Da Costa's syndrome

Da Costa's syndrome
Specialty	Psychiatry

Da Costa's Syndrome is a type of anxiety disorder named for the surgeon Jacob Mendes Da Costa who first observed it in soldiers during the American Civil War. It has also been called effort syndrome, neurocirculatory asthenia, or "soldier's heart". It causes symptoms similar to heart disease - such as fatigue upon exertion, shortness of breath, palpitations, sweating and chest pain - but upon examination, nothing is found to be physically wrong with the patient. The term is infrequently used in modern texts, and the syndrome is now usually interpreted as one of a number of imprecisely characterized "postwar syndromes".

History

Da Costa's Syndrome was first described among soldiers in 1869 by Arthur Bowen Richards Myers, who called it neurocirculatory asthenia or cardiovascular neurosis, but acquired its more usual name from the 1871 Da Costa study, which reported the latter's observations made during the American Civil War. Use of the term "Da Costa's syndrome" peaked in the early 20th century. Towards the mid-century, the condition was generally re-characterized as a form of neurosis. It was initially classified as "F45.30" (under somatoform disorder of the heart and cardiovascular system) in ICD-10, and is now classified under "somatoform autonomic dysfunction". A 1987 historical overview by Oglesby described it as having "a long and honourable history in the medical literature", considering it to still exist "as a disorder of unknown origin", "more often identified and labelled in psychiatric terms such as "anxiety state" or "anxiety neurosis" - and affecting 2-4% of the population.

1861-1950

In 1871 Jacob Mendez Da Costa’s report on more than 200 soldiers in the American Civil War identified a set of symptoms that included chest pains, palpitations, breathlessness, dizziness and fatigue, typically brought on by strenuous exertion. As seen in orthostatic intolerance, the pulse was always greatly and rapidly influenced by position, such as stooping or reclining. A typical case involved a man who was on active duty for several months or more and contracted an annoying bout of diarrhoea or fever, and then, after a short stay in hospital, returned to active service. The soldier soon found that he could not keep up with his comrades in the exertions of a soldier's life as previously, because he would get out of breath, and would get dizzy, and have palpitations and pains in his chest, yet upon examination some time later he looked like a man in sound condition.

Sir Thomas Lewis re-named this collection of symptoms effort syndrome in 1919. During this time, several synonyms were used to describe collections of symptoms which are related to Da Costa's syndrome, including soldier's heart, neurocirculatory asthenia, effort syndrome, and anxiety neurosis.

Several limited studies of these conditions were undertaken during the mid-20th century A 20-year longitudinal study of 173 patients with "effort syndrome" concluded that patients self-reported disability improved with a low-stress lifestyle. One detailed report on the breathlessness seen in effort syndrome stated, "In neurocirculatory asthenia, anxiety neurosis, or effort syndrome many respiratory symptoms occur in high incidence....The evidence of poor ventilatory efficiency corresponds interestingly, although it may not explain, another symptom which patients have which is that they 'can't get in enough air' or that 'air doesn't seem to do as much good as it should'". These reported symptoms are essentially identical to what is now called hyperventilation syndrome (which, paradoxically, results in less effective breathing).

