The cause of the mystery illness among US and Canadian diplomats in Havana is most likely to be emotional trauma and fear according to a leading sociologist and an expert in neurodegenerative diseases, writing in the Journal of the Royal Society of Medicine.
Concussion-like symptoms, including headaches, dizziness, nausea and fatigue, were initially reported among dozens of US embassy staff between late 2016 and June 2018.
They were described by the US State Department as ‘medically confirmed symptoms’ and government physicians suspected the involvement of a sonic device.
Studies on the embassy patients, however, have been inconclusive and contradictory. A similar array of symptoms was reported in over two dozen Canadian diplomats during this same period.
The paper’s lead author, Dr Robert Bartholomew, concludes that ‘Havana Syndrome’ is more akin to shell shock, with the symptoms paralleling those associated with war trauma.
“A characteristic feature of combat syndromes over the past century is the appearance of an array of neurological complaints from an overstimulated nervous system that are commonly misdiagnosed as concussions and brain damage”, he writes.
He adds: “A signature feature of shell shock was concussion-like symptoms.
Like today, their appearance initially baffled physicians until a more careful review of the data determined that what they were seeing was an epidemic of psychogenic illness.
In fact, some of the descriptions from 100 years ago are virtually identical, right down to the use of the phrase ‘concussion-like symptoms’.”
Dr Bartholomew is a medical sociologist based in Auckland, New Zealand. The report was co-authored by Dr Robert W. Baloh, Director of the Neurotology Laboratory at the UCLA Medical Center.
The authors describe the diplomats who became sick as participants in a continuation of the Cold War, living in a hostile foreign country where they were under constant surveillance.
Between late 2016 and 2017, staff in Havana were living in a cauldron of stress and uncertainty, amid rumours of an enigmatic sonic weapon.
A similar array of symptoms was reported in over two dozen Canadian diplomats during this same period. The image is credited to the US government.
“The political and scientific evidence for the perpetration of an attack on US embassy staff in Cuba is inconclusive,” they write.
“What is the more likely, that the diplomats were the target of a mysterious new weapon for which there is no concrete evidence, or they were suffering from psychogenic symptoms generated by stress?
The evidence overwhelmingly points to the latter.”
They add: “There have been four separate studies of ‘Havana Syndrome’ to date. Each have critical design flaws including the use of inappropriate controls, inflated conclusions, and a lack of evidence for exposure to an energy source or toxin.
None adequately test the hypotheses they propose, while promoting exotic explanations that are not supported by the facts. Our conclusions are grounded in the prosaic and known science.
There is no need to resort to exotic explanations. Claims that the patients were suffering from brain and auditory damage are not borne out by the data.”
Usher syndrome (USH, OMIM 276900, OMIM 276905, OMIM 605472, ORPHA: 886) is the most prevalent deaf-blindness of genetic origin. It is a recessive inherited disease characterized by sensorineural hearing loss (HL), visual loss due to retinitis pigmentosa (RP), and, in some cases, vestibular dysfunction. Prevalence estimates range from 3.2 to 6.2/100,000 (Espinós et al., 1998; Keats and Corey, 1999).
Patients with USH are classified into three clinical subtypes (USH1, USH2, or USH3), based on the severity and progression of hearing impairment and the presence or absence of vestibular dysfunction. Usher syndrome type I (USH1) is the most severe type, characterized by severe to profound congenital sensorineural hearing loss, vestibular dysfunction, and prepubertal onset of RP eventually leading to legal blindness. USH2 is characterized by moderate to severe hearing impairment, normal vestibular function and later onset of retinal degeneration. USH3 displays progressive hearing loss, RP and variable vestibular phenotype (Saihan et al., 2009; Millán et al., 2010).
Currently, up to 13 genes have been associated with Usher syndrome: MYO7A, USH1C, CDH23, PCDH15, USH1G, and CIB2 are responsible for USH1, although the role of CIB2 in the Usher syndrome has recently been put on doubt (Booth et al., 2018). USH2A, ADGRV1, and WHRN are the three genes responsible for USH2, and the CLRN1 gene is the only one associated with USH3 cases to date. Besides, PDZD7 has been reported to behave as a modifier of the retinal phenotype in conjunction with USH2A, and a contributor to digenic inheritance with ADGRV1 (Ebermann et al., 2010). In addition, HARS was postulated as a novel causative gene of USH3, based on a mutation found in two patients (Puffenberger et al., 2012). Finally, mutations in CEP250 have been reported to cause cone-rod dystrophy, isolated RP and atypical forms of USH, characterized by early onset hearing loss and mild RP (Khateb et al., 2014; Fuster-García et al., 2018; Kubota et al., 2018).
In the last years, next generation sequencing (NGS) techniques have revolutionized the world of the molecular genetic diagnosis, allowing the whole genome, whole exome and targeted gene sequencing more feasible, and making easier, rapid and cost-effective the identification of disease genes and the underlying mutations. It has been especially useful in genetically heterogeneous diseases, such as hearing loss or retinal dystrophies (Choi et al., 2013; Fu et al., 2013; Mutai et al., 2013; Glöckle et al., 2014). We previously developed a targeted next generation sequencing method for Usher syndrome that proved to be highly efficient (Aparisi et al., 2014; Fuster-García et al., 2018).
Although the molecular epidemiology of the Usher syndrome and the distribution of mutations causing the disease among these genes has been well studied in Europe and United States, there is a lack of studies in other regions like Africa or Central and South America.
Here, we show for the first time a molecular landscape of the Usher syndrome in Cuba, and we provide as well a clinical description of all the cases.
Source:
Sage
Media Contacts:
Rosalind Dewar – Sage
Image Source:
The image is credited to the US government.
Original Research: Closed access
“Challenging the diagnosis of ‘Havana Syndrome’ as a novel clinical entity”. Robert E Bartholomew and Robert W Baloh.
Journal of the Royal Society of Medicine doi:10.1177/0141076819877553.
