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Ultrasound-guided glenohumeral joint injections for
shoulder pain in ALS: A Case Series
Katherine M. Burke ( Katherine.Burke@mgh.harvard.edu )
Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital, Harvard Medical School
Amy S. Ellrodt
Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital, Harvard Medical School
Benjamin C. Joslin
Northwestern University Feinberg School of Medicine
Pia P. Sanpitak
Northwestern University Feinberg School of Medicine
Claire MacAdam
Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital, Harvard Medical School
Prabhav Deo
Shirley Ryan AbilityLab
Kevin Ozment
Shirley Ryan AbilityLab
Cristina Shea
Spaulding Rehabilitation Hospital
Stephen A. Johnson
Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital, Harvard Medical School
Doreen Ho
Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital, Harvard Medical School
Samuel K. Chu
Shirley Ryan AbilityLab
Colin K. Franz
Shirley Ryan AbilityLab
Ashwin N. Babu
Massachusetts General Hospital, Sports Medicine
Sabrina Paganoni
Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital, Harvard Medical School
Research Article
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Keywords: Amyotrophic Lateral Sclerosis, Motor Neuron Disease, Shoulder Pain, Frozen Shoulder,
Adhesive Capsulitis
Posted Date: September 15th, 2022
DOI: https://doi.org/10.21203/rs.3.rs-1990390/v1
License: This work is licensed under a Creative Commons Attribution 4.0 International License.
Read Full License
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Abstract
INTRODUCTION: Shoulder pain is a common secondary impairment for people living with ALS (PALS).
Decreased range of motion (ROM) from weakness can lead to shoulder pathology, which can result in
debilitating pain. Shoulder pain may limit PALS from participating in activities of daily living and may
have a negative impact on quality of life. This case series explores the ecacy of glenohumeral joint
injections for management of shoulder pain due to adhesive capsulitis in PALS.
METHODS: PALS with shoulder pain were referred to sports medicine certied physiatrists for diagnostic
evaluation and management. They completed the Revised ALS Functional Rating Scale and a
questionnaire asking about their pain levels and how it impacts sleep, function, and quality of life at
baseline pre-injection, one-week post-injection, one-month and three-months post-injection.
RESULTS: We present 5 cases of PALS who were diagnosed with adhesive capsulitis and underwent
glenohumeral joint injections. Though only one PALS reported complete symptom resolution, all had at
least partial symptomatic improvement during the observation period. No complications were observed.
CONCLUSIONS: PALS require a comprehensive plan to manage shoulder pain. Glenohumeral joint
injections are safe and effective for adhesive capsulitis in PALS, but alone may not completely resolve
shoulder pain. Additional therapies to improve ROM and reduce pain should be considered.
Introduction
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease.1 Management of ALS is largely
focused on symptom management as current FDA-approved treatments to slow disease progression
have modest ecacy.1
ALS symptoms include muscle weakness and atrophy, reduced range of motion, spasticity, and cramping
leading to progressive disability. Pain is a common secondary complication of ALS from musculoskeletal
dysfunction due to limited mobility, loss of range of motion, and diculty with positioning in bed, chairs,
or wheelchairs.2 Progressive, often non-uniform, weakness likely also contributes by disrupting agonist-
antagonist muscle pair equilibrium leading to increased stress on the joint and surrounding structures.
Pain negatively affects quality of life for both PALS and their caregivers.2,3,4,5,6,7 The most commonly
reported loci of pain in PALS are the lower back, neck, legs, and shoulders.2,8 Shoulder pain is reported by
roughly 20% of the total ALS population.8 Many PALS develop shoulder pain due to weakness of the peri-
scapular muscles with subsequent loss of range of motion, subluxation, and development of shoulder
pathology, including adhesive capsulitis or ‘frozen shoulder’.