After 1950

In 1951 the fourth edition of Paul Dudley White’s book “Heart Disease” contained a chapter on “Neurocirculatory Asthenia”, because, as he explains, the symptoms are similar to heart disease, but are not the same, and he adds, that they are also similar to, but can occur in the absence of anxiety, and therefore need to be discussed separately. He gives the definition of N.C.A. as the typical group of symptoms of breathlessness, often with sighing, palpitations, precordial aches and pains, exhaustion, and related symptoms such as dizziness and faintness, which are precipitated by excitement or effort, and “it constitutes a kind of fatigue syndrome” . . . and in some cases . . . “it is more or less a chronic condition“ . . . and . . . “That such a state of ill health exists there can be no doubt, no matter what its pathogenesis or exciting factors.” and “the symptoms are not exactly like those produced by effort in a normal healthy person. In some patients the neurocirculatory symptoms are prominent but in others it is gastrointestinal or cerebral symptoms, but the reason for those differences “has not been explained”. The general causes of the condition appear to include such strains as worry over business, social, or family matters, emotional conflicts, physical or nervous fatigue, and exhaustion from acute infections or illnesses. The organic basis is not known although it may involve a disorder of the autonomic nervous system. Other possibilities which have been considered in the past 25 years, include thyrotoxicosis, low-grade infection, adrenal hyperactivity, hyperventilation, and lack of salt, but none have been confirmed, However many of the patients have thin physiques with an “unusually vertical position of the heart”, and “Another interesting finding is abnormality of shape of the capillary loops at the base of the nail”. . . “It is common to find that close relatives have had similar problems, and “Recent studies have suggested that neurocirculatory asthenia belongs to the Mendelian dominant group of inherited disorders.” It was common in World War 1, occurred in civilians as well as soldier’s, and it is generally seen in young adults, but can occur at any age, and is more common in women than men. The symptom of frequent sighing distinguishes the condition from heart disease, and the fatigue sometimes produces more incapacity than heart disease, and in some cases results in complete disability. “It is a real and not an imaginary incapacity, even though at first glance it may have appeared imaginary in World War 1 (1914-1918) when it was sometimes labelled ‘malingering,’ and even though in civilian practice it has frequently been diagnosed as ‘mere nervouseness’.” It is milder in civilian life than in war and it is so commonly associated with psycho-neurosis of the anxiety type “that the two conditions have sometimes been confused one for the other or considered to be synonymous, the term anxiety neurosis having come to mean for many the same collection of symptoms which identify neurocirculatory asthenia.” Treatment involves rest for days or months or as long as required, and elaborate psychotherapy is generally not needed. “In fact, since this condition is neither heart disease nor mental disorder, both cardiologist and psychiatrist are well kept away after the diagnosis has been established, so that the patient may not develop unnecessary fears about either heart or mental state.” “The condition must be discussed seriously, not lightly as if it was of no importance”, and it is equally wrong to dismiss it as negligible or imaginary, as it is to to regard it as dangerous or serious and a threat to life which demands bed rest. Careless disregard will alienate patients and have them seeking advice from charlatans. “The plan of life for the patient needs to be worked out with care” where usually normal but quiet work and play are required, with the avoidance long working hours or new and burdensome tasks. “Often the patient himself is aware of this necessity, but he has perhaps disliked to humor his symptoms or to fall behind his fellows in strenuous living in the business, professional, or social world. With clear medical advice, however, he realizes the wisdom of doing so, and gradually he adjusts himself to suit his symptoms, and is surprised at recapturing a feeling of well being.”

In 1956 Paul Wood’s 2nd edition of Diseases of the Heart and Circulation included a chapter on the effort syndrome . He described how "The syndrome is characterised by a group of symptoms which unduly limit the subject's capacity for effort" and recorded that " The cardinal symptoms" of effort syndrome, neurocirculatory asthenia, irritable heart, soldier's heart, disordered action of the heart (D.A.H.), etc. are "breathlessness (93%), palpitations (89%), fatigue (88%), left inframammary pain (78%), and dizziness (78%), or syncope (fainting) (35%)". He also suggested a variety of methods for diagnosing the difference between the symptoms and those of heart disease. For example (the) “Left inframammary pain (in the lower rib area) is commonly described as aching or as sharp and stabbing in quality” and “It may be initiated” “by fatigue or strain of respiratory muscles” caused by such things as “incessant minimum trauma from” “faulty posture" . . . and with regard to the breathlessness "It is not only a question of breathlessness on effort, but patients will say they are not able to obtain a satisfying breath, and may take "frequent deep sighs". This can sometimes occur at night when it "may be confused with bronchial asthma or paroxysmal cardiac dyspnea". and "A simple and illuminating test" for the symptom involves forced hyperventilation where "The patient is asked to breath deeply and rapidly for one minute." When a healthy person is asked to stop he feels breathless for about 20 seconds, but a patient with Da Costa's syndrome "continues forced breathing, explaining later that he felt breathless." i.e. there is "Dyspnoea instead of apnoea after forced breathing", and "Normal subjects have no difficulty holding the breath for at least 30 seconds, but patients with Da Costa's syndrome usually give up very quickly, 30 per cent of them in less than 10 seconds; moreover, in contrast to controls, they show little distress when the reach the breaking-point." . . . With regard to the fatigue the patients often do not feel refreshed when they wake up in the morning, as if their sleep has been of no value, and they may "feel tired and listless during the day, and are unduly fatigued by effort. " . . . and . . . Orthostatic dizziness is related to orthostatic hypotension and "The effort-tolerance test (for effort-intolerance) consists of stepping on and off a chair ten times, and counting the pulse rate before, immediately after, and subsequently at minute intervals until the resting speed is regained. The deceleration time is abnormal (over 2 minutes) in 33% of these patients." and "Physical signs of autonomic dysfunction are helpful in” “assessing the severity of the case." The photo of a painting of a typical round shouldered, thin chested, kyphotic patient is included on page 941.

