Portal de Periódicos da CAPES

Tetanus: An Uncommon Cause of Dysphagia

1989; Elsevier BV; Volume: 64; Issue: 3 Linguagem: Inglês

10.1016/s0025-6196(12)65253-7

1942-5546

DAVID G. SCHOLZ, JEANETTE M. OLSON, DELORAN L. THURBER, David E. Larson,

Pleural and Pulmonary Diseases

A 53-year-old woman was examined at our medical center because of progressive dysphagia of 14 days' duration and a severe inability to open her mouth and swallow saliva. A barium esophagogram showed no obstruction, but pooling of barium in the hypopharynx suggested a neuromuscular disorder. The clinical diagnosis of tetanus was confirmed by electromyography. With appropriate therapy, the patient recovered during a period of 6 weeks. This case illustrates both an uncommon cause of dysphagia and an uncommon initial manifestation of tetanus. A 53-year-old woman was examined at our medical center because of progressive dysphagia of 14 days' duration and a severe inability to open her mouth and swallow saliva. A barium esophagogram showed no obstruction, but pooling of barium in the hypopharynx suggested a neuromuscular disorder. The clinical diagnosis of tetanus was confirmed by electromyography. With appropriate therapy, the patient recovered during a period of 6 weeks. This case illustrates both an uncommon cause of dysphagia and an uncommon initial manifestation of tetanus. The clinical signs and symptoms of tetanus can be either generalized or localized, and the disorder may manifest as a fulminating, life-threatening illness or as a less severe condition. During the past 2 decades, the incidence of tetanus in the United States has declined considerably; the cumulative incidence reported to the Centers for Disease Control in 1987 was only 39 cases.1Centers for Disease Control Notifiable diseases of low frequency, United States (Table II).MMWR. 1988; 36: 826Google Scholar Because of the low incidence, the wide spectrum of clinical manifestations, and the paucity of diagnostic tests, tetanus may be difficult to diagnose, and, therefore, initiation of appropriate therapy may be delayed. A 53-year-old woman was transferred to our medical center for evaluation and treatment of dysphagia of acute onset. She had been in good health until 14 days previously, when she noted mild difficulty in swallowing her evening meal. During the next 48 hours, she experienced progressive difficulty in swallowing both solids and liquids and was admitted to her local hospital for assessment. The general physical examination at that time was unremarkable except for signs of dehydration. A barium study of the esophagus revealed no evidence of obstruction. Results of a Tensilon test, magnetic resonance imaging of the head, and a test for acetylcholine receptor antibodies were normal. An otolaryngologist noted only edema and friability below the cricopharyngeus. While hospitalized, the patient experienced difficulty in opening her mouth and in swallowing saliva. A nasojejunal feeding tube was placed, and a regimen of dexamethasone (Decadron) and amitriptyline hydrochloride was begun. On admission to a hospital affiliated with our institution 14 days after the onset of dysphagia, the patient was noted to be a well-nourished woman who had difficulty swallowing saliva. The following vital signs were recorded: blood pressure, 140/80 mm Hg; pulse rate, regular at 60 beats/min; respiratory rate, 20 breaths/min; and temperature, 37.5°C. Physical examination disclosed no visible signs of trauma. The platysma muscle was noticeably contracted, however, and the patient was able to open her mouth only 6 mm (measured between the incisors). She spoke in a soft, hoarse voice. The remainder of the physical examination was unremarkable, and she had no recollection of any previous tetanus immunization, recent illness, or injury. Results of routine laboratory studies and chest roentgenography were normal. A repeat barium esophagogram (accomplished by filling the oropharynx with use of a syringe because of the patient's inability to open her mouth) showed normal elevation of the tongue and soft palate and no evidence of obstruction. A small amount of barium was aspirated during swallowing. Some pooling of barium was noted in the hypopharynx, as well as evidence of asymmetry in that area on swallowing, but no distal esophageal abnormalities were observed (Fig. 1). The hypopharyngeal abnormalities were not believed to be related to the presence of the nasojejunal feeding tube. An otolaryngologic evaluation, including laryngoscopy, revealed normal findings. Although a neurologist believed that the patient exhibited evidence of trismus, no specific neurologic deficits were detected. The results of the radiologic studies and the clinical manifestations were consistent with a diagnosis of tetanus. The diagnosis was confirmed by electromyography, which demonstrated that firing of bulbar motor units could not be inhibited by activation of antagonistic muscles and that the masseter muscle silent period