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"Azola, Alba M."
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Provocation of brachial plexus and systemic symptoms during the elevated arm stress test in individuals with myalgic encephalomyelitis/chronic fatigue syndrome or idiopathic chronic fatigue
2025
Background
We have noted that some adolescents and young adults with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) report difficulty with arms-overhead activities, suggestive of brachial plexus dysfunction or thoracic outlet syndrome (TOS). In the TOS literature, diagnostic maneuvers focus on the provocation of upper limb symptoms (arm fatigue and heaviness, paresthesias, neck and upper back pain), but not on elicitation of systemic symptoms.
Objectives
To estimate the proportion of patients with fatiguing illness who experience local and systemic symptoms during a common maneuver used in evaluating TOS—the elevated arm stress test (EAST).
Methods
Patients were eligible for this retrospective study if they had been referred to the Johns Hopkins Chronic Fatigue Clinic between January 2020 and July 2023 and (a) reported difficulty maintaining arms-overhead postures, (b) were evaluated with an abbreviated one-minute EAST, and (c) had not undergone surgery in the upper limb, neck, or skull base. Modified EAST procedure: patients sat with their arms in a “hands up” or “candlestick” position while opening and closing their hands every 2–3 s repeatedly for 1 min, rather than the customary 3 min. The test was considered abnormal for local symptoms if the participant experienced pain, fatigue, heaviness, paresthesias, warmth or tremulousness in the upper limb, shoulder, neck, head, or upper back. The test was considered abnormal for systemic symptoms if the participant experienced overall fatigue, cognitive fogginess, lightheadedness, racing heart, diaphoresis, dyspnea, overall warmth, or nausea.
Results
Of 154 patients evaluated during the study period, 64 (42%) met the eligibility criteria (61/64 female, median age 18 years [range, 13 to 50]). Of the 64, 50 (78%) had ME/CFS, 13 (20%) had idiopathic chronic fatigue with associated orthostatic intolerance (OI), and one had idiopathic chronic fatigue without OI. Of the 64, 58% had evidence of joint hypermobility. Local symptoms were provoked by EAST in 62/64 (97%) within a median of 20 s. During EAST, 26/64 (41%) reported systemic symptoms (1 had only systemic but no upper limb symptoms), most commonly lightheadedness (19%) and generalized fatigue (11%).
Conclusions
Even with an abbreviated test duration, the EAST maneuver provoked local and systemic symptoms in a substantial proportion of patients with chronic fatigue, OI, and ME/CFS who had reported difficulty with arms-overhead postures. Further studies are needed to explore the prevalence of brachial plexus or TOS symptoms in unselected individuals with ME/CFS or OI, and the proportion with systemic symptoms during and after EAST.
Journal Article
Kinematic Visual Biofeedback Improves Accuracy of Learning a Swallowing Maneuver and Accuracy of Clinician Cues During Training
2017
Submental surface electromyography (ssEMG) visual biofeedback is widely used to train swallowing maneuvers. This study compares the effect of ssEMG and videofluoroscopy (VF) visual biofeedback on hyo-laryngeal accuracy when training a swallowing maneuver. Furthermore, it examines the clinician’s ability to provide accurate verbal cues during swallowing maneuver training. Thirty healthy adults performed the volitional laryngeal vestibule closure maneuver (vLVC), which involves swallowing and sustaining closure of the laryngeal vestibule for 2 s. The study included two stages: (1) first accurate demonstration of the vLVC maneuver, followed by (2) training—20 vLVC training swallows. Participants were randomized into three groups: (a) ssEMG biofeedback only, (b) VF biofeedback only, and (c) mixed biofeedback (VF for the first accurate demonstration achieving stage and ssEMG for the training stage). Participants’ performances were verbally critiqued or reinforced in real time while both the clinician and participant were observing the assigned visual biofeedback. VF and ssEMG were continuously recorded for all participants. Results show that accuracy of both vLVC performance and clinician cues was greater with VF biofeedback than with either ssEMG or mixed biofeedback (
p
< 0.001). Using ssEMG for providing real-time biofeedback during training could lead to errors while learning and training a swallowing maneuver.
