Submission to the RACP CFS Clinical Guidelines Review Committee
Continued

Authors: Christine Hunter, Annette Leggo, Anne Gotsis and Maureen Stephenson

4.0 Diagnostic Criteria for ME/CFS

"We shut our eyes to observations which do not agree with the conclusions we wish to reach. We close our eyes to bits of history which seem out of place, or to noises coming down our stethoscopes which are not included in the catalogue of official sounds we have been taught to recognise." - Dr. Richard Asher, doctor and writer, warned thirty years ago, B.M.J, 308, 19 March 1994.
"When my Mum suggested to the G.P. I could be referred to a doctor who was knowledgeable on CFS, he said if I went to a doctor who 'believed' in CFS, I would be diagnosed with it and if I went to a doctor who 'didn't believe' in CFS I wouldn't be diagnosed with it. It is difficult to hear someone describe the need to believe in an illness when you are suffering its effects every day" - Adolescent with ME/CFS

4.1 The most recognised symptom check lists for diagnostic criteria are as follows:
The Wallis/Behan Diagnostic Description*
Following an initial infectious process the child may demonstrate:

1. Depression: This often occurred with weeping tendencies, and appeared early. Nearly all affected children are diagnosed first as hysterical, depression or "parental over-involvement."
2. Loss of energy: This occurred in all but the mildest cases and frequently persisted.
3. Retardation of thought process: Work involving abstract thought was difficult to perform in all with protracted illness or recurrences. Serial seven test was poorly performed, often with errors, often starting the test well and then getting bogged down.
4. Impairment of thought process: This was a common feature, and the contents of papers or magazines read only a few minutes earlier could not be recollected.
5. Impairment of memory: Recentl retention and recall: Items of work to be done or purchases to be made had to be listed as memorising proved unreliable.
6. Disorders of sleep: Inversion of sleep rhythm was common with nightmares in children, often with hallucinations on waking.
7. Behavior disorders: Temper tantrums were frequent in young children. In older children unsociability, lack of attention and effort on return to school was frequent. If behaviour was checked, children tended to weep. There is anxiety and clinging dependency, with a reluctance to attend school.
8. Physical activities: There is lack of interest in playing games with other children. When forced to attend school and take part in physical exercises, this has been followed by disastrous deterioration in the clinical condition, with overwhelming exhaustion and weakness supervening.
9. Weight loss: A significant amount of body weight may be lost early in the disease process.
10. Profound weakness: The weakness may be so severe that the child is confined to a wheel chair.

*This list is combined from two separate publications - of A.L. Wallis and P.O. & W. Behan. Clinicians with broad experience regard the Wallis/Behan Diagnostic Description as an invaluable aid for diagnosing young people.
The Henderson and Shelokov Summary

1. Fatigability, malaise, prodormal sore throat
2. Muscle weakness, cramps, spasms and twitches
3. Bursting headache, neck pain, eye pain
4. Irritability, depression, lack of concentration, emotional instability, impaired mentation
5. Visual problems, photophobia, diplopia
6. Dizziness, nausea, cutaneous sensory changes
7. Urinary, mentrual difficulties

From the New England Journal of Medicine - 1959

1988 CDC Criteria for the Chronic Fatigue Syndrome:
Major Criteria

1. New onset of debilitating fatigue that does not resolve with bedrest and that impairs average daily activity below 50% of premorbid levels for at least 6 months.
2. Exclusion of conditions that may cause fatigue, including malignancy, autoimmune and chronic inflammatory disease; infections; neuromuscular or endocrine disease; specific psychiatric disorders; chronic use, abuse or side effects of prescription or illicit drugs; and exposure to toxins.
   

Minor Symptom Criteria
To fullfill a symptom criterion, a symptom must have begun at or after the time of onset of increased fatigability and must have persisted or recurred over at least 6 months.

