Study of Sleep Disturbance in Ehlers-Danlos

Sleep Disturbance in Ehlers-Danlos Syndromes: Related to Chronic Pain or an Independent Entity?

K.W. Mandel, B. Griswold, C.A. Francomano, N.B. McDonnell
Presented at the annual meeting of The American Society of Human Genetics, October 10, 2006, New Orleans, Louisiana.


Patients with Ehlers-Danlos Syndromes (EDS) often complain of disturbed and non-refreshing sleep.

There is a paucity of data available on the etiology of this complaint in EDS. Since many patients also suffer from chronic musculoskeletal pain, we hypothesized that the reported sleep abnormalities are related to the chronic pain syndrome.

Multiple sleep and pain assessment tools were administered to sixty five consecutive patients with a diagnosis of EDS enrolled in the National Institutes of Aging protocol 2003-086 on the natural history of hereditary disorders of connective tissue.

Data was collected utilizing the Epsworth Sleepiness Scale (ESS), Pittsburgh Sleep Inventory (PSI) as well as a sleep questionnaire designed to elucidate the specific problem areas such as difficulty falling or remaining asleep, snoring, early morning awakening and non-refreshing sleep.

Pain was assessed through the Brief Pain Inventory (BPI) and review of systems data focusing on musculoskeletal and generalized pain. Results indicate that 33/65 (50%) of patients had an ESS scale > 9, and 49/65 (75%) a PSI Quotient > 5, suggesting the presence of a sleep disorder. Almost all patients (62/65; 95%) had pain in at least one location, and 29/65 (44 %) reported chronic generalized pain.

Of the patients with ESS > 9, a complaint of generalized pain was present in 15/33 (45 %). Of the 49 patients with PSIQ > 5, generalized pain was present in 24 (48 %).

The discordance of sleep disturbance with chronic generalized pain in EDS suggests that the pathogenesis of sleep complaints in this disorder may be unrelated to the pain syndrome, at least in part. Further investigation regarding the etiology of this phenomenon in EDS, using formal sleep studies, is warranted.