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Adhesive capsulitis is a complex disorder that involves pain and restricted range of motion (ROM) of the
shoulder9 and can lead to considerable disability. The capsule surrounding the glenohumeral (GH) joint
stiffens and becomes inamed, which results in decreased ROM at the shoulder. There are overlapping
stages of adhesive capsulitis. In the earlier stages, patients present with severely restricted ROM and
signicant pain, often in the absence of injury. In the later stages, restrictions in ROM remain, but with
improvements in pain. As ROM and pain improve, there may be continued evidence of a tight joint space,
but no evidence of synovitis.10 Adhesive capsulitis is largely a clinical diagnosis, with any imaging done
to exclude other potential diagnoses.9
For individuals without ALS, the management of adhesive capsulitis involves exercises and supervised
physical therapy focused on gentle ROM and periscapular stabilization.First-line medications typically
prescribed to treat pain in ALS are non-steroidal anti-inammatory agents (NSAIDs) and non-opioid
analgesics.4,12 Early corticosteroid injection to the GH joint should be considered for moderate to severe
symptoms,13,14as there is evidence suggesting improved outcomes with earlier steroid injection.15This
in-clinic procedureis fast and safe. It is routinely performed in the seated position, which is preferable for
PALS who may be in a power wheelchair and/or have limited tolerance of the supine position. Adverse
effects such as bleeding or infection are rare when performed with proper clean technique. In cases where
conservative measures are ineffective, surgical interventions such as arthroscopic release and
manipulation under anesthesia are considered, especially in the early stages when the pain can be
debilitating.16,17
To the best of our knowledge, there is no evidence from controlled trials that would substantiate a
standard best practice for addressing shoulder pain in people with ALS. Thus, management of shoulder
pain has long been based on individual clinician’s experience and preferences with very little empirically
derived evidence to support the implementation of one therapeutic method over another. When a PALS is
presenting with progressive shoulder pain, accompanying restrictions in ROM, and no history of trauma,
referral to physiatry can help assess the pain generator (e.g., adhesive capsulitis versus other shoulder
pathology) and help guide intervention. There is growing evidence to support the use of GH steroid
injections for adhesive capsulitis for those without ALS13,14,15,16,17 but to our knowledge, no evidence for
its use in individuals with ALS.
The purpose of this study was to assess the ecacy of ultrasound-guided GH joint injections for treating
shoulder pain due to adhesive capsulitis in PALS. We present a case series of 5 patients referred for
physiatric evaluation and consideration of GH joint injections as part of their clinical care. Participants
completed surveys pre- and post-injections regarding their pain levels and impact on function.
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Methods
Participants were recruited from the Sean M. Healey & AMG Center for ALS at Massachusetts General
Hospital and the Northwestern University Feinberg School of Medicine ALS Clinic. Eligible participants
were informed of the study and study staff consented interested participants. Both the Mass General
Brigham Institutional Review Board (IRB) and the Northwestern IRB approved this study.
Participants were asked to participate in this study if they had ALS and a clinical suspicion of adhesive
capsulitis (pain with restricted ROM, without history of trauma). Participants were seen by physiatrists
certied in Sports Medicine by the American Board of Physical Medicine and Rehabilitation for diagnostic
conrmation and consideration of GH steroid injections. The physiatrists completed a history, physical
and ultrasound examinations to assess likelihood of adhesive capsulitis versus other common shoulder
pathologies (such as rotator cuff or biceps tendinopathy, periscapular myofascial pain, etc.). Once
clinical diagnosis of adhesive capsulitis was established, ultrasound-guided GH joint injection was
performed. For Cases 1-3, a solution of 4mL of 1% Lidocaine and 1mL (40mg) of Kenalog was injected
into the affected joints. For Cases 4 and 5, a solution of 2 mL of 1% Lidocaine, 2 mL 0.25% Bupivacaine,
and 1mL (40mg) of Kenalog was injected into the involved joints. Differences were due to preference of
the physiatrist performing the intervention. Study duration was from May 2019 through August 2021.
