O09 Recurring brachial plexopathy- the zebra among the horses

Abstract Case report - Introduction A case study of a teenage boy presenting with severe upper limb pain and recurring loss of upper limb function with no clear mechanism of injury. His progress in therapy was initially as expected; however, symptoms would recur despite consistency and compliance with treatment from the patient. This led to a referral for further investigations where a diagnosis of a rare inflammatory neurological condition was made. This case study is relevant for paediatric physiotherapists working in non-inflammatory, musculoskeletal and pain services as it highlights a lesser-known pathology that presents in a similar way to a more common condition. Case report - Case description M is a 13-year-old boy that presented with a 5-week history of stabbing pains followed by loss of motor function and sensation in his right arm after swinging a remote. A diagnosis of brachial nerve plexopathy had been suggested. M had been diagnosed with Hypermobile Ehlers-Danlos Syndrome (hEDS) but was otherwise fit and well with no significant birth, developmental or family history. He experienced similar episodes of loss of motor function throughout the entire right upper limb following an episode of acute pain aged 4 and aged 12. The episodes were presumed to be a brachial plexus injury following a shoulder subluxation; however, there was no real mechanism of injury to suggest this and symptoms self-resolved after several months in both instances. Age 8 he lost function and sensation in the left arm after a minor pulled elbow, he underwent elbow surgery at another centre to help restore the function of the left arm; however, function didn’t return for approximately 1 year. On examination he had diminished reflexes throughout the right upper limb and reduced sensation along a C3-8 & T1 distribution. He had a correctable thoracic spine kyphosis with significant medial boarder scapula winging on the right. His right shoulder sat lower than the left and he had muscle atrophy at right supraspinatus, infraspinatus, and serratus anterior and deltoid with tight pectoral muscles. He was compensating using upper trapezius to achieve 90—100 degrees of shoulder flexion and abduction with 2/5 muscle power. His elbow muscle strength was reduced to 4/5 in all movements on the right. He could only actively extend his right wrist to 30 degrees and only had flickers of active radial deviation. He lacked active finger extension in digits 2-5 and had 0/5 muscle activity at the right thumb. Case report - Discussion M underwent exercise therapy with a focus on regaining scapula control in lying and isometric rotator cuff strengthening as he had such significant wasting and was unable to control the upper limb in sitting. We also worked on improving his thoracic spine posture and on active assisted finger and wrist exercises to prevent contractures. I initially provided a sling to be worn at school and in busy environments to prevent any subluxations in view of his significant rotator cuff weakness and history of hEDS. The sling also served as a thoracic posture reminder for M. After just 2—3 weeks of input and initially making gains in strength and function, M had an episode of severe pain in the right shoulder followed by worsening motor and sensory symptoms. The recurrent nature of episodes and the weak mechanism of injury, led me to discuss M with a consultant. The consultant referred M to genetics where it was discovered he had idiopathic neuralgic amyotrophy (INA; also known as Parsonage—Turner Syndrome), a rare inflammatory neurological disorder. M had the classic signs and symptoms of INA but as he had presented to various different clinicians and centres with each episode a correlation wasn’t made until this latest presentation to pain clinic Case report - Key learning points The insubstantial mechanism of injury for his current presentation (motor loss from swinging a remote) led me to probe further into past episodes of his upper limb pain. This information spurred me to research alternative causes of his symptoms and discuss the case with a consultant who made an onward referral. As physiotherapists we are highly likely to receive referrals for patients like M, with little more information than ‘shoulder pain’ or ‘brachial plexus injury’ given, which is why our subjective is such an important part of the overall assessment. M’s case highlights how important collating an extensive medical history is to proper investigation and eventual diagnosis. M had a long history of upper limb events for which he had seen a variety of clinicians at various centres. Each event had been treated as an individual episode rather than one larger recurring pattern. Drawing that history together gave a more holistic picture which triggered the referral that identified a diagnosis 8 years after his first presentation to healthcare. M’s case also highlighted the importance of a good patient—therapist relationship. Motivating a patient with this type of condition is challenging; their progress is not linear and they often have to take steps backwards before they can progress again. This is exceptionally difficult for children and their parents, as it is a frustrating and repetitive cycle. They need to trust that you are giving them the correct therapy and as a therapist you need to trust that the patient is compliant with recommendations and exercise. Finally, the shoulder rehabilitation for M was, clinically speaking, the same as any other brachial plexus type injury. The main key difference was the need to intermittently take the exercises down a level in the incidence of a new episode of pain and motor loss.

