is apparently not directly nephrotoxic; instead, it seems to potentiate the nephrotoxic effects of other factors like myoglobinuria by concentrating the urine [ 1, 171. Figure 1 depicts the relationship between rhabdomyolysis and acute renal failure. PSYCHIAT R IC IMPLICATIONS Psychiatric patients, especially those who are psychotic or in seclusion, are often less able to communicate distressing symptoms to staff and less able to cooperate with physical examinations and diagnostic tests. For various other reasons, it may prove difficult to rigorously monitor psychiatric patients' physical condition, vital signs, and laboratory data. The diagnosis of rhabdomyolysis in psychiatric patients can therefore be delayed, thus increasing the risk of mortality. Several factors predispose psychiatric patients in particular to developing both rhabdomyolysis and renal complications [4, 241. For example, when RHABDOMYOLYSIS IN RETROSPECT I 167 psychiatric patients refuse oral medication, they may be subjected to intramuscular injections, leading to muscle necrosis and possible rhabdomyolysis v11. Psychiatric patients are also placed at high risk by erratic oral intake, a not uncommon manifestation of psychopathology. Fasting seems to encourage the development of exertional rhabdomyolysis [l] , and, as mentioned above, dehydration contributes to the development of acute renal failure [4]. Neuroleptics can cause dystonias and NMS, and both in turn can cause rhabdomyolysis [5,9,161. In addition, restraint and seclusion can contribute to the development of rhabdomyolysis if the patient is immobilized in one position for some time or if the patient struggles vigorously against the restraints [14, 151 . Psychosis itself predisposes patients to rhabdomyolysis as described by Coryell et al., who reported a case of rhabdomyolysis and acute renal failure in a psychotic former college football player who assumed a rigid three-point stance for an hour [8] . Similarly, Finlayson et al. reported a middle-aged psychotic woman who developed rhabdomyolysis because she was unable to free herself or call out for help after becoming trapped in a Murphy bed [4] . Acutely psychotic, drug-free patients are known to have increased histochemical abnormalities of a myopathic type and elevated serum levels of skeletal muscle enzymes such as CPK and aldolase [25, 261. CPK elevations associated with psychosis are generally two- or threefold greater than normal but can be as high as 100,000 IU/L [ 111 . Such myopathies and enzyme elevations suggest that acute psychosis is associated with increased skeletal muscle membrane permeability [27] . Many substances abused by psychiatric patients-alcohol, LSD, PCP, heroin, amphetamines, and barbiturates-may cause rhabdomyolysis [2, 141 . Many of the psychotropic medications used to treat psychiatric patients-loxapine, perphenazine, molindone, amoxapine, amitriptyline, lorazepam, diazepam, glutethimide, and methadone-have also been implicated as causes of this syndrome [2, 7, 14, 171. Together, these represent nearly every class of psychoactive agent: neuroleptics, antidepressants, benzodiazepines, barbiturates, amphetamines, opiates, hallucinogens, and alcohol.\ Alcohol, the most frequent cause of rhabdomyolysis, is directly myotoxic [2]. It is not clear however how other psychoactive agents cause rhabdomyolysis. It has been suggested that at least amoxapine is also directly myotoxic [ 171. However, reports of rhabdomyolysis associated with psychoactive agents other than alcohol remain relatively rare despite the epidemic abuse of illicit drugs and the liberal use of licit psychotropics (for instance, in 1976, 128 million prescriptions were written just for sedative-hypnotics in the United States alone [28]). Perhaps the psychoactive agents other than alcohol are myotoxic only in certain predisposed patients, just as inhalational anesthetics trigger malignant hyperthermia in only rare, susceptible individuals. Or perhaps they are not 168 / S. B. JOHNSON, W. A. ALVAREZ AND J. P. FREINHAR myotoxic enough to cause rhabdomyolysis unless they are taken in massive doses or in the presence of other factors. In their study of eighty-seven episodes of rhabdomyolysis, Gabow et al. identified multiple factors capable of injuring muscle in fifty-one cases [2]. Ralph suggests, “Multiple mechanisms of injury may contribute to rhabdomyolysis, e.g., ischemia, acidosis and trauma with Severe exercise” [ 6 ] .In our own review of the literature, most case reports of rhabdomyolysis associated with psychoactive agents involved at least one other factor capable of injuring muscle: psychosis, catatonic posturing, repeated intramuscular injections, dystonias, seizures, NMS, immobilization in and exertion against restraints, or erratic oral intake [5,7,8-11, 151. As stated before, psychosis apparently increases muscle membrane permeability as indicated by elevated aldolase and CPK. Both NMS and intramuscular injection are also associated with moderate increases in serum CPK [1 11,which, again, might represent a disturbance of muscle membrane integrity. Elevated serum CPK levels have been used to screen patients for susceptiblility to malignant hyperthermia [29] (ths practice has been challenged by Paasuke and Brownell [30]). In malignant hyperthermia, the triggering anesthetic blocks the reentry of calcium into the sarcoplasmic reticulum, leading to cell membrane depolarization [311 . Intracellular ions such as potassium, magnesium, and phosphate then escape into the plasma, followed by larger molecules like myoglobin [3 11 . NMS is thought to be related to malignant hyperthermia [lo] and