was abnormal when the jaw reflex was tested (Fig. 2). The patient was treated with 2,000 units of human tetanus immune globulin and a 10-day course of penicillin and gentamicin, tetanus toxoid, and diazepam, in addition to enteral feedings. No other signs of tetanus developed, and the patient was dismissed from the hospital as soon as she was able to accomplish her tube feedings without assistance. After 3 weeks, salivary control was achieved, and 2 weeks later, the patient was able to discontinue her enteral feedings because the dysphagia had resolved completely. She has since been completely asymptomatic (17 months of follow-up). The clinical signs and symptoms of tetanus are caused by a potent neurotoxin produced by an anaerobic, spore-forming, gram-positive bacillus, Clostridium tetani, a common organism that can be found in the soil and in the intestinal tract of some animals and humans.2Weinstein L Tetanus.N Engl J Med. 1973; 289: 1293-1296Crossref PubMed Scopus (102) Google Scholar In its vegetative form, C. tetani produces two toxins, tetanolysin and tetanospasmin. Although the importance of tetanolysin in vivo is unclear, it may damage nearby tissue and thus reduce the local oxidative-reduction potential; hence, the local environment will promote bacterial replication. The potent toxin tetanospasmin most likely affects the release of the transmitter that normally inhibits firing of motor neurons. The result is increased resting tone of lower motor neurons and frequent muscle contractions.3Bleck TP Pharmacology of tetanus.Clin Neuropharmacol. 1986; 9: 103-120Crossref PubMed Scopus (42) Google Scholar These processes cause the clinical signs and symptoms of tetanus, which, in generalized tetanus, consist of contraction of muscle groups of the back, neck, thighs, and abdomen that produces spasms, stiffness, and pain. Trismus or difficulty opening the mouth is common and, together with facial spasms, produces a sneering expression known as risus sardonicus. Dysfunction of the sympathetic nervous system may also occur.2Weinstein L Tetanus.N Engl J Med. 1973; 289: 1293-1296Crossref PubMed Scopus (102) Google Scholar, 3Bleck TP Pharmacology of tetanus.Clin Neuropharmacol. 1986; 9: 103-120Crossref PubMed Scopus (42) Google Scholar, 4Christensen NA Thurber DL Clinical experience with tetanus: 91 cases.Proc Staff Meet Mayo Clin. 1957; 32: 146-158PubMed Google Scholar As our case illustrates, not all the “classic” signs of tetanus may be present initially; the diagnosis of tetanus was delayed in our patient because the only initial manifestation was dysphagia. In a 1957 review of 91 patients treated at our institution, Christensen and Thurber4Christensen NA Thurber DL Clinical experience with tetanus: 91 cases.Proc Staff Meet Mayo Clin. 1957; 32: 146-158PubMed Google Scholar noted initial dysphagia in only 9 (10%); however, dysphagia subsequently developed in 47 patients (52%). Wang and Karmody5Wang L Karmody CS Dysphagia as the presenting symptom of tetanus.Arch Otolaryngol. 1985; 111: 342-343Crossref PubMed Scopus (10) Google Scholar reported a case of progressive dysphagia due to tetanus that developed during a 14-day period in a patient who had no identifiable associated wound. Barium studies demonstrated complete obstruction at the level of the cricoid and spasm of the cricopharyngeal muscle. The patient had a sudden respiratory arrest 16 days after the onset of dysphagia but was resuscitated and recovered during a 10-week period. Watanabe and associates6Watanabe H Makishima K Arima T Mitsuyama S Dynamics of swallowing in tetanus.J Laryngol Otol. 1984; 98: 953-956Crossref PubMed Scopus (6) Google Scholar also reported a case of tetanus that manifested as progressive dysphagia about 14 days after injury to an upper extremity. Fluoroscopic examination showed that the patient could hold barium in his mouth but was unable to pass it into the esophagus and that some barium entered the trachea. Trismus, spasms of the extremities, and opisthotonos gradually developed, but the symptoms resolved after 7 days of treatment. His dysphagia occurred early in the second phase of deglutition, when the larynx ascended to close the epilarynx as a bolus was propelled from the oral cavity. The authors speculated that this case of dysphagia and tetanus differed from other reported cases, which often involve difficulty with mastication and the first phase of deglutition. Several other general points should be noted. First, the diagnosis of tetanus traditionally has been primarily clinical and one of exclusion as normal results of laboratory studies (blood chemistry, erythrocyte sedimentation rate, and analysis of cerebrospinal fluid) become available. Hematologic studies may reveal an elevation of the leukocyte count, although this abnormality is not uniformly observed. Although electromyographic studies are not diagnostic, they can confirm the diagnosis, as demonstrated in our case. Struppler and associates7Struppler A Struppler E Adams RD Local tetanus in man: its clinical and neurophysiological characteristics.Arch Neurol. 