In 1980 Soviet researcher V.S.Volkov studied the physical fitness levels of patients with angina heart disease, and compared them to those with neurocirculatory dystony (Da Costa’s syndrome). He divided heart disease patients into three groups with heart pain at rest, heart pain every day, and heart pain occasionally. He also divided NCD patients into three stages of mild, moderate, and severe. 80% of Da Costa’s syndrome patients were fitter than heart disease patients, but 20% were not, and had to stop the exercise because of changes in their heart rate, or overwhelming and radiating chest pain, general fatigue, and fear for their hearts.

In 1990 S.D. Rosen and his colleagues from the Department of Cardiology in the Charing Cross Hospital, London conducted a study of patients who had been diagnosed with the chronic fatigue syndrome, myalgic encephalomyelitis, and postviral syndrome, which they referred to as the modern terms for the effort syndrome. Their objective was to determine the role of emotional factors and chronic habitual hyperventilation in producing the symptoms by testing the levels of CO2 in the lungs during, and after 3 minutes of deliberate rapid and deep breathing, and then again while the patients were thinking about prior personal experiences which involved anger or fear. The results showed that 93 of the 100 patients had evidence characteristic of chronic habitual hyperventilation. However other studies have found that the breathlessness of the effort syndrome or Da Costa's syndrome was once regarded as just subjective, or imaginary until 1947 when it was found to be due to abnormal function of the thoracic diaphragm - the main breathing muscle. Rosen and his colleagues also noted "It has long been recognized that hyperventilation-related illness can appear after or be aggravated by injury or infection”, so they studied that aspect and found evidence of chronic hyperventilation symptoms before the viral infection, and suggested that the infective illness simply made the fatigue worse. Their final paragraph mentioned the opinions of three authors who regarded normal health as being maintained by leading a moderate lifestyle and staying within reasonable boundaries, and that leading an excessively demanding lifestyle beyond those limits may be the cause of the effort syndrome.

In 1994 S.G, Saish and his colleagues from The College of Thoracic Medicine, Kings College School of Medicine and Dentistry, London, U.K. studied 31 patients with chronic fatigue and found that 71% “had no evidence of hyperventilation during any aspect of the test” and that “There is only a weak association between hyperventilation and chronic fatigue syndrome”.

In 1997 E. Bazelmans and his research colleagues from the Department of Medical Psychology of the University Hospital, Nijmegen, The Netherlands shed doubt on the hyperventilation cause with their evidence that it is not related to the number or severity of symptoms and is probably a consequence rather than a cause of the condition

Related conditions

Some researchers have described similarities between orthostatic intolerance, chronic fatigue syndrome, mitral valve prolapse syndrome, and the observations of Da Costa.

Treatment

The reports of Da Costa, and Wheeler show that patients recovered from the more severe symptoms when removed from the strenuous activity or sustained lifestyle that caused them. In many cases relapses were prevented by determining the limits of exertion and lifestyle and keeping within them. The limits were related to abnormalities in respiration and circulation. Other treatments evident from the previous studies were improving physique and posture, appropriate levels of exercise where possible, wearing loose clothing about the waist, and avoiding postural changes such as stooping, or lying on the left or right side, or the back in some cases, which relieved some of the palpitations and chest pains, and standing up slowly can prevent the faintness associated with postural or orthostatic hypotension in some cases.

See also

• Exercise intolerance
• Chronic fatigue syndrome
• Soldier's Heart (novel)