Enrollment was limited by the COVID-19 pandemic, as sites had temporary holds on observational
studies, and patient care shifted to telemedicine visits.
Participants were asked to complete the revised ALS Functional Rating Scale (ALSFRS-R) and a
questionnaire that assessed pain, sleep, function, and quality of life (Supplementary Materials) at
baseline prior to undergoing the GH joint injections. They were then asked to complete the same
questionnaires at one week post injection and one month post injection. There was an optional phone
call at 3 months to complete the same questionnaires.
Electronic medical record review was available for all participants, from which information regarding their
ALS diagnosis, disease duration and current functional status was gathered.
Results
Of 9 participants enrolled in the study, 3 initially agreed but ultimately declined the referral for GH joint
injections and were provided with ROM and stretching exercises by clinic physical therapists. Follow-up
of these individuals was not pursued as part of this study. Another participant was seen by physiatry:
diagnostic evaluation pointed to myofascial pain with muscle spasms as the most likely pain generator.
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For this reason, the participant underwent bilateral trapezius, infraspinatus, levator scapulae trigger point
injections rather than GH joint injections. This participant did have resolution of his shoulder pain 18
days post injection, but this was not sustained 2 months post-injection. We report on the remaining 5
participants who were diagnosed by sports medicine certied physiatrists with adhesive capsulitis and
did undergo ultrasound guided GH joint steroid injections. None of the participants experienced
procedure-related complications. Average pain scores and impact on sleep and quality of life for each
participant are shown in Table 1. Complete data for each participant are shown in Supplementary Tables
1-5.
Case 1.
Participant was a 41-year-old female with limb onset sporadic ALS who used a wheelchair for mobility
and was dependent on caregivers for activities of daily living (ADLs). ALS symptom onset was over three
years prior with left hand weakness. She presented with atraumatic bilateral shoulder pain, right greater
than left, with passive range of motion restrictions. She was taking NSAIDs as needed and undergoing
physical therapy with shoulder range of motion and stretching exercises. Physiatry evaluation conrmed
the diagnosis of adhesive capsulitis and she underwent right GH joint injection with ultrasound guidance.
Post injection, she demonstrated improvements in shoulder pain, which were sustained at the 3-month
visit, as well as sustained improvements in sleep, function, and quality of life. (Supplementary Table 1)
Case 2.
Participant was a 42-year-old male with limb onset sporadic ALS who was ambulatory with single point
cane and had modied independence with ADLs. ALS onset was over two years prior with lower
extremity weakness. He presented with atraumatic right shoulder pain that started a few months prior,
with limited ROM. He was not taking any medications and was not participating in physical therapy. He
had been prescribed shoulder range of motion and stretching exercises at multidisciplinary clinic, but was
unable to perform due to pain. Physiatry evaluation conrmed the diagnosis of adhesive capsulitis and
he underwent right GH joint injection with ultrasound guidance. Post injection, he demonstrated
improvements in shoulder pain, which were sustained at the 1-month visit, as well as improvements in
sleep, function and quality of life (Supplementary Table 2). He initiated a stretching program for bilateral
shoulders post-injection.
Case 3.
Participant was a 57-year-old male with limb onset sporadic ALS. ALS symptom onset was over three
years prior with lower extremity weakness. He was ambulatory without an assistive device and required
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assistance with ADLs due to bilateral upper extremity weakness. He presented with bilateral atraumatic
shoulder pain, left greater than right. He had been prescribed shoulder range of motion and stretching
exercises at multidisciplinary clinic, but was unable to perform due to pain. He was not taking any pain
medications. Physiatry evaluation conrmed the diagnosis of adhesive capsulitis and he underwent
bilateral GH joint injections with ultrasound guidance. Overall pain was improved in bilateral shoulders
post-injection, and this was sustained at the 3-month visit. At one month, pain was not impacting sleep,
function, and quality of life, but this was not sustained at three-months (Supplementary Table 3). He was
stretching at the 1-month mark, but this was not sustained at 3-months.