Mycophenolate Mofetil (MMF), presented with high-grade fever without focus close to his 18th birthday. History or examination did not point to any focus of infection, but blood tests showed systemic inflammation. Extensive investigations, including extended infection screen and bone marrow aspiration, were negative. His blood tests were suggestive of macrophage activation syndrome with no clinical or laboratory evidence of flare of lupus. MRI scan revealed extensive para-aortic lymphadenopathy and a diagnosis of classical Hodgkin's lymphoma confirmed on lymph node biopsy. Case report -Case description: Interestingly he first presented with fever and cervical lymphadenopathy at 13 years of age to local hospital, when a biopsy had revealed lymphoma but the diagnosis was changed to SLE following review at tertiary hospital. He was started on steroids and low-dose methotrexate. He later developed facial swelling, abdominal distension and groin swelling, following which he was referred to tertiary renal centre. His examination revealed scarring malar rash, anaemia, ankle and scrotal oedema, ascites and pleural effusion. His investigations showed anaemia, thrombocytopaenia, ESR over 100mm/hour, hypoalbuminaemia, low complement levels, positive ANA and double-stranded DNA. He had nephrotic range proteinuria and renal biopsy showed Class V lupus nephritis. He was treated with intravenous and later weaning dose of oral steroids and started on MMF. He remained in remission on a stable dose of MMF, except for brief flare of proteinuria, which settled with short course of oral steroids. A few months before his eighteenth birthday, he presented to local hospital with recurrent episodes of high-grade fever with no clear focus. He was twice managed with broad spectrum antibiotics, despite negative septic screen and an extended infection screen including viral PCRs and IGRA for tuberculosis. He had no focal symptoms or symptoms to suggest flare of underlying lupus, but had loss of appetite and loss of weight. He developed splenomegaly with increasing inflammatory markers and fall in all cell lines of full blood count with deranged coagulation. He satisfied the clinical and laboratory criteria of the preliminary Diagnostic Guidelines for Macrophage Activation Syndrome in JSLE (2009), following which he was commenced on three days of IV methylprednisolone following normal bone marrow aspirate. However, fever recurred after initial response and MRI scan revealed mediastinal and para-aortic lymphadenopathy, and a diagnosis of classical Hodgkin's lymphoma was confirmed on lymph node biopsy. Case report -Discussion: This young man initially presented with fever and cervical lymphadenopathy, when a diagnosis of lymphoma was made. Yet, following review of the biopsy at the local tertiary centre and the abnormal immunological profile, a diagnosis of lupus was made. He then developed nephrotic syndrome, when a diagnosis of lupus nephritis was confirmed on biopsy. Other clinical and laboratory profile (raised ESR, hypocomplementeamia, serositis and haematological parameters) corroborated the diagnosis. He responded well to steroids and immunosuppression, when he presented with fever again. He had no evidence of flare of underlying lupus or any infection, but blood tests and splenomegaly pointed to macrophage activation syndrome (MAS). A negative bone marrow examination suggested that pancytopaenia was unlikely to be due to bone marrow infiltration, which is why he was treated with steroids. Yet, when fever recurred, MRI was arranged to investigate further, which eventually led to revelation of lymphadenopathy and biopsy, which eventually led to the right diagnosis. Another dilemma was if his MAS was to respond to steroids, whether we should change his baseline immunosuppression which had worked well so far for him and if we chose to, what would be the best agent. Case report -Key learning points: 1. Although extremely uncommon, children and young people with rheumatic diseases can develop malignancy, which may be unrelated to the primary condition or the therapeutic agents used. Unexplained symptoms in the absence of flare of underlying condition and with infections ruled out, warrant extension of investigations to rule out an underlying malignancy. 2. Diagnosis of secondary MAS in a rheumatological condition without any evidence of flare of the underlying condition should be viewed as suspicious and warrants more investigations to rule out other causes. 3. In patients who develop MAS, there are very few agents which work for both lupus and MAS, choosing an agent which treats both conditions is challenging.