1. Mild fever - oral temperature between 37.5 degrees C and 38.6 degrees C - or chills.
2. Sore throat.
3. Painful axillary, anterior, or posterior cervical nodes.
4. Unexplained generalized muscle weakness.
5. Muscle discomfort or myalgia.
6. Prolonged (24 hours or more) generalized fatigue after levels of exercise that would have been easily tolerated in the premorbid state.
7. New of different generalised headaches.
8. Migratory arthralgia without joint swelling or redness.
9. Neuropsychological problems (including photophobia, transient visual scotomata, forgetfulness, excessive irritability, confusion, difficulty thinking, inability to concentrate, and depression.)
10. Sleep disturbance (hypersomnia or insomnia).
11. Onset of the main symptom complex over hours to a few days.

Physical Examination
Criteria Physical criteria must be documented by a physician on at least two occasions at least 1 month apart.

1. Low-grade fever - oral temperature of 37.6 degrees C to 38.6 degrees C or rectal temperature of 37.8 degrees C to 38.8 degrees C.
2. Nonexudative pharyngitis.
3. Palpable or tender anterior or posterior cervical or axillary lymph nodes.

From Annals of Internal Medicine 1995; 123; 81-88. Evaluation and classification of unexplained chronic fatigue. From Annals of Internal Medicine 1994; 121; 953 - 959. ALT = alanine aminotransferase; BUN = blood urea nitrogen; CBC = complete blood counts; ESR = erythrocyte sedimentation rate; PO4 = phosphorus; TSH - thyroid stimulating hormone; UA = urinalysis.

I. Clinically evaluate cases of prolonged or chronic fatigue by:
A. History and physical examination;
B. Mental status examination (abnormalities require appropriate psychiatric, psychologic, or neurologic examination);
C. Tests (abnormal results that strongly suggest an exclusionary condition must be reported):

1. Screening lab tests: CBC, ESR ALT, total protein, albumin alkaline phosphaiase, Ca, PO4, BUN, electrolytes, creatinine, TSH, and UA.
   
2. Additional tests as clinically indicated to exclude other diagnoses. Exclude case if another cause for chronic fatigue is found.
   

II. Classify case as either chronic fatigue syndrome or idiopathic chronic fatigue if fatigue persists or relapses for 6 months or more.
A. Classify as chronic fatigue syndrome if: a. Criteria for severity of fatigue are met, and b. Four or more of the following symptoms are concurrently present for 6 or more months:

1. impaired memory or concentration.
   
2. sore throat.
   
3. tender cervical or axillary lymph nodes.
   
4. muscle pain.
   
5. multi-joint pain.
   
6. new headaches.
   
7. unrefreshing sleep, and
   
8. post-exertion malaise
   

B. Classify as idiopathic chronic fatigue if fatigue severity or symptom criteria for chronic fatigue syndrome are not met.

III. Subgroup research cases by the presence or absence of the following essential parameters:
A. Comorbid conditions (psychiatric conditions must be documented by use of an instrument);
B. Current level of fatigue (measured by a scale);
C. Duration of fatigue;
D. Current level of physical function (measured by an instrument).
Subgroup research cases further as needed by optional parameters such as epidemiologic or laboratory features of interest.
4.2 The existence of varied diagnostic criteria:

• illustrates the complexity of ME/CFS
  
• demonstrates the diversity of expert medical opinion
  
• highlights the danger of affording priority to any one diagnostic list, thus excluding new knowledge as it emerges
  
• emphasises the need to ensure guidelines for doctors are presented in a format which can be readily updated.

4.3 Careful consideration must be given to the terminology used in the preparation of diagnostic lists for doctors, as many criteria are ill defined, e.g.

• "can't concentrate" trivialises the serious deterioration of the neurocognitive function.

"My cognitive difficulties were frightening and confusing and I often feared I was going crazy. Ordinarily an intelligent and avid learner, thinking was suddenly clouded and confused. I forgot things extremely easily. I mixed up words and I couldn't think of phrases I wanted to use; my concentration span was extremely short and my mathematical ability almost disappeared." - Female with ME/CFS (18 years)

• "loss of energy" and "fatigue" inadequately describe the profound exhaustion.
  