may injure muscle membranes by depolarization, too. It thus appears that at least several disordels reported to cause rhabdomyolysis may do so by disrupting skeletal muscle membrane integrity, either in a direct, physically traumatic manner like intramuscular injection or by somehow depolarizing the membrane. Presumably, one factor acting alone produces a mild to moderate membrane disturbance and thus a mild to moderate CPK elevation, as often seen in NMS, intramuscular injection, and psychosis. If the offending factor is unusually traumatic (e.g., drug overdose), then the muscle membrane permeability is more severely disturbed. When the cumulative effect of several factors or, less commonly, the effect of one very traumatic event exceeds some threshold of permeability, enough muscle contents are released to constitute rhabdomyolysis. This theory would account for 1) the preponderance of cases involving multiple etiologic factors, and 2) the rarity or absence of cases arising from routine neuroleptic doses and non-alcohol drug abuse, excluding overdoses. Research is needed to resolve this question of how psychoactive agents and rhabdomyolysis are related. RECOMMENDATIONS Rhabdomyolysis and accompanying complications like acute renal failure and severe hemorrhage are potentially lethal. Early detection and treatment can reduce morbidity and mortality [ 6 , 24,]. Patients, psychiatric or otherwise, who are exposed to one or more rhabdomyolysis-predisposingfactors (e.g., alcohol RHABDOMYOLYSIS IN RETROSPECT / 169 or drug abuse, high doses of psychotropic medication, overdosage of any psychoactive drug, psychosis, catatonic posturing, intramuscular injection, dystonia, NMS, seclusion and restraint, seizures, or malnutrition) merit periodic evaluation for muscle tenderness, weakness, and dark red or brown urine. It should be remembered, however, that this classic triad is not always clinically observed. For example, in the aforementioned study by Gabow et al., half of the patients experienced no myalgias, and only four of the eighty-seven episodes involved muscle swelling [ 2 ] . Less than half of rhabdomyolysis victims have dark urine [2]. Thus this screening interview is useful as an inexpensive tool for large populations, but any suspicion should be followed by appropriate laboratory tests. The presence of both myoglobinuria and elevated serum levels of skeletal muscle contents, particularly CPK, will confirm the diagnosis [ 1 ] . A fivefold or greater increase in serum CPK is, by itself, diagnostic if cardiac and brain injury have been ruled out [2]. Because drug overdose by definition implies a massive dose, often of more than one drug, we recommend serial assessments of serum CPK and urine myoglobin in all patients suspected of psychoactive drug overdose. A state of euhydration and adequate nutrition should be maintained in patients taking psychotropic medication. Initial treatment of rhabdomyolysis consists of 1) prompt fluid replacement to counter volume depletion and 2 ) mannitol to further promote volume expansion and renal perfusion [3]. Alkalinization of the urine with bicarbonate to increase myoglobin excretion may aggravate hypocalcemia [ 1] . Oral phosphate-binding antacids are generally sufficient to treat hyperphosphatemia [l ] . Hyperkalemia, an occasionally fatal complication, will eventually resolve with normal renal perfusion and excretion; in the presence of renal failure, treatment with dialysis or exchange resins may prove necessary [6]. Nerve damage presumably precedes signs like paresthesias and absent tendon reflexes. Pressure transducers can be used to monitor interstitial pressure in muscle compartments, especially the anterior tibia1 muscles, the soleus, the lateral muscles’of the thigh, and the glutei [ I ] . Fasciotomy is recommended if pressure exceeds 35 mm Hg [ I ] . Furosemide may worsen volume depletion and should not be used [6]. Hypocalcemia usually resolves spontaneously after several days; furthermore, the administration of calcium salts may actually promote further muscle damage and is thus contraindicated [ 11 . Disseminated intravascular coagulation complicating rhabdomyolysis is often accompanied by severe hemorrhage, so herapin administration is controversial [ I ] . ACKNOWLEDGMENT The authors thank Mildred D. Brown and Catherine D. Wheeler for typing this manuscript. 170 / S. 6. JOHNSON, W. A. ALVAREZ AND J. P. FREINHAR REFERENCES 1. J. P. Knochel, Rhabdomyolysis and Myoglobinuria, Annual Review of Medicine, 33, pp. 435-443, 1982. 2. P. A. Gabow, W. D. Kaehny, and S. P. Kelleher, The Spectrum of Rhabdomyolysis, Medicine, 61, pp. 141-152, 1982. 3. D. W. Martin, H. D. Watts, and L. H. Smith, Rhabdomyolysis-Medical Staff Conference, University of California, San Francisco, Western Journal of Medicine, 125, pp. 298-304, 1976. 4. R. E. Finlayson and J. J. Cavanaugh, Drs. Finlayson and Cavanaugh Reply, Journal of Clinical Psychiatry, 46, p. 406, 1985. 5. J. J. Cavanaugh and R. E. Finlayson, Rhabdomyolysis Due to Acute Dystonic Reaction to Antipsychotic Drugs, Journal of Clinical Psychiatry, 45, pp. 356-357, 1984. 6. D. Ralph, Rhabdomyolysis and Acute R.enal Failure, JACEP, 7 , pp. 103-106, 1978. 7. S. B. Johnson, W. A. Alvarez, and J. P. 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Mertz, Defect of Intracellular Calcium Channels is Culprit in Malignant Hyperthermia, Journal of the American Medical Association, 255, pp. 710-711, 1986. Direct reprint requests to: Steven B. Johnson, M.D. Department of Psychiatry and Human Behavior University of California Irvine Medical Center 101 The City Drive South Orange, CA 92668