Connective tissue, Ehlers–Danlos syndrome(s), and head and cervical pain

Self Cite

372

348

The hypermobile type of Ehlers-Danlos syndrome (hEDS) is likely the most common hereditary disorder of connective tissue. It has been described largely in those with musculoskeletal complaints including joint hypermobility, joint subluxations/dislocations, as well as skin and soft tissue manifestations. Many patients report activity-related pain and some go on to have daily pain. Two undifferentiated syndromes have been used to describe these manifestations-joint hypermobility syndrome and hEDS. Both are clinical diagnoses in the absence of other causation. Current medical literature further complicates differentiation and describes multiple associated symptoms and disorders. The current EDS nosology combines these two entities into the hypermobile type of EDS. Herein, we review and summarize the literature as a better clinical description of this type of connective tissue disorder.

Section: Clinical Descriptionmentioning

confidence: 99%

Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history

Tinkle

Castori²,

Berglund

et al. 2017

Self Cite

372

348

“…Headaches, in particular migraine, was higher in prevalence and more often more disabling than the control population (N = 232). Castori et al [] reported the incidence of headaches in no less than 1/3 of patients with EDS with migraines as the most common headaches. Other possible causes of headaches include tension‐type headache, new daily persistent headache, temporomandibular joint dysfunction (TMD), Chiari malformation, cervicogenic, neck–tongue syndrome, and medication related [Neilson and Martin, ].…”

Section: Literature Reviewmentioning

confidence: 99%

Pain management in the Ehlers–Danlos syndromes

Chopra¹,

Tinkle²,

Hamonet³

et al. 2017

148

172

Chronic pain in the Ehlers–Danlos syndromes (EDS) is common and may be severe. According to one study, nearly 90% of patients report some form of chronic pain. Pain, which is often one of the first symptoms to occur, may be widespread or localized to one region such as an arm or a leg. Studies on treatment modalities are few and insufficient to guide management. The following is a discussion of the evidence regarding the underlying mechanisms of pain in EDS. The causes of pain in this condition are multifactorial and include joint subluxations and dislocations, previous surgery, muscle weakness, proprioceptive disorders, and vertebral instability. Affected persons may also present with generalized body pain, fatigue, headaches, gastrointestinal pain, temporomandibular joint pain, dysmenorrhea, and vulvodynia. Pain management strategies may be focused around treating the cause of the pain (e.g., dislocation of a joint, proprioceptive disorder) and minimizing the sensation of pain. Management strategies for chronic pain in EDS includes physical therapy, medications, as well as durable medical equipment such as cushions, compressive garments, and braces. The different modalities are discussed in this paper. © 2017 Wiley Periodicals, Inc.

“…EDS patients commonly suffer a variety of headache types [Jacome, ; Martin and Neilson, ; Castori et al, ]. These include headaches due to migraines, muscle tension, intracranial hypertension, craniocervical instability, and cervical spine disorders, temporomandibular joint disease, carotid dissection, and other physical conditions.…”

Section: Headache In Ehlers–danlos Syndromementioning

confidence: 99%

“…Migraine, common in the general population, is more prevalent in women [Nappi and Nappi, ]. Migraine is also more prevalent among EDS which also has a female predilection [Bendik et al, ; Castori and Voermans, ; Castori et al, ]. Therefore, EDS may be considered a risk factor for migraine.…”

Section: Headache In Ehlers–danlos Syndromementioning

confidence: 99%

Neurological and spinal manifestations of the Ehlers–Danlos syndromes

Henderson¹,

Austin²,

Benzel³

et al. 2017

182

174

The Ehlers–Danlos syndromes (EDS) are a heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin extensibility, and tissue fragility. This communication briefly reports upon the neurological manifestations that arise including the weakness of the ligaments of the craniocervical junction and spine, early disc degeneration, and the weakness of the epineurium and perineurium surrounding peripheral nerves. Entrapment, deformation, and biophysical deformative stresses exerted upon the nervous system may alter gene expression, neuronal function and phenotypic expression. This report also discusses increased prevalence of migraine, idiopathic intracranial hypertension, Tarlov cysts, tethered cord syndrome, and dystonia, where associations with EDS have been anecdotally reported, but where epidemiological evidence is not yet available. Chiari Malformation Type I (CMI) has been reported to be a comorbid condition to EDS, and may be complicated by craniocervical instability or basilar invagination. Motor delay, headache, and quadriparesis have been attributed to ligamentous laxity and instability at the atlanto‐occipital and atlantoaxial joints, which may complicate all forms of EDS. Discopathy and early degenerative spondylotic disease manifest by spinal segmental instability and kyphosis, rendering EDS patients prone to mechanical pain, and myelopathy. Musculoskeletal pain starts early, is chronic and debilitating, and the neuromuscular disease of EDS manifests symptomatically with weakness, myalgia, easy fatigability, limited walking, reduction of vibration sense, and mild impairment of mobility and daily activities. Consensus criteria and clinical practice guidelines, based upon stronger epidemiological and pathophysiological evidence, are needed to refine diagnosis and treatment of the various neurological and spinal manifestations of EDS. © 2017 Wiley Periodicals, Inc.