ME Advocates Ireland (MEAI): Diagnosis of Myalgic Encephalomyelitis (ME) Part 3

Sunday 8 May 2022

Diagnosis of Myalgic Encephalomyelitis (ME) Part 3

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FIRST - DO NO HARM

In the case of Myalgic Encephalomyelitis (ME) patients -

Do as little harm as possible while trying to help the

patient improve quality of life.

International Consensus Criteria & Primer

The “Myalgic Encephalomyelitis – International Consensus Criteria (ICC)” advocates for removing fatigue as a characteristic symptom and defines the illness as an acquired neurological disease with complex global dysfunctions.

The ICC defines specific symptom requirements: post-exertional neuroimmune exhaustion (PENE), neurological impairments, immune, gastrointestinal, and genitourinary impairments, and energy metabolism impairments.

The ICC identifies patients who have measurable and reproducible pathophysiological abnormalities in response to exertion, which people with general fatigue or depression do not have.

The International Consensus Criteria (ICP) for medical practitioners is a valuable tool based on the ICC for working with your doctor to get a diagnosis and treatment for ME. Unfortunately, very few doctors are familiar with this important tool.

International Consensus Primer (ICP): “Myalgic Encephalomyelitis (ME): complex, acquired multi-systemic disease Pathophysiology: -

Profound dysfunction/dysregulation of the neurological control system results in faulty communication and interaction between the CNS and major body systems, notably the immune and endocrine systems, dysfunction of cellular energy metabolism and ion transport, and cardiac impairments. Cardinal symptom: a pathological low threshold of fatigability that is characterized by an inability to produce sufficient energy on demand. There are measurable, objective, adverse responses to normal exertion, resulting in exhaustion, extreme weakness, exacerbation of symptoms, and a prolonged recovery period. Note: Myalgic encephalomyelitis (ME) is the name recommended for those meeting the ICC.”

The primer was written to provide clinicians a one-stop, user-friendly reference for ME. See ICP here.

Diagnostic steps as per the International Consensus Primer (ICP)

There are diagnostic steps that can be taken as per the International Consensus Primer:

1. A medical history and a physical assessment. See pages 10- 12 International Consensus Primer (ICP) 2012.

2. A tentative diagnosis is based on symptoms and features as outlined in the ICP pages 7-8 and evolves throughout the clinical assessment.

The primary compulsory feature of ME is Post Exertional Neuro Immune Exhaustion (PENE), often referred to as PEM, the failure to recover after physical or mental activities. This is a substantial reduction to previous levels that significantly interferes with daily work and activities.

And at least 1 symptom from 3 symptom categories (B) Neurological Impairments; : at least 1 symptom from 3 symptom categories (C) Immune/gastro-intestinal/genitourinary Impairments; and 1 symptom from (D) Energy metabolism/ion Transport Impairments, all detailed further in the ICP on pages 7 and 8.

3. A standard series of routine medical tests*, involving blood tests will be completed to investigate symptoms and exclude other possible illnesses. See the International Consensus Primer pages 11-12.

4. After the screening tests, more specific tests depending on symptoms and any previous test results. Tests may guide treatment and symptom management. See pg.13 onwards in the ICP.

5. Laboratory and other investigations confirm or refute the tentative diagnosis.

6. Referral to appropriate specialists/consultants for specific investigations or management of more complex issues. There is currently no HSE ME consultant available in Ireland across nine CHO areas and multiple hospital groups.

Further, objective serial stress tests recorded by heart monitors and magnetic resonance spectroscopy (MRS) can confirm many symptoms, e.g., post exertional exhaustion and pain, decreased cerebral oxygen, and significantly reduced anaerobic threshold heart rate, VO(2) peak and peak work, prolonged recovery period. Tilt tests can identify orthostatic intolerance (OI), however, the test is not suitable for most people with ME; there are other tests e.g., the Grip Test. The use of 24‐h Holter heart monitors and cardiac MRS can identify abnormalities in heart function.

7. The doctor should suggest the importance of early rest to prevent a worsening of symptoms and tell the person with ME not to push through illness, to pace and avoid activity that may cause relapse.

8. The collective pathophysiology of ME is quite distinctive. However, based on the patient’s history, risks, and symptoms, it is important to rule out other infectious diseases that could simulate the collective, complex pathophysiology of ME.

See notes on Differential Diagnosis pg.12 International Consensus Primer.