Case 4.
Participant was a 49-year-old male with limb onset sporadic ALS. He was power wheelchair dependent
for community mobility and used either a cane or power wheelchair for household mobility. He had
modied independence with ADLs. He presented with 6 months of progressively worsening atraumatic
bilateral anterolateral shoulder pain and stiffness, left greater than right. He had passive and active ROM
restrictions bilaterally. He had been taking ibuprofen as needed for several years. He had been prescribed
shoulder range of motion and stretching exercises at multidisciplinary clinic, but was not able to comply
due to pain. He underwent bilateral GH joint injections with ultrasound guidance. He exhibited
improvements in impact of shoulder pain that lasted only about 1 month, but improvements on his
quality of life and function were largely sustained at the 3-month follow-up (Supplementary Table 4).
Case 5.
Participant is a 58-year-old female with limb onset sporadic ALS. She was ambulatory and required
assistance with ADLs due to upper extremity weakness. She presented with atraumatic left shoulder pain
and stiffness for 2 months. She managed her pain with nightly dose of ibuprofen and had not yet started
physical therapy prior to initial referral for injection. She had limited active and passive shoulder ROM.
She underwent left GH joint injections with ultrasound guidance. In early and late follow up, the patient
reported resolution of shoulder pain and it was no longer impacting sleep. This allowed her to engage in
shoulder home exercise program provided by ALS interdisciplinary clinic occupational therapist with
assistance from her family. Resolution of pain and impact on sleep, function, and quality of life were
sustained at the 3-month visit (Supplementary Table 5).
Table 1. Pain and Impact on Sleep, Function, and Quality of Life
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Case 1 Case 2 Case 3 Case 4 Case 5
Average Pain 0-10 (R/L)
pre-injection (days from
baseline) 7/7 (-19) 7/0 (-22) 3/4 (-3) 6/5 (-18) 1/7 (0)
1st follow-up (days from
baseline) 4/7 (7) 2/0 (7) 0/0 (7) 1/2 (10) 0/0
(11)
2nd follow-up (days from
baseline) 5/7 (28) 2/0 (21) 2/3 (25) 4/4 (31) 0/0
(28)
3rd follow-up (days from
baseline) 0/0 (74) not
collected 0/0 (87) 6/6 (87) 0/0
(87)
Max Pain 0-10 (R/L)
pre-injection 9/9 9/0 4/5 8/8 1/8
1st follow-up 8/7 4/0 5/5 4/4 0/0
2nd follow-up 8/8 5/0 2/3 8/8 0/0
3rd follow-up 5/5 not
collected 10/10 8/6 0/0
My shoulder pain keeps me from getting enough sleep at night.
pre-injection Often Sometimes Rarely Always Always
1st follow-up Never Rarely Rarely Never Never
2nd follow-up Never Sometimes Never Never Never
3rd follow-up Never not
collected Never Never Never
My shoulder pain limits my ability to complete daily hygiene.
pre-injection Always Often Always Always Never
1st follow-up Often Rarely Always Never Never
2nd follow-up Sometimes Sometimes Never Never Never
3rd follow-up Rarely not
collected Always Never Never
My shoulder pain limits my ability to get dressed each day.
pre-injection Always Often Always Always Always
1st follow-up Often Sometimes Always Rarely Never
2nd follow-up Sometimes Sometimes Never Rarely Never
3rd follow-up Always not Always Sometimes Never
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collected
My shoulder pain limits me from leaving the house for other activities.
pre-injection Always Rarely Often Sometimes Never
1st follow-up Never Rarely Always Rarely Never
2nd follow-up Never Sometimes Never Rarely Never
3rd follow-up Never not
collected Rarely Rarely Never
My shoulder pain has a negative impact on my quality of life.
