The ME Association believes that the creation of an ME/CFS Post-Mortem Tissue Bank would allow a better understanding of what is happening in parts of the body that are involved in this disease – skeletal muscle, the brain and nervous system in particular.
We therefore supported and commended the efforts of Kara Jane Spencer and her appeal to raise £100,000 for this purpose. With Kara’s tragic death, we wanted to clarify what is happening with the appeal, with post-mortem research and the charity’s plans for the future.
Background
The rationale and importance of establishing an ME/CFS post-mortem tissue bank was highlighted in a research study from 2014. The key points being:
“Post-mortem examinations have greatly helped to clarify the aetiology and pathogenesis of a wide range of medical disorders, e.g., Creutzfeldt-Jakob disease (CJD) and Parkinson’s disease, opening possibilities for better diagnosis and in many cases treatment.
“The poor understanding and lack of consensus on the causes and pathophysiological mechanisms involved in ME/CFS and the lack of specific animal models suggest its suitability for study through pathology.
“However, and rather surprisingly, only a very small number of (ad hoc) pathological studies have been conducted in people who have died with ME/CFS. A few but not all autopsies conducted have shown nervous system abnormalities, including inflammation in the dorsal root ganglia [here] and evidence of viral encephalitis [here and here].
“These do not, however, constitute sufficient evidence to confirm the role of the central or peripheral nervous system in its aetiology or pathogenesis…”
If a permanent ME/CFS post-mortem tissue bank could be established and more tissue studied, this would help confirm if these preliminary findings are more prevalent and provide a better understanding of the disease process that perpetuates the symptoms and disability that we have come to associate with ME/CFS. We might then be in a position to establish diagnostic tests and effective treatments.
The ME Association has therefore been involved in a number of initiatives that are aimed establishing a more formalised service for the collection, examination and storage of post-mortem tissue from people with ME/CFS.
- A steering group including members of the ME/CFS Biobank – which is funded by the ME Association – has examined the complex ethical, legal, practical and financial issues that would be involved in establishing such a facility.
- A report of the work that was done by this group was published in The BMJ Journal of Clinical Pathology in 2010.
- Researchers then went on to determine that such a facility would be more efficient and cost effective if it could be part of an established tissue bank.
- They created a protocol for the operation of an ME/CFS post-mortem tissue bank and recommended:
“The Addenbrooke’s Hospital Brain and Tissue Bank in Cambridge is very well placed for this purpose due to its long-term experience in tissue banking and previous involvement in ME/CFS research and is intended as the initial site for the ME/CFS Tissue Bank.”
- The protocol and recommendations were published in Biomed Central in 2014.
The current situation
To take these initiatives forward the ME Association played a key role in setting up a small and informal post mortem research group who have been involved in all aspects of post mortem research over a number of years.
Dr Charles Shepherd (Hon. Medical Adviser to the charity) has dealt with much of the complex initial work involving liaison with relatives, coroners and pathologists where a death took place involving someone with ME/CFS who had consented for their tissue to be used for research purposes.
Dr Dominic O’Donovan, a neuropathologist at Addenbrooke’s Hospital in Cambridge, kindly performed a number of ad hoc autopsies and collected relevant tissue samples for study. The examination of some of these tissue samples showed promise and initial findings from some of the early post-mortems were presented at a conference in 2010 (referred to in the study from 2014 above and shown in full below).
Unfortunately, Addenbrooke’s Hospital decided that they were no longer able to continue with this very informal arrangement. We had to stop promoting the Statement of Intent (whereby people could confirm in writing that they wished to have tissues removed at a post-mortem) and arranging for the collection, preparation and transfer of post-mortem tissue to Cambridge. This suspension will remain in place until such time as a new relationship can be established.
Whether an ME/CFS post-mortem tissue bank can be created with Addenbrooke’s Hospital, or with another member of the UK Brain Banks Network, is something that we will be exploring with members of the ME/CFS Biobank team in due course.
The ME Association still wants to create a permanent post-mortem tissue bank facility that will expand on the work we have been doing with the neuropathology team at Addenbrooke’s. We have been the only charity in the world to be involved in the collection and study of post-mortem tissue and we do not want to abandon this important and promising area of research.
The money raised by Kara’s appeal will be ring-fenced by the ME Association in its Ramsay Research Fund and used to create and support the operational costs of a tissue bank once a satisfactory agreement has been reached with a suitable centre, possibly an existing Brain Bank, here in the UK. We will be discussing these future plans with Kara’s family in due course.
We do not take any administration expenses from Ramsay Research Fund donations. All the money raised will be spent directly on an ME/CFS Post-Mortem Tissue Bank and we would encourage anyone who can comfortably afford to do so to support Kara’s appeal.
We will provide further updates in due course.
Recent news
- BBC News: Kara Jane Spencer: Severely ill singer who recorded hit album dies | 06 January 2023
- Liverpool Echo: Tributes to ‘warrior' woman, 32, who died after battle with chronic fatigue syndrome | 08 January 2023
More information
2010 Conference Presentation: A Pilot Study of Four Autopsy Cases.
International Science Symposium 3-4 – Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) Queensland, Australia: Population Health and Neuroimmunology Unit, Bond University.
DG O’Donovan1, 2, T Harrower3, S Cader2, LJ Findley2, C Shepherd4, A Chaudhuri2
1 Addenbrooke’s Hospital Cambridge UK, 2 Queen’s Hospital Romford Essex UK, 3 Royal Devon & Exeter Hospitals UK, 4 Honorary Medical Advisor to The ME Association UK.
Chronic Fatigue Syndrome/Myalgic Encephalomyelitis is a disorder characterised by chronic exercise-induced fatigue, cognitive dysfunction, sensory disturbances and often pain. The aetiology and pathogenesis are not understood. We report the post-mortem pathology of four cases of CFS diagnosed by specialists.
The causes of death were all unnatural and included: suicidal overdose, renal failure due to lack of food and water, assisted suicide and probable poisoning. Selected portions of tissue were made available by the various Coroners in the UK and with the assent of the persons in a qualifying relationship. The cases were 1 male, and 3 females. Ages (years) M32, F32, F43 & F31.
- One case showed a vast excess of corpora amylacea in spinal cord and brain of unknown significance, but Polyglucosan Body Disease was not supported by clinicopathologial review. No ganglionitis was identified.
- One case showed a marked dorsal root ganglionitis and two other cases showed mild excess of lymphocytes with nodules of nageotte in the dorsal root ganglia.
This raises the hypothesis that dysfunction of the sensory and probably also the autonomic nervous system may lead to abnormal neural activity e.g., hyperalgesia & allodynia rather than anaesthesia and may explain some of the symptoms of CFS/ME such as pain, hypotension, hyperacusis and photophobia. However, the syndrome may be heterogeneous.
Nevertheless, the precise relationship of fatigue, which may be either peripheral or central, to abnormalities in the peripheral nervous system (PNS) needs to be studied.
The differential diagnosis of ganglionitis should be investigated in CFS/ME patients hence Varicella Zoster, Lyme disease, HIV, Sjogren’s disease, paraneoplastic sensory ganglionopathy should be excluded by appropriate history and tests.
Thorough histopathological study of cases coming to autopsy may help to confirm or refute the hypothesis, that CFS is a disease process, and whether the symptomatology may be explained by inflammation of the sensory and autonomic divisions of the PNS.