Funtabulously Frivolous Friday Five 285

A sense of impending doom

The term was Initially used in 1928 by the English physician John Alfred Ryle (1889–1950) to describe “the aura of a nervous storm having its origin in those medullary centres upon which the act of living depends” during his work on vertigo and tumours of the vagus nerve and CNS.  He considered it a ‘medullary aura’, akin to aura’s associated with seizures or migraines.  Ryle later developed angina in 1942 and described his own experiences of angor animi in 1950, linking the term to a cardiac origin and not only neurogenic phenomena.

The term persists today but is most often used to describe the sensations from either acute myocardial infarction or the administration of adenosine. 

When asking in your history taking if a patient has ever directly experienced angor animi, we hope you have a better experience than that described in 1964 in the BMJ by Dr J.C. Barker:

I was called to examine a man aged about 60 with a history of effort angina which had increased progressively during the preceding few weeks. He was sitting up in bed chain-smoking and appeared extremely tense.

I inquired about the character and distribution of the pain and its relationship to exertion, and his replies were lucid and gave a classical picture of angina pectoris. I then inadvertently asked him whether he ever felt he was going to die during one of the attacks. He did not reply to this question but his attitude immediately changed. He seemed to become increasingly distressed and looked at me with fixed staring eyes.

He died about one minute later while I was auscultating his heart.”

Barker JC 1964

References

• Ryle JA. Angor Animi, or the sense of dying. Guy’s Hospital Report. 1928; 78: 371-376
• Ryle JA. The sense of dying–a postscript. Guy’s Hospital Report. 1950; 99(4): 204-229.
• Ryle JA. Angor animi, or the sense of dying. Guy’s Hospital Report. 1950; 99(4): 230-235.
• Angor animi. The Lancet, 1951; 257(6660): 898
• Ban TA, Lohrenz JJ, Lehmann HE. Observations on the action of Sernyl-a new psychotropic drug. Can Psychiatr Assoc J. 1961; 6(3): 150-157.
• Barker JC. Angor Animi. Br Med J 1964; 2: 688

Cry of the cat

Genetic condition resulting from partial deletion of the short arm of chromosome 5.

Originally described in 1963 by Jérôme Lejeune (1926 – 1994), the condition was initially termed Lejeune syndrome.

The condition is now more commonly termed cri du chat syndrome after the distinctive cat-like cry that people affected by this genetic disorder display in the first three months of life. The cry itself resembles the mewing of a cat and has an abnormal monotonic quality.

Cri du chat syndrome is caused by a deletion of the end of the short (p) arm of chromosome 5 occuring in 1/25,000 to 1/50,000 of live births. The disorder is characterized by intellectual disability and delayed development, microcephaly , low birth weight, and hypotonia in infancy. Affected individuals also have distinctive facial features, including hypertelorism, low-set ears, a small jaw, and a rounded face

The size of the deletion varies among affected individuals whilst larger deletions tend to result in more severe intellectual disability and developmental delay than smaller deletions.

References

• Lejeune J et al. [ 3 Cases of partial deletion of the short arm of chromosome 5 ] C R Hebd Seances Acad Sci. 1963 Nov 18;257:3098-102.
• Pentiuk S, Mezoff A. Cri du Chat Syndrome. NORD

Ghana

Kwashiorkor is derived from the Ga language of coastal Ghana, translated as “the sickness the baby gets when the new baby comes” or “the disease of the deposed child”.

In 1933, Cicely Delphine Williams (1893 – 1992) a Jamaican pediatrician first published her initial description “A nutritional disease of children associated with a maize diet” in the journal ‘Archives of Diseases in Children’. This publication was based on the observation on significant mortality and morbidity that children in then British ‘Gold Coast Colony’, now Ghana. 

Williams believed that kwashiorkor was a nutritional disease affecting children between 1 and 4 years, secondary to a diet of mainly maize after they cease breast feeding. Williams provided descriptions of skin and mucosal membrane involvement, diarrhoea, and post mortem fatty liver changes. Two years later she published an article in the Lancet which named the disease outright

In fact, a name means very little except to classify a certain conception. Until pathologists and biochemists can give us more precise information about the defects, we may well accept the word kwashiorkor in all its cacophony.

Cicely Williams, 1953.

