Bornholm Disease

Synonyms: epidemic myalgia, epidemic pleurodynia, Sylvest's disease, Bamble disease and Devil's Grip.

Bornholm disease is a viral myalgia causing pain in the lower chest and upper abdomen, which can be severe. (The synonym pleurodynia is misleading, as it implies that the pathology lies in the pleural lining of the lungs, whereas the pain probably originates from the muscles.)

The disease is usually caused by a Coxsackie group B virus;¹ rarely by coxsackie A or echoviruses.² All these belong to the group of enterovirus infections.

The disease has occurred in epidemics - in temperate climates these tend to be in spring and autumn. Bornholm disease mainly affects children and young adults. Coxsackie B virus infections are common and can cause a range of conditions from subclinical infection to myocarditis.

Transmission:

• Is by the faeco-oral route, including shared drinks containers.³
• It is highly contagious.
• The incubation period is short - a few days.⁴

• Pain in the lower chest or upper abdomen:
  • Onset may be sudden. The pain is sometimes spasmodic.
  • It is exacerbated by deep breathing or movement, and can be severe enough to cause dyspnoea.
  • Duration is normally a few days, but may be ≤ 3 weeks and symptoms can recur/relapse.⁴
• Other symptoms:
  • Fever, headache or non-specific abdominal pain - may occur either as prodromal symptoms or with the onset of pain.
  • Myalgia elsewhere.
• Signs: fever and localised tenderness at the site of pain.

Usually the diagnosis is clinical, but investigations may be needed:

• To exclude other important diagnoses or to assess complications, for example: ECG, chest x-ray, D-dimer or other tests according to the clinical picture. Chest x-ray is normal in uncomplicated Bornholm disease.²
• Viral studies may be useful if there are complications or vulnerable contacts, e.g. with neonates or late pregnancy. Possible tests are:⁶
  • Serology for coxsackie B virus IgM and IgG.
  • Culture of the virus from faeces and serology.

Other causes of pleuritic-type pain or chest pain are:

• Pulmonary embolus, pleural effusion
• Pneumonia
• Myocardial infarction or pericarditis
• Pneumothorax
• Tietze's syndrome
• Muscle strain or chest trauma
• Shingles

With subcostal pain, consider:

• Peptic ulcer
• Cholecystitis, gallstones or hepatitis
• Subphrenic abscess
• Splenomegaly - in the context of febrile illness, consider glandular fever; if acute pain or injury, consider splenic rupture

• Exclude other important diagnoses
• Supportive treatment - analgesia
• Consider risk to neonates (see complications and prevention)

An overview of the literature suggests that many or most cases are uncomplicated.

Reported complications, mainly from case reports, are:

• Pericarditis and myocarditis;⁶ possibly, myocarditis is more common in young children and pericarditis in adults⁵
• Transient paroxysmal tachycardia (one case report, probably due to myocarditis)⁵
• Orchitis⁷
• Viral meningitis¹

Also, there are other recognised complications of coxsackie and echo virus infections (the literature is less clear how these relate to Bornholm disease specifically):^1,2

• Neonatal generalised infection, including myocarditis and meningo-encephalitis, which can be fatal;⁸ prophylaxis may be given to exposed neonates (see prevention)
• Respiratory infection
• Skin or oropharyngeal manifestations
• Transient paralytic illness

Possible associations have been suggested between Coxsackie B virus and:

• Chronic fatigue⁹
• Type 1 diabetes¹⁰

• Enteroviral infections in pregnancy are common, and most are not associated with significant maternal or neonatal disease.
• However, neonatal infection is an important risk:
  • Transmission from mother to baby can occur via intrauterine, vaginal or postnatal modes.
  • Neonatal disease may range from inapparent infection to overwhelming systemic illness and death.
  • Immunoglobulin is advised for prophylaxis of exposed neonates (see prevention).
• Enterovirus infections in pregnancy are not known to cause any fetal abnormalities.
• Maternal echovirus or coxsackie virus B infections are not known to increase the risk of spontaneous abortions, although cases of stillbirths late in pregnancy have been reported.

• For neonates exposed to enterovirus infections, the Health Protection Agency advises immunoglobulin prophylaxis,¹¹ quoting its use in an outbreak of echovirus infection.¹² In this situation, discussion with a microbiologist would also be appropriate.
• To reduce transmission, handwashing and hygiene; avoid sharing food and drink utensils.³

The disease was described by doctors Homan and Daae in Norway in 1872, and was called Bamble disease as their first case lived in Bamble. The name Bornholm disease was given by a Danish doctor, Sylvest, who observed the illness on the island of Bornholm in Denmark in the 1930s. UK epidemics occurred in 1956⁷ and 1963.⁵