OPTIMISING THE BIOMECHANICS OF YOUNG PEOPLE WITH INFLAMMATORY AND NON-INFLAMMATORY PRESENTATIONS
O09 RECURRING BRACHIAL PLEXOPATHY-THE ZEBRA AMONG THE HORSES

Brooke Mara
Cambridge University Hospitals Trust, Cambridge, United Kingdom Case report -Introduction: A case study of a teenage boy presenting with severe upper limb pain and recurring loss of upper limb function with no clear mechanism of injury. His progress in therapy was initially as expected; however, symptoms would recur despite consistency and compliance with treatment from the patient. This led to a referral for further investigations where a diagnosis of a rare inflammatory neurological condition was made. This case study is relevant for paediatric physiotherapists working in non-inflammatory, musculoskeletal and pain services as it highlights a lesser-known pathology that presents in a similar way to a more common condition.
Case report -Case description: M is a 13-year-old boy that presented with a 5-week history of stabbing pains followed by loss of motor function and sensation in his right arm after swinging a remote. A diagnosis of brachial nerve plexopathy had been suggested. M had been diagnosed with Hypermobile Ehlers-Danlos Syndrome (hEDS) but was otherwise fit and well with no significant birth, developmental or family history. He experienced similar episodes of loss of motor function throughout the entire right upper limb following an episode of acute pain aged 4 and aged 12. The episodes were presumed to be a brachial plexus injury following a shoulder subluxation; however, there was no real mechanism of injury to suggest this and symptoms self-resolved after several months in both instances. Age 8 he lost function and sensation in the left arm after a minor pulled elbow, he underwent elbow surgery at another centre to help restore the function of the left arm; however, function didn't return for approximately 1 year. On examination he had diminished reflexes throughout the right upper limb and reduced sensation along a C3-8 & T1 distribution. He had a correctable thoracic spine kyphosis with significant medial boarder scapula winging on the right. His right shoulder sat lower than the left and he had muscle atrophy at right supraspinatus, infraspinatus, and serratus anterior and deltoid with tight pectoral muscles. He was compensating using upper trapezius to achieve 90-100 degrees of shoulder flexion and abduction with 2/5 muscle power. His elbow muscle strength was reduced to 4/5 in all movements on the right. He could only actively extend his right wrist to 30 degrees and only had flickers of active radial deviation. He lacked active finger extension in digits 2-5 and had 0/5 muscle activity at the right thumb. Case report -Discussion: M underwent exercise therapy with a focus on regaining scapula control in lying and isometric rotator cuff strengthening as he had such significant wasting and was unable to control the upper limb in sitting. We also worked on improving his thoracic spine posture and on active assisted finger and wrist exercises to prevent contractures. I initially provided a sling to be worn at school and in busy environments to prevent any subluxations in view of his significant rotator cuff weakness and history of hEDS. The sling also served as a thoracic posture reminder for M. After just 2-3 weeks of input and initially making gains in strength and function, M had an episode of severe pain in the right shoulder followed by worsening motor and sensory symptoms. The recurrent nature of episodes and the weak mechanism of injury, led me to discuss M with a consultant. The consultant referred M to genetics where it was discovered he had idiopathic neuralgic amyotrophy (INA; also known as Parsonage-Turner Syndrome), a rare inflammatory neurological disorder. M had the classic signs and symptoms of INA but as he had presented to various different clinicians and centres with each episode a correlation wasn't made until this latest presentation to pain clinic Case report -Key learning points: The insubstantial mechanism of injury for his current presentation (motor loss from swinging a remote) led me to probe further into past episodes of his upper limb pain. This information spurred me to research alternative causes of his symptoms and discuss the case with a consultant who made an onward referral. As physiotherapists we are highly likely to receive referrals for patients like M, with little more information than 'shoulder pain' or 'brachial i6 https://academic.oup.com/rheumap plexus injury' given, which is why our subjective is such an important part of the overall assessment. M's case highlights how important collating an extensive medical history is to proper investigation and eventual diagnosis. M had a long history of upper limb events for which he had seen a variety of clinicians at various centres. Each event had been treated as an individual episode rather than one larger recurring pattern. Drawing that history together gave a more holistic picture which triggered the referral that identified a diagnosis 8 years after his first presentation to healthcare. M's case also highlighted the importance of a good patient-therapist relationship. Motivating a patient with this type of condition is challenging; their progress is not linear and they often have to take steps backwards before they can progress again. This is exceptionally difficult for children and their parents, as it is a frustrating and repetitive cycle. They need to trust that you are giving them the correct therapy and as a therapist you need to trust that the patient is compliant with recommendations and exercise. Finally, the shoulder rehabilitation for M was, clinically speaking, the same as any other brachial plexus type injury. The main key difference was the need to intermittently take the exercises down a level in the incidence of a new episode of pain and motor loss. Case report -Introduction: High-intensity exercise is effectiv treating Axial Spondyloarthritis (SpA). A reduction in morbidity fore should be gained from accurate diagnosis early in the history.
