What Is Bornholm Disease;Diagnosis,Treatment And Pathology

Bornholm disease is a specific acute infectious disease of viral etiology, characterized by the sudden onset of severe abdominal and/or chest pain, fever, and headache. It occurs most often in the summer or early autumn, frequently in outbreaks, but may also occur sporadically.

History.

Numerous outbreaks of this disease were described in early medical literature. Windorfer relates that Haimaeus described an outbreak occurring in Schleswig-Holstein in 1732. In 1872, Daae and Homann each reported an epidemic in Drangedal, Norway. Two years later Finsen, using the term pleuro­dynia, described outbreaks in 1856 and 1865 in Iceland. In 1888, Dabney provided the first description of such an outbreak in the United States; a patient described the severe chest pain as the “devil’s grip”—a term subsequently applied on occasion to designate this disease.

Hanger, McCoy, and Frantz reported 16 cases in 1923 and called the syndrome epidemic pleurodynia. Sylvest in 1930 described an outbreak on Bornholm, a Danish island in the Baltic; in 1933 he wrote a classic monograph “Epidemic Myalgia,” in which he reviewed the world literature and presented detailed case histories of 93 patients with the disease in Bornholm and Copenhagen; the designation Bornholm disease was frequently applied following Sylvester’s descriptions.

Etiology.

The Coxsackie B viruses are recog­nized as the etiologic agents of epidemic pleuro­dynia or Bornholm disease. In 1949 Curnen, Shaw, and Melnick recovered a Coxsackie B virus from the feces of a 14-year-old boy with acute pleuro­dynia; in addition, they reported the occurrence of illnesses resembling pleurodynia in several laboratory personnel working with this virus.Subsequently, numerous studies appeared re­lating temporally epidemic pleurodynia and Cox­sackie B virus infections

Epidemiology.

Outbreaks of Bornholm disease have been reported from most parts of the world. It is  noteworthy that they generally occur in the summer and early autumn. Although all age groups can be affected, children and young adults experience the highest incidence. Intrafamilial spread is common, multiple cases in a single family being observed frequently; however, ab­sence of intrafamilial spread should not exclude the diagnosis. In one large study, about one third of the patients developed the illness as single cases in a household . Although both large outbreaks and sporadic cases have been described, epidemic pleurodynia is not a common disease in that it is not generally prevalent each summer and early fall, but rather occurs in sharp outbreaks confined to geographically limited areas. The mode of transmission is probably by person-to-person contact, with an incubation period of about two to five days.

Pathology

The specific pathologic manifesta­tions in humans are unknown as no deaths have been reported.

 

Clinical Manifestations of Bornholm Disease

Epidemic pleurodynia is characterized typically by the sudden onset of abdominal and/or chest pain, fever, and headache. The spasmodic abdominal or chest pain which may range from mild to extremely severe and which is often aggravated by respiration and movement is the most characteristic feature. In a report on 22 patients with epidemic pleurodynia who had a mean age of 25 years, Huebner et al. described the sensation of difficult breathing as particularly striking, and cited some characteristic descrip­tions of this symptom: “I can’t breathe,” “pain cut off my breath,” “can’t get a real long breath,” and “it hurts to breathe.”

In a study of 114 patients hospitalized with epidemic pleurodynia in Boston, Finn et al. reported that the typical severe paroxys­mal pleuritic type of pain was often described as “smothering,” “stabbing,” “knifelike,” “catching,” and “like a vise around the lower ribs.” It is note­worthy that, although the onset of pain is typically the initial symptom, in the Boston study about one quarter of the patients had prodromal symptoms one to ten days before the onset of pain  symptoms such as “head cold,” headache, anorexia, and myalgia.

Review of several outbreaks reveals that the location of the characteristic pain in infants and young children tends to be more abdominal than thoracic, whereas in older children and adults it tends to be more thoracic than abdominal. For example, in the 1949 Boston study in which almost three quarters of the patients were between 10 and 30 years of age, the characteristic pain was located in the chest alone in 48 per cent, in the chest and abdomen in 37 per cent, and in the abdomen alone in 14 per cent.

In contrast, in a 1953 Birmingham, England, study of 104 hospitalized children whose average age was 5V2 years, the characteristic pain was located in the abdomen alone in 81 percent, in the abdomen and chest in 11 per cent, and in the chest alone in 9 per cent (Disney et al.). Similar findings were reported in a South African outbreak in which 32 (80 per cent) of 40 children had abdominal pain alone, 4 both abdominal and chest pain, and 4 chest pain alone.