"The pain and exhaustion of the walk from my bed to the bathroom would take half an hour to subside." - B.M.J. 30, 19 March 1994, p 797 - Claire Fleming, G.P.

4.4 Doctors need to be aware that:

• onset can be sudden or gradual
  
• there are implications arising from gradual onset, especially with children - recurring infections, delayed recovery from colds, flu etc, declining health, declining work skills, difficulties with education, adaptation to decline, i.e. loss of sense of normality in relation to physical health
  
• symptoms at onset can change during the 6 month requirement for an ME/CFS diagnosis.
  

"My illness began so mildly and innocently more than ten years ago - as the years have passed I've developed more and more complications and faced several life threatening episodes. I've been hospitalised five times for a total of over 13 weeks and now have chronic gastroparesis and paralytic ileus. I have been nil by mouth for 18 months; I've had three PICC lines lost due to rejection infection and phlebitis. I've had three central lines." - Female with ME/CFS.

• criteria lists fail to acknowledge severe symptoms. This reflects a serious omission in current knowledge.

4.5 Criteria lists could be strengthened by the inclusion of other symptoms. This requires an open-minded approach, given the lack of conclusive research findings at this point in time, e.g. in "Examination of the Working Case Definition of Chronic Fatigue Syndrome", American Journal of Medicine, January 1996, 100, 56 - 64, Komaroff et al. argued for the inclusion of anorexia and nausea and restoration of infectious type symptoms of fever, chills, sore throat and painful glands, to strengthen the CDC case definition of ME/CFS. Komaroff et al. also found additional minor criteria which were strong discriminators of ME/CFS.

Table II
Minor Criteria Symptoms in Patients With Chronic Fatigue Versus Healthy Controls and Disease Comparison Groups

* P < 0.001 when compared to patients with chronic fatigue.
** P < 0.01 when compared to patients with chronic fatigue.
^ Neuropsychiatric symptoms and sleep disturbances are each treated as single symptoms by the CDC case definition. In this table, each of these symptoms is broken down into a group of more specific symptoms. The percentage of patients reporting at least one of the more specific symptoms listed is shown in the rows entitled Neuropsychiatric symptoms and sleep disturbance. The frequency with which each of the more specific symptoms was reported is also displayed.
NA= Not applicable.

Table III
Non-CDC Symptoms in Patients with Chronic Fatigue Versus Healthy Controls and Disease Comparison Groups

* p < 0.001 when compared to patients with chronic fatigue.
** P < 0.01 when compared to patients with chronic fatigue.
^ Data obtained from second subset of healthy control subjects

4.6 Komaroff's study (4.5 above) is important in its distinction between ME/CFS, multiple sclerosis and depression, namely:

• early morning awakening is a discriminator for depression
  
• anorexia and nausea discriminated ME/CFS from multiple sclerosis and depression
  
• CFS is distinguished from multiple sclerosis and depression by certain symptoms - myalgias, postexertional malaise, headaches.

"There are time lags between occurrence recognition, finding a label, acceptance and scientifically proven treatment. At times like this my profession rarely covers itself in glory. There is tragedy associated with the delay between a 'new' disease arising and professional acceptance. CFS wears the delays in relative silence." - Prof. Malcolm Fisher, 29 April 1993.
"It's one of the loneliest illnesses in the world because we don't have anything to show for it." - Lyn Drysdale, 1993 Order of Australia
"When the weight loss I had been complaining about for months could no longer be ignored by my doctor he asked, 'Do you engage in exercise that could be considered excessive?' I replied 'Does washing one's hair sitting down count?'" - Adolescent with ME/CFS.
4.7 The injustice of misdiagnosis occurs in labelling ME/CFS patients with:

• shirker and malingerer
  
• school phobia
  
• anorexia nervosa
  
• pervasive refusal syndrome
  
• Munchausen's Syndrome by proxy
  
• abnormal illness behaviour
  
• somatisation disorder
  
• hysteria
  
• postnatal depression

4.8 Thus, it is essential that: guidelines for doctors emphasise the need for an open-mind and acceptance of the complexities and idiosyncratic nature of ME/CFS when diagnosing patients.

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