Presentation and physical therapy management of upper cervical instability in patients with symptomatic generalized joint hypermobility: International expert consensus recommendations

Abstract

Experts in symptomatic generalized joint hypermobility (S-GJH) agree that upper cervical instability (UCI) needs to be better recognized in S-GJH, which commonly presents in the clinic as generalized hypermobility spectrum disorder and hypermobile Ehlers-Danlos syndrome. While mild UCI may be common, it can still be impactful; though considerably less common, severe UCI can potentially be debilitating. UCI includes both atlanto-occipital and atlantoaxial instability. In the absence of research or published literature describing validated tests or prediction rules, it is not clear what signs and symptoms are most important for diagnosis of UCI. Similarly, healthcare providers lack agreed-upon ways to screen and classify different types or severity of UCI and how to manage UCI in this population. Consequently, recognition and management of UCI in this population has likely been inconsistent and not based on the knowledge and skills of the most experienced clinicians. The current work represents efforts of an international team of physical/physiotherapy clinicians and a S-GJH expert rheumatologist to develop expert consensus recommendations for screening, assessing, and managing patients with UCI associated with S-GJH. Hopefully these recommendations can improve overall recognition and care for this population by combining expertise from physical/physiotherapy clinicians and researchers spanning three continents. These recommendations may also stimulate more research into recognition and conservative care for this complex condition.

Keywords: atlantoaxial instability; craniocervical instability; generalized joint hypermobility; hypermobile Ehlers-Danlos syndrome; upper cervical instability.

FIGURE 1

Flow chart describing the screening process for determining that patients have upper cervical instability (UCI) associated with symptomatic generalized joint hypermobility (S–GJH), irritability and identifying yellow flags and red flags. The screening steps can be implemented in any order, using the check boxes on the right to keep track of decisions made at each step. See text and tables for more detailed discussion of each step in the process.

FIGURE 2

Flow chart describing classification of patients for physical examination and intervention, based on outcome of process described in Figure 1. Red flag physical test results (Table 6) can be identified in any group of patients; patients with red flags are automatically classified High Irritability. Irritability may increase during or up to 24 h after the physical exam (dotted arrow labeled “Flare”), shifting patients to a higher irritability group. See text and tables for more detailed discussion of each step in the process.