pre-injection Always Often Always Always Never
1st follow-up Often Sometimes Sometimes Never Never
2nd follow-up Often Sometimes Never Never Never
3rd follow-up Always not
collected Always Never Never
Discussion
In this case series, we present empiric evidence for benet from GH joint steroid injections for PALS who
present with shoulder pain in the setting of adhesive capsulitis. All study participants presented here
reported improvement in their pain levels and positive impact on sleep, function, or quality of life. One of
the participants (Case 5) reported complete resolution of her shoulder pain. There were no adverse events
reported by the participants in this study. These results demonstrate the safety of, and suggest a role for,
ultrasound-guided GH joint steroid injections for PALS with adhesive capsulitis, though additional
measures may be needed for further pain management.
The development of shoulder pain from adhesive capsulitis in PALS is likely a secondary effect of their
disease resulting from multiple factors such as decreased shoulder muscle strength, muscle atrophy, and
reduced activity levels. The progressive lack of motion may contribute to inammation and tightening of
the joint capsule. Similar pathology has been reported in both stroke and Parkinson’s disease where there
is motor dysfunction involving the shoulder.18,19 Motion is critical to maintain and restore ROM.13,16 For
PALS with shoulder pain, stretching and range of motion exercises are recommended, and strategies to
increase compliance can be helpful.20 Shoulder approximation sleeves can also be used to help support
the shoulder and manage pain, as well as modalities such as transcutaneous electrical nerve stimulation
(TENS) devices. However, while these tools and strategies have shown promise, these interventions are
not always enough to completely relieve pain or improve function.20Unfortunately, there may be limited
ability to complete the recommended exercises due to disease progression or pain. Often the shoulder will
become quite painful and will limit ability to participate in ADLs, including daily hygiene, and may impact
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sleep quality and overall quality of life. When pain has become a limiting factor, the provider is
challenged to make appropriate recommendations for adequate pain control and to optimize the person’s
ability to participate in ADLs. This study highlights the need for multifaceted approach, which may
include GH joint injections, as well as education on the cause of shoulder pain and importance of a long-
term stretching program to maintain a functional ROM to minimize risk for re-freezing or ongoing pain.
Referral to musculoskeletal specialists for consideration for steroid injections should be considered for
PALS with shoulder pain. The procedure offers the advantages of being a fast, safe, in-oce procedure
that requires no ionizing radiation or sedation. Evidence in non-ALS populations shows improved pain
relief with steroid injections compared to manual therapy and exercise without injection.21 Shoulder
injections have also been shown to be benecial for hemiplegic shoulder pain in patients status post
stroke.22With pain relief from the injections, the person may be able to tolerate ROM and stretching
exercises, which will restore motion and in turn may further improve pain, and help restore function and
quality of life. Appropriate pain relief with localized steroid injections also offers a strategy to reduce
reliance on pain medications with systemic medications with potential GI, renal and neurologic side
effects.
It is important to minimize delays in referrals to musculoskeletal specialistsgiven the emerging evidence
of improved outcomes with earlier steroid injections in people without ALS, as well as improved ecacy
compared to manual therapy with exercise.14,21 While early diagnosis and intervention is ideal, our cohort
suggests that GH joint injection for adhesive capsulitis likely benets PALS across the progression of
ALS. If employed in early ALS, GH injection may allow a person to maintain ROM, functional
independence, and participation in active physical therapy programs. In later-stage ALS, GH injection may
offer improved passive ROM and caregiver ability to assist with ADLs, such as dressing and bathing.
Throughout all ALS stages, the pain-reducing effect of GH injection may have benets on activity, sleep
and mood.
Our study is limited by the small number of participants and case series design. The ongoing COVID-19
pandemic also limited participation, such that we were not able to collect range of motion measurements
as the participants completed their post injection visits over the telephone, and many did not present to
clinic again during the study period due to COVID-19 restrictions. Further research is also needed to
compare outcomes between those who choose not to have the injections to those who do choose this
intervention. Finally, as more evidence emerges regarding specic injection techniques and steroid dose
for people without ALS, it will be important to explore these same questions in the ALS population.