9. Reassessment, regular ongoing follow-up.

What the person with ME can do to assist with the diagnosis:

- keep a diary to provide accurate information about their symptoms and about fluctuations, to helps link symptom patterns and identify what affects symptoms and symptom severity.

- Complete the PERSONALIZED CLINICAL ASSESSMENT & DIAGNOSTIC WORKSHEET FOR ME on page 10 of the ICP and bring to consultations, International Consensus Primer.

* Testing as part of Diagnosis and Management

Myalgic Encephalomyelitis (ME) is not a diagnosis of exclusion or an untestable disease. Tests will only all be normal in ME patients, as with most illnesses, if the wrong tests are conducted, or if those tested do not have ME.

Tests mentioned in the ICP and more below (with possible overlaps) can be used to confirm an ME diagnosis include:

SPECT and xenon SPECT scans of the brain.
MRI scans of the brain.
PET scans of the brain.
EEG/QEEG brain maps.
Neurological examination.
The Romberg test.
Immune system tests.
Insulin levels and glucose tolerance tests.
Erythrocyte Sedimentation Rate (ESR) tests.
Circulating blood volume tests.
24 hour Holter monitor testing.
Tilt table examination and standing/sitting/reclining blood pressure tests.
Exercise testing and chemical stress tests.
Physical exam.

These tests are the most important in the diagnosis of ME, although various other types of tests are also useful.

Despite the existence of the above tests for ME the unfortunate reality is that many people who suspect they have ME do not have access to the appropriate tests or to doctors could do the tests or who are able to make a diagnosis.

Symptom and Severity Questionnaires

The ICP does not include a symptom and severity questionnaire so we suggest using the De Paul University Symptom and severity questionnaire, as seen in the Diagnosis Part 1 post, to record and track symptoms for diagnosis and as evidence for medical appointments etc.

Symptom Questionnaire (DePaul Symptom Questionnaire DSQ-2)

The DePaul Symptom Questionnaire (DSQ-2) is a self-report assessment created by Leonard Jason at DePaul University, Chicago, Illinois, US. With 54 questions in total, the DSQ Symptom Questionnaire assesses key symptoms of ME such as post-exertional malaise, sleep, pain, neurological/cognitive impairments and autonomic, neuroendocrine and immune symptoms. At each item, participants have to rate the frequency and severity of the symptom on a scale from 0 to 4. You can put a score for how frequently you have each symptom and how severe it is. The questionnaire is based on research so useful to print out to complete and shown to your GP/Consultant/other.
As much as anything the questionnaire can teach your GP what ME is.

This is the link to the DSQ-2 symptom questionnaire.

Along with the symptom questionnaire you could also look at your experience with exertional intolerance and the post exertion response, i.e. PENE, often referred to as PEM; it is referred to as PEM by De Paul University (US).

By completing the useful DPEMQ questionnaire you can record and evidence your exertion intolerance.

PEM Questionnaire (De Paul DSQ PEM Questionnaire - DPEMQ)

Post exertional neuro immune exhaustion (PENE) also referred to as PEM is a cardinal symptom of Myalgic Encephalomyelitis (ME). The PEM questionnaire by De Paul is a questionnaire on the post exertional response, an essential criterion for an ME diagnosis.

See more on PENE (PEM) further on in this post.

By answering the questions, you get an idea of how ‘activity’, anything you do physically, cognitively, emotionally, affects you and what your individual post exertional response is, i.e., what symptoms occur and increase. Every person with ME is different.

Post exertional response for a lot of people might not occur straight away and can be delayed by up to 24 hours or 48 hours after activity.

The questionnaire includes key indicators that show within answering a set of questions that it sounds like ME.

This is the link to the De Paul Post Exertional Questionnaire.

Paediatric Questionnaires

The DSQ- PED questionnaires from De Paul consists of a parent form and a child form. Parents can fill in a symptom questionnaire to evidence the child's symptoms and severities to present to the child’s medic. Medics can receive data about the child with ME from both child and their parents to obtain a thorough understanding of the child's ME. Children under the age of 12, or those with reading or comprehension difficulties, complete this questionnaire with the assistance of a parent or guardian.

These forms are based on research by De Paul.

DSQ-Ped Parent Form here

DSQ-Ped Child Form here

Illness and Symptom Management

See our post on Management of Myalgic Encephalomyelitis: - About Managing & Treating Myalgic Encephalomyelitis (ME)

About Myalgic Encephalomyelitis (ME)