1963; 8: 162-178Crossref PubMed Scopus (47) Google Scholar characterized electromyographic features in localized tetanus as continuous activity during hypertonia and spasm, prolonged activity into periods of attempted relaxation, and failure of inhibition of activated motor neurons. The last-mentioned feature is highly characteristic of tetanus. During voluntary activity in normal persons, a tendon reflex (such as a jaw jerk) is followed by abrupt suppression of motor neurons. This phase of inhibition is called the silent period. In patients with tetanus, this inhibition does not occur and there is no silent period (Fig. 2). Second, according to statistics from the Centers for Disease Control for 1985 and 1986,8Centers for Disease Control Tetanus—United States, 1985–1986.MMWR. 1987; 36: 477-481PubMed Google Scholar only 99 of the 140 reported cases of tetanus in the United States (71%) were associated with an identified acute injury. Similarly, no focus of infection was identified in our patient. Third, according to the Centers for Disease Control, 71% of the 140 cases occurred in patients older than 50 years of age, and only 5% occurred in patients younger than age 20 years.8Centers for Disease Control Tetanus—United States, 1985–1986.MMWR. 1987; 36: 477-481PubMed Google Scholar Only nine of the patients (6%) had received at least a primary series of tetanus toxoid. Tetanus is most prevalent in the older population group because many of these persons have not been adequately immunized, as was the case with our patient. Persons in this age group should be urged to obtain and maintain immunizations. The prognosis for patients with tetanus is guarded and is related to the severity and rapidity of the onset of symptoms and to the availability of medical care. In a review by Singh and associates9Singh GP Sikka PK Gupta MM Tetanus—a method of scoring to determine the prognosis.Indian J Med Sci. 1986; 40: 124-128PubMed Google Scholar of 100 cases from India, the mortality rate was 68% if symptoms occurred after less than 5 days of incubation, whereas mortality rates declined to 51% and 21%, respectively, with incubation periods of 6 to 10 days and more than 10 days. If the time between the first symptom and the first spasm was less than 24 hours, the mortality rate was 71%. A primary principle of medical care after the diagnosis of tetanus is prevention of and preparation for progression of the disease. This treatment plan involves administration of antitoxin and enteral nutrition, management of airways, and close monitoring for sympathetic dysfunction. Although the toxin that has already been taken up by the neurons cannot be neutralized, free toxin can be bound and neutralized by human tetanus immune globulin. An intramuscularly administered dose of 3,000 to 5,000 units is recommended, although no minimal dose has been established. The evident wound should be débrided after antitoxin has been administered, but cultures are not usually helpful inasmuch as they are positive in only about 30% of cases. Penicillin effectively eradicates the remaining C. tetani, and treatment of associated aerobic organisms is often warranted. Because of its direct relationship to mortality in many cases of tetanus, appropriate management of airway obstruction is crucial. Benzodiazepines (most commonly diazepam) are helpful in decreasing neuromuscular symptoms of stiffness and spasm. Depending on the severity of the disease, other neuromuscular blocking agents such as pancuronium bromide (Pavulon) or d-tubocurarine may be lifesaving, along with mechanical ventilation. Sympathetic crises may require both α- and β-adrenergic blockade.2Weinstein L Tetanus.N Engl J Med. 1973; 289: 1293-1296Crossref PubMed Scopus (102) Google Scholar, 3Bleck TP Pharmacology of tetanus.Clin Neuropharmacol. 1986; 9: 103-120Crossref PubMed Scopus (42) Google Scholar, 4Christensen NA Thurber DL Clinical experience with tetanus: 91 cases.Proc Staff Meet Mayo Clin. 1957; 32: 146-158PubMed Google Scholar Recovery is variable. In a review by Edmondson and Flowers,10Edmondson RS Flowers MW Intensive care in tetanus: management, complications, and mortality in 100 cases.Br Med J. 1979; 1: 1401-1404Crossref PubMed Scopus (115) Google Scholar the mean duration of paralysis was 21 days, and some mild symptoms lasted 3 to 4 weeks. Tetanus is much less common in the United States than in Third-World countries. Regardless of the initial manifestations, prevention of and preparation for progression of the disease are the primary objectives. In the United States, tetanus is most likely to occar in unimmunized patients older than 50 years of age, and an identifiable associated wound may not be found. Our case illustrates that tetanus should also be considered in the differential diagnosis of dysphagia. Certainly, early diagnosis and therapy should minimize exposure to toxins and the resultant effects.