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This case series supports the safety and ecacy of GH joint injections for adhesive capsulitis in PALS.
Larger studies to determine prevalence of adhesive capsulitis versus other pathologies and to optimize
comprehensive management plans aimed at complete shoulder pain resolution are needed.
Conclusions
PALS require a comprehensive plan to manage shoulder pain. Referrals to musculoskeletal specialists are
helpful to determine the underlying cause of pain. Glenohumeral joint injections are safe and effective for
adhesive capsulitis in PALS, but alone may not completely resolve shoulder pain. Additional therapies to
improve ROM and reduce pain should be considered.
Declarations
Ethics approval and consent to participate:Both the Mass General Brigham Institutional Review Board
(IRB) and the Northwestern IRB approved this study. Eligible participants were informed of the study and
study staff consented interested participants.
Consent for publication: Not Applicable
Availability of data and materials:All data generated or analyzed during this study are included in this
manuscript and its supplementary information les.
Competing Interests:
Doreen Ho – reports the following disclosures: compensation for consulting from Alexion
pharmaceuticals
Sabrina Paganoni – reports the following disclosures: Research grants from Amylyx Therapeutics,
Revalesio Corporation, Alector Therapeutics, UCB, Biohaven Pharmaceuticals, Clene Nanomedicine,
Prilenia Therapeutics, Seelos Therapeutics. Research grants from the ALS Association, the American
Academy of Neurology, the CDC, ALS Finding a Cure, the Salah Foundation, the Spastic Paraplegia
Foundation, the Muscular Dystrophy Association, I AM ALS, Tambourine, Target ALS, Columbia University,
the Cullen Education and Research Fund. Site PI: Alector Therapeutics, Cytokinetics, Inc., Anelixis
Therapeutics. Institutional consulting agreements with Amylyx Therapeutics, Frequency Therapeutics,
Sola Biosciences. Personal consulting agreements with Cytokinetics, Inc., Arrowhead Pharmaceuticals,
Orthogonal Neurosciences. Honoraria from Medscape
All other authors report no disclosures.
Funding: No targeted funding.
Authors’ Contributions:
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Katherine M. Burke – concept and design; data collection; data analysis; drafting of manuscript; critical
review of manuscript
Amy S. Ellrodt – concept and design; data collection; data analysis, critical review of manuscript
Benjamin C. Joslin – concept and design; data collection; data analysis, drafting of manuscript, critical
review of manuscript
Pia P. Sanpitak - data collection; data analysis, critical review of manuscript
Claire MacAdam - data analysis and critical review of manuscript
Prabhav Deo - data collection; data analysis, critical review of manuscript
Kevin Ozment - data collection; data analysis, critical review of manuscript
Cristina Shea - data analysis and critical review of manuscript
Stephen A. Johnson - data analysis and critical review of manuscript
Doreen Ho – data analysis and critical review of manuscript
Samuel K. Chu - data collection; data analysis; critical review of manuscript
Colin K. Franz - concept and design; data collection; data analysis; drafting of manuscript; critical review
of manuscript
Ashwin N. Babu- concept and design; data collection; data analysis; critical review of manuscript
Sabrina Paganoni - concept and design; data collection; data analysis; critical review of manuscript
Acknowledgements
This research would not be possible without the contributions from our patients taking the time to
complete the surveys and provide feedback on the impact of the shoulder injections.
Abbreviations
ADLs – activities of daily living
ALS – amyotrophic lateral sclerosis
ALSFRS-R – amyotrophic lateral sclerosis functional rating scale - revised
GH – glenohumeral
IRB – Institutional Review Board
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NSAIDS – non-steroidal anti-inammatory agents
PALS – people living with ALS
ROM – range of motion
TENS – transcutaneous electrical nerve stimulation
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Supplementary Files
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SupplementaryMaterial2022.08.11.docx