Journal Article
The Relationship Between Submental Surface Electromyography and Hyo-Laryngeal Kinematic Measures of Mendelsohn Maneuver Duration
2015
Purpose: The Mendelsohn Maneuver (MM) is a commonly prescribed technique that is taught to individuals with dysphagia to improve swallowing ability. Due to cost and safety concerns associated with videofluoroscopy (VFS) use, submental surface electromyography (ssEMG) is commonly used in place of VFS to train the MM in clinical and research settings. However, it is unknown whether ssEMG accurately reflects the prolonged hyolaryngeal movements required for execution of the MM. The primary goal of this study was to examine the relationship among ssEMG duration, duration of laryngeal vestibule closure, and duration of maximum hyoid elevation during MM performance. Method: Participants included healthy adults and patients with dysphagia due to stroke. All performed the MM during synchronous ssEMG and VFS recording. Results: Significant correlations between ssEMG duration and VFS measures of hyo-laryngeal kinematic durations during MM performance ranged from very weak to moderate. None of the correlations in the group of stroke patients reached statistical significance. Conclusion: Clinicians and researchers should consider that the MM involves novel hyo-laryngeal kinematics that may be only moderately represented with ssEMG. Thus, there is a risk that these target therapeutic movements are not consistently being trained.
Journal Article
Swallowing Changes in Community-Dwelling Older Adults
2018
Older adults may evidence changes in swallowing physiology. Our goals were to identify dysphagia risk in community-dwelling older adults with no history of dysphagia, and to compare swallowing physiology and safety between older and younger adults. Thirty-two older adults with no history of dysphagia were prospectively recruited and completed the Dysphagia Handicap Index (DHI), two trials of a 3 oz. swallow screen, and videofluoroscopy (VFSS). Self-ratings of swallowing function were compared to published norms by paired t tests, and multivariate logistic regression models were generated to determine whether these ratings and VFSS analysis of swallowing function were associated with failure of one or both swallow screen trials. Archived VFSS of 33 younger adults were compared to older adults with Wilcoxon rank-sum tests. The DHI scores of older adults were higher than published non-dysphagic adults but lower than dysphagic adults. Older participants with greater Oral Residue scores were more likely to fail both swallow screen trials. Older adults received higher median MBSImP™© scores for select pharyngeal components than younger adults. The two age groups did not differ on Penetration-Aspiration Scale scores, and no aspiration was observed. Measures of swallowing in older individuals may reflect age-related sensory and motor changes in the context of functional swallowing and adequate airway protection.
Journal Article
Improvement in Upper Limb and Systemic Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) Symptoms After Surgical Treatment of Neurogenic Thoracic Outlet Syndrome
by
Azola, Alba M
,
Sroge, Sally C
,
Christoforou, Maritsa E
in
Cardiac/Thoracic/Vascular Surgery
,
Physical Medicine & Rehabilitation
2025
Thoracic outlet syndrome (TOS) is characterized by compression of nerves or blood vessels as they pass through the scalene triangle and the costoclavicular space, and under the pectoralis minor. Common symptoms include arm fatigue and heaviness, paresthesias, and neck and upper back pain, provoked by arm extension or elevation. We have recently reported that some myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) patients report symptoms suggestive of TOS, specifically with respect to overhead activity, but there is uncertainty whether this overlap in symptoms is more related to ME/CFS itself or a direct contribution by TOS. This case report describes an ME/CFS patient diagnosed with TOS, who experienced major decreases in many expected and unexpected symptoms after bilateral TOS surgery. A 19-year-old female patient with ME/CFS and the hypermobile Ehlers-Danlos syndrome (hEDS) developed progressive symptoms of numbness and tingling in the upper limbs, which did not improve after two months of physical therapy. The patient elected to undergo the rib resection with neurolysis and scalenectomy surgery on her left side. Due to the success in the reduction of symptoms, she elected to undergo the same procedure on the right side three months later. By eight weeks after the second surgery, the patient had experienced an expected complete resolution of upper limb numbness and tingling. She also reported a complete resolution of migraines, occipital neuralgia, vertigo, and visual disturbances, along with a marked improvement in cognitive fogginess and lightheadedness. This case report highlights the potential for marked improvements in clinical function after recognition and surgical treatment of TOS in a patient with comorbid hEDS and ME/CFS. In addition to expected improvement in upper limb symptoms and the resolution of occipital headaches, our patient noted improvement in systemic symptoms of lightheadedness, cognitive dysfunction, and visual disturbances. This experience suggests that those with hEDS and ME/CFS should be more carefully screened for brachial plexus dysfunction. Conversely, ascertainment of systemic symptoms may enhance the diagnosis of TOS and the items assessed in surgical treatment outcome studies.
Journal Article