In 2021 the National Axial Spondylarthritis Society (NASS) statement to reduce time to diagnosis (TTD) of Axial SpA from 8 to 1, to reduce morbidity and long-term complications of inflammation. This case study aims to provide an example of how a multidiscipli team working in close clinical proximity (CCP MDT) can be reduce the TTD and optimise treatment and biomechanics of acute chronic SpA. Case report -Case description: Cases 1 and 2 compare presenting to a musculoskeletal clinic with lower back pain, and reduced range of movement. Case 1. Subacute presentation: 25-year-old male with a 3-month tory of lower back pain and stiffness. This progressed to a month o being able to walk, disturbed sleep and morning stiffness of > Clinical examination showed no extra musculoskeletal and no other joint pain. Tests showed HLA B27 positive with florid bilateral sacroiliitis on MRI. Case 2. Chronic presentation: 34-year-old male with 20-year of insidious onset lower back pain previously diagnosed as specific" back pain by a series of health care (HCPs). HLA B27 negative, MRI showed acute on chronic SIJ athy with juxtarticular sclerosis. Patients were seen by a HCP who referred to a rheumatologist. In clinical and radiological signs indicated Axial SpA which was with 120mg of IM Depo-Medorone and 8-weeks of strength-b active intervention from a physiotherapist and strength and con ing coach. BASDAI, 5-rep max single leg press scores were pre-and post-intervention (Table 1.). Case report -Discussion: Conventional therapies for SpA back pain are effective even in chronic cases; this is reflected in the improvements made across both cases. Using a similar approach of high-intensity exercise and mobility both were able to significantly improve BASDAI scores and lower limb strength.
We can hypothesise that attempts to innovate the biomechanics of the treatments is missing the fundamental issue surrounding SpA back pain highlighted by NASS; TTD takes too long for too many.
There is a feasible argument therefore that it is the structures of how we operate as HCPs that is reducing the efficacy of said treatments. This is evidenced by case 2 (Table 1), whose multiple presentations to different HCPs over 20 years led to potentially preventable prolonged morbidity as well as increased the risk of future complications due to chronic inflammation. This is a story that is sadly reflective of the disease histories of many SpA patients. Much like any primary care provider, patients at this clinic will first present to a HCP. The difference is that all HCPs are trained to recognise features of SpA back pain and encouraged to flag concerns to a doctor. HCPs work in adjacent spaces allowing for better sharing of ideas and concerns that is often reduced by physical separation. This creates the concept of a CCP MDT. HCPs are encouraged to share concerns face to face without reproach or the formality or delay of a written request. This structure has proven to be effective in diagnosis in both of these cases but has proven to be as useful in many more instances to rule out a differential or allay a concern. These cases argue that the low lying fruit of improving clinical outcomes in this cohort is addressing how we as HCPs interact and cooperate.
Case report -Key learning points: Biomechanics of the treatments of SpA are effective in the majority of cases when applied by sufficiently skilled practitioners. It is the TTD that we should address to aid the reduction in morbidity in this patient group through the following recommendations: Foster education of SpA in allied health professionals. This allows us to be aware of the boundaries of our own knowledge and at what point a different clinical perspective should be sought. Diversify clinical environments and encourage MDT working. The use of CCP MDT structures allows more nuanced access to different opinions in a safe supportive manner, allowing for sharing of ideas and concerns -allowing for supporting diverse expertise and insight.
If we are to reduce TTD to the 1-year NASS hopes for, we should first decreased range of movement. Five days prior to presentation she suffered from a sore throat, fever and lower limb rash for which she was started on flucloxacillin with some improvement. Co-morbidities included hyperlipidaemia and a hysterectomy. Regular medication included naproxen. On examination her temperature was 37.7C and she was haemodynamically stable. Both knees demonstrated active synovitis. Admission bloods were remarkable for a CRP of 399 and deranged liver function tests. Knee X-rays revealed a moderate effusion in the left knee, and a large effusion in the right. The patient was given a dose of intravenous Co-amoxiclav with a working diagnosis of septic arthritis. Aspiration of both knees was performed with 120 mls of yellow-coloured fluid aspirated from the left knee and 90 mls from the right knee.
No organisms were identified on Gram Stain and no growth at 48 hours on culture. Synovial fluid from both knees was sent for broad-based bacterial 16S rDNA PCR molecular testing.