In the Boston study the chest pain was located tVve lower ribs and for varying distances up the chest, usually on the lateral aspect but not in­frequently over the front and back” except in six patients whose thoracic pain was limited to the substernal region only; about one third of patients with chest pain experienced referral of such pain to one or both shoulders, one or both scapulae, the interscapular region, or the neck. Among those with abdominal pain, approximately three quart­ers experienced epigastric or upper abdominal pain; it was noteworthy that in two patients the site of pain was in the right lower quadrant.

Other symptoms among the total group included moder­ately severe or severe headache (44 per cent), cough (33 per cent), anorexia (26 per cent), nausea (24 per cent), chilly sensations (21 per cent), Chills (18 per cent), “head cold” (16 per cent), vomiting (16 per cent), sore throat (12 per cent), and diarrhea (7 per cent). Physical examination re­vealed that 95 per cent were febrile (99 to 104° F.) with a mean temperature of 101° F.; the fever lasted a mean of 3V2 days with a range of 1 to 14 days. Recrudescences of fever after the tempera­ture had returned to normal or nearly normal were common.

The pulse rate was proportional to the temperature. Visible splinting of the chest was observed commonly, especially during parox­ysms of pain. In addition, a pleural friction rub confined to the lower half of the chest was heard in about one quarter of all cases. Localized tender­ness to pressure was found in about one quarter of cases and was usually confined to those areas of the chest where pain was present.

Tenderness con­fined to the abdomen (in 29 per cent), or tender­ness in both abdomen and chest (in 9 per cent) were the most common abdominal findings, al­though “splinting and rigidity of the upper ab­dominal area were not infrequently present, espe­cially when the pain was severe” (Finn et al.). In the Birmingham children’s study, generalized ab­dominal tenderness was present in 34 per cent and right iliac fossa tenderness in 13 per cent (Disney et al., 1953). Roentgenographic examinations of the chest have revealed no characteristic abnor­malities. The white blood cell count is usually within normal limits; occasionally a moderate leukopenia, leukocytosis, or eosinophilia may be present. The majority of patients are well within one week of onset, but longer periods of illness are not unusual. It is noteworthy that not infrequently relapses may occur a few days or more than one month after recovery.

Complications.

Complications of epidemic pleurodynia occur relatively infrequently and include orchitis, aseptic meningitis, and peri­carditis. Cases of Coxsackie B myocarditis of new­born infants have occasionally been reported during outbreaks of epidemic pleurodynia. It is noteworthy, however, that myocarditis has only very rarely been reported as a complication in a patient with epidemic pleurodynia; however, the possibility should be 

borne in mind.

Diagnosis of Bornholm Disease

A clinical diagnosis is usually not difficult once the presence of an epidemic is known. However, during the early stages of an outbreak or when a sporadic case is encountered, it may be quite difficult, if not impossible, to make the correct diagnosis. A vivid illustration of this difficulty is provided by a children’s hospital study in which it was found that, until the house staff and other medical personnel in the community became aware that a pleurodynia outbreak was occurring, admission diagnoses on children even­tually shown to have epidemic pleurodynia in­cluded the following: acute abdomen, possible appendicitis, possible duodenal ulcer, pyelone­phritis, pneumonia, pleurisy with pneumonia, rheumatic fever, pain of unknown origin, trauma (fractured rib), myositis (influenzal), collagen dis­ease, tuberculosis, and intussusception  Symptoms of common duct obstruction, pancreatitis, coronary occlusion, and intestinal in­fections may also be mimicked by epidemic pleurodynia.

Laboratory Finding

Laboratory diagnosis of Coxsackie B infection is made by isolation of the virus from throat washings or stools during the early phase of illness and by demonstrating a rise in neutralizing antibody against one of the Cox­sackie B viruses in acute and convalescent phase sera. Unfortunately results of such tests become available too late to be of assistance in making a diagnosis in the acute case.

Treatment and Prognosis.

There is no specific therapy for the Coxsackie virus infection. Treat­ment is supportive and symptomatic. Recovery is usually complete and follows after variable periods as noted above. However, some patients may ex­perience lingering after effects of tiredness and weakness, with a gradual return to normal health.

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