Currently, kwashiorkor is principally noted by its oedema, secondary to low serum protein, thus its modern term ‘oedematous malnutrition’. Death is from general weakened state and susceptibility to infections. Although very commonly believed to be due to protein under-nutrition, the true aetiology is much less well refined and more complex.

References

• Williams CD. A nutritional disease of childhood associated with a maize diet. Arch Dis Child. 1933 Dec; 8(48): 423–433.
• Williams CD. Kwashiorkor: a nutritional disease of children associated with a maize diet. Lancet 1935; 226(5855): 1151-1152 [PMC2572388]
• Williams CD. Kwashiorkor: Council on Foods and Nutrition. JAMA. 1953; 153(14): 1280-5.
• Stanton J. Listening to the Ga: Cicely Williams’ discovery of kwashiorkor on the Gold Coast. Clio Med. 2001;61:149-71.
• Heikens GT, Manary M. 75 years of Kwashiorkor in Africa. Malawi Med J. 2009 Sep;21(3):96-8.
• Kwashiorkor. BMJ Best Practice.

I will harm

Derived from the Latin verb nocere – to harm, hurt (physically); more common medical usage as primum non nocere (first do no harm)

The nocebo effect refers to non-pharmacodynamic, harmful, unpleasant, or undesirable effects a person experiences after receiving an inactive treatment. Like the placebo effect, this additionally has the potential to impact on active therapy.

Originally described in 1961 by Walter P. Kennedy, to designate noxious effects produced by a placebo after noticing non-specific adverse reactions to drugs, which he hypothesised to have parallels to the placebo effect.

Shortly after placebo (Latin: ‘I will please‘ ) effects were recognized and catalogued, Kennedy noted that a number of subjects across various trials were exhibiting negative effects of their placebic therapies, chiefly through gastrointestinal or CNS complaints.  Kennedy emphasized the importance of recognizing the negative effects and to attempt to avoid confusion with true pharmacological effects.  Kennedy described his experience with a patient that may not pass ethics council approval today:

I prescribed the same powder but in a (different) mixture…adjuring the patient that the mixture was a strong one and he must shake the bottle well. He returned in ten days to say he had not felt so well for years.

Another classic example of the nocebo phenomenon is illustrated by the experiment of Schweiger and Parducci (Nocebo: the psychologic induction of pain, 1981). They recruited a group of students and informed them that they would be subjected to a procedure “administration of electric current to the head” which participants were informed “may induce headaches“. Even though no electricity was administered, the majority of participants reported headache.

References

• Kennedy WP. The nocebo reaction. Med World. 1961; 95: 203-5.
• Schweiger A, Parducci A. Nocebo: the psychologic induction of pain. Pavlov J Biol Sci. 1981; 16(3): 140-3.
• Data-Franco J, Berk M. The nocebo effect: a clinicians guide. Aust N Z J Psychiatry. 2013; 47(7): 617-23.
• Planès S, Villier C, Mallaret M. The nocebo effect of drugs. Pharmacol Res Perspect. 2016 Apr; 4(2):

84% of the time!

Yes, I know there is no difference! 

But also nary a week goes by when I don’t have to explain the difference to a family who have had a run in (more accurately a fall-off) with a set of monkey bars.  

Kampa et al, 2006, performed a prospective questionnaire set to measure how often their adult patients maintained the belief that the two terms had different meanings in an effort to highlight the ongoing importance of accurate doctor-patient communication.  Importantly they were able to demonstrate the importance of verbal communication and not to rely on pamphlets.  

Of 50 patients, 84% thought there was a difference between a ‘fracture’ and a ‘break’, with 68% believing a ‘break’ to be worse than a ‘fracture’. 

Following the introduction of an information leaflet, a re-audit of 61 new patients took place. This time 67% felt there was a difference between a ‘fracture’ and a ‘break’, with 65% believing a ‘break’ to be worse than a ‘fracture’. Only 21% had read the supplied information leaflet, and 69% of those still believed there was a difference between a ‘fracture’ and a ‘break’. 

• Kampa RJ, Pang J, Gleeson R. Broken Bones and Fractures – An Audit of Patients’ Perceptions. Ann R Coll Surg Engl. 2006; 88(7): 663–666.