LAUSR

Dear Sir, We read with interest the article by Palmer [1] (Palmer, S., Denner, E., Riglar, M., Scannell, H., Webb, S. and Young, G., 2020. Quantitative measures of tissue mechanics to detect hypermobile Ehlers-Danlos syndrome and hypermobility syndrome disorders: a systematic review.Clinical Rheumatology, 39(3), pp.715–725). Palmer et al. question the validity of the Beighton Score, which assesses hyper-extensibility in a limited number of joints, but does not include the most affected joints. Furthermore, the present criteria for hEDS (EDS—hypermobility type) and HSD (the hypermobility spectrum disorders) do not address the wider issues of connective tissue pathology. Palmer et al. reviewed 203 potentially relevant studies, of which 4 were chosen for systematic review; three described at least one measure of tissue mechanics that differentiated subjects with hEDS/HSD from healthy controls. These included assessment of skin, muscle and tendon. We draw attention to another measure of tissue mechanics that of the measureable hyper-extensibility of craniocervical ligaments causing “floppy head syndromes” in this population. To address the differentiation of pathological hyperextensibility from benign hypermobility, a consensus conference was sponsored jointly by the Office of the Medical Commissioner of the National Health Service, Bobby Jones Chiari Syringomyelia Foundation, the Ehlers-Danlos Society and Lady TrishMalloch-Brown. Lectures and arguments were put forward to address the presentation and diagnosis of craniocervical instability and ventral brainstem compression in the EDS population. A robust “round table” discussion enjoined by representatives of neurosurgery, neurology, neuro-radiology, genetics, rheumatology and physiatry reached the following consensus: