Intraneural ganglion cysts originating from the hip joint: A single-center experience.

INTRODUCTION/AIMS: Intraneural ganglion cysts (INGCs) are non-neoplastic mucinous cysts within the epineurium of peripheral nerves. Characteristics of INGCs around the hip joint have not been adequately described. We aimed to describe clinical features, imaging findings, and treatment outcomes in patients with INGCs originating from the hip joint. METHODS: We retrospectively included cystic lesions around the hip joint satisfying the following inclusion criteria over 6 years: (1) multilocular elongated hyperintense cystic mass on T2-weighted imaging; and (2) distribution along the course of the peripheral nerve and its branches on magnetic resonance imaging (MRI). RESULTS: Six patients with an INGC around the hip joint were identified. Parent peripheral nerves were the sciatic nerve (four patients), the superior gluteal nerve (one patient), and the nerve to quadratus femoris (one patient). Buttock, groin, or lower extremity pain/paresthesias were the initial symptoms in all patients. INGCs within the articular branches of the hip joint were identified on MRI. Four patients underwent arthroscopic debridement and capsulotomy. All patients showed generally favorable outcome regardless of treatment. DISCUSSION: Physicians should consider the possibility of INGCs originating from the hip joint as a cause of nontraumatic hip, buttock, or lower extremity pain. This can occur in any nerve innervating the hip joint, and usually it originates in the posterior capsule of the hip joint. Arthroscopic surgery shows promising results; however, more information about the surgical technique and long-term follow-up results are needed.

Lumbosacral plexopathy caused by the perineural spread of pelvic malignancies: clinical aspects and imaging patterns.

BACKGROUND: Perineural spread (PNS) of tumors from pelvic malignancies is a rare phenomenon but constitutes an important differential diagnosis of lumbosacral plexopathy (LSP). Herein, we describe the clinical and imaging features of patients with LSP due to PNS of pelvic malignancies along with a literature review. METHODS: We retrospectively reviewed 9 cases of LSP caused by PNS of pelvic malignancy between January 2006 and August 2021, and all clinical and imaging parameters were recorded in detail. Clinical symptoms and signs of patients were described and listed in the order in which they occurred. The results of imaging test were analyzed to describe specific findings in LSP caused by PNS. RESULTS: This study enrolled nine adult patients (mean age, 50.1 years). Two cases initially presented as LSP and were later diagnosed with pelvic malignancy. Pain in the perianal or inguinal area preceded pain at the extremities in six patients. Neurogenic bladder or bowel symptoms developed in five patients. On the magnetic resonance imaging (MRI), the S1-S2 spinal nerve was most commonly involved, and S1 myotome weakness was more prominent in six patients than the other myotomes. One patient had an intradural extension. 18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET) and computed tomography (CT) showed abnormal signal intensity in six patients. No abnormality in 18F-FDG PET/CT was detected in the nervous structures in one patient. Only four patients survived until the last follow-up visit. CONCLUSIONS: Though rare, physicians should always keep in mind the possibility of LSP due to the PNS in patients with pelvic malignancy. Thorough physical examination and history taking could provide clues for diagnosis. Pelvic MRI and 18F-FDG-PET/CT should be considered for patients with LSP to rule out neoplastic LSP.

The emerging genetic diversity of hereditary spastic paraplegia in Korean patients.

Hereditary Spastic Paraplegias (HSP) are a group of rare inherited neurological disorders characterized by progressive loss of corticospinal motor-tract function. Numerous patients with HSP remain undiagnosed despite screening for known genetic causes of HSP. Therefore, identification of novel genetic variations related to HSP is needed. In this study, we identified 88 genetic variants in 54 genes from whole-exome data of 82 clinically well-defined Korean HSP families. Fifty-six percent were known HSP genes, and 44% were composed of putative candidate HSP genes involved in the HSPome and originally reported neuron-related genes, not previously diagnosed in HSP patients. Their inheritance modes were 39, de novo; 33, autosomal dominant; and 10, autosomal recessive. Notably, ALDH18A1 showed the second highest frequency. Fourteen known HSP genes were firstly reported in Koreans, with some of their variants being predictive of HSP-causing protein malfunction. SPAST and REEP1 mutants with unknown function induced neurite abnormality. Further, 54 HSP-related genes were closely linked to the HSP progression-related network. Additionally, the genetic spectrum and variation of known HSP genes differed across ethnic groups. These results expand the genetic spectrum for HSP and may contribute to the accurate diagnosis and treatment for rare HSP.

Focal vasculitic myositis as a primary manifestation of Behçet's disease: a case series of 10 Korean patients in a locomotive medicine clinic.

OBJECTIVES: Muscle involvement in Behçet's disease (BD) is rare, and several cases have been reported in the literature. Therefore, this study aimed to describe the clinical, laboratory and imaging findings in adult patients presenting with BD-associated myositis before the diagnosis of BD. METHODS: We retrospectively screened patients who visited a locomotive medicine clinic presenting with myalgia, local swelling, or tenderness of extremities without an established diagnosis of BD. We enrolled patients whose pain in the extremities was proven to be suggestive of focal vasculitic myositis and who were eventually diagnosed as having BD at the initial visit or during follow-up. We thoroughly reviewed the clinical, histological and imaging findings and treatment outcomes in patients who presented with focal vasculitic myositis as the primary manifestation of BD. RESULTS: Ten adult patients with focal vasculitic myositis as the primary manifestation of BD were enrolled. The lower and upper extremities were affected in eight and two patients, respectively. The affected lower extremities were the calf (n = 6) and thigh muscles (n = 2). The common findings of MRI included high signal intensity of the affected muscles and intermuscular fascia on fat-suppressed images, suggestive of myofascitis and oedematous changes in the subcutaneous layer. The results of skin or muscle biopsy were suggestive of vasculitis. All the patients were pain-free at the short-term follow-up (1-3 weeks) after oral steroid therapy. CONCLUSION: Focal vasculitic myositis can be a primary manifestation of BD warranting medical attention. BD-associated myositis responds well to oral steroid therapy.

Is frozen shoulder completely resolved at 2 years after the onset of disease?

BACKGROUND: Although numerous studies have suggested that frozen shoulder (FS) is a self-limiting disease with most patients recovering within 2 years, its long-term outcome is still controversial. The aims of this study were to evaluate the clinical outcomes after conservative treatment for FS and to determine the predictors of its clinical outcome. METHODS: This study included 234 shoulders of 215 patients who received conservative treatment for FS. The mean follow-up period was 41.8 months (range 27-117 months). Initial evaluation included demographics, detailed medical history, and clinical assessments of shoulder status. Questionnaires, which included the Visual Analogue Scale (VAS) pain score, American Shoulder and Elbow Surgeons (ASES) score, Subjective Shoulder Value (SSV) and satisfaction grading for the current shoulder status were assessed at the final follow-up. RESULTS: The mean VAS pain score, ASES score, and SSV significantly improved from 6.7, 37.0, and 40.1% at the time of initial evaluation to 1.5, 87.6, and 85.0% at the final follow-up evaluation (all p < 0.001). According to satisfaction grading, the shoulder status at the final follow-up was very satisfied in 101 shoulders (43.2%), satisfied in 68 (29.1%), fair in 37 (15.8%), unsatisfied in 20 (8.5%), and very unsatisfied in 8 (3.4%). Univariate analysis revealed that gender, diabetes, simultaneous bilateral involvement, overall bilateral involvement and duration of symptoms were associated with clinical outcomes at the final follow-up. Multivariate analysis revealed that duration of symptoms (p = 0.002) was an independent risk factor for unsuccessful outcome. CONCLUSIONS: At the mean follow-up period of 41.8 months, 72.3% of patients revealed subjective satisfaction for the current shoulder status. Duration of symptoms was an independent risk factor for poor prognosis.

Hourglass-like constriction neuropathy of the suprascapular nerve detected by high-resolution magnetic resonance neurography: report of three patients.

Hourglass-like constriction neuropathy is a neurological condition caused by non-traumatic, non-compressive fascicular constrictions of one or more individual peripheral nerves. Based on clinical manifestations, it is very difficult to differentiate hourglass-like constriction neuropathy from idiopathic neuralgic amyotrophy. Focal hourglass-like constriction neuropathy may be erroneously diagnosed as idiopathic neuralgic amyotrophy. Previous studies demonstrated hourglass-like constrictions of peripheral nerves in the surgical exploration of patients diagnosed with idiopathic neuralgic amyotrophy. Recently, high-resolution ultrasound or magnetic resonance neurography (MRN) have been introduced to detect focal hourglass-like constrictions of peripheral nerves in spontaneous nerve palsy. We present a series of three cases in which the suprascapular nerve was affected by hourglass-like constrictions, which were visualized by high-solution MRN, including a nerve-selective morphological MR pulse sequence with strong fat- and water-signal suppression.

Ultrasound measurements of axillary recess capsule thickness in unilateral frozen shoulder: study of correlation with MRI measurements.

OBJECTIVE: The aims of this study were to compare the ultrasound thickness of the affected axillary recess capsule (ARC) with that of the unaffected ARC in patients with frozen shoulder (FS), to analyze whether the ultrasound measurements of the ARC thickness correlate with those obtained using MRI, and to assess whether the ultrasound thickness of the ARC correlates with the patterns of range of motion limitation. MATERIALS AND METHODS: Forty-four patients with clinically diagnosed unilateral FS and MRI evaluation performed ultrasound measurement of ARC. The ultrasound measurement of the ARC thickness was performed with the patients in a supine position with their shoulder abducted by 40°. The ARC thickness was also measured by MRI on oblique coronal images by another physician blinded to the ultrasound measurements. With both ultrasound and MRI, ARC thickness was determined at the widest portion of the capsule. RESULTS: The ultrasound thickness of ARC was significantly higher in the affected shoulder (4.4 ± 1.1 mm) than in the unaffected one (2.2 ± 0.5 mm) (p < 0.001). The ultrasound thickness of the ARC in the affected shoulder correlated with that measured by MRI (8.9 ± 1.9 mm) (p < 0.001, r = 0.83). The ARC thickness, whether measured by ultrasound or MRI, was not significantly related to the limitation of movement in specific directions. CONCLUSION: Ultrasound can demonstrate the difference in ARC thickness between affected and unaffected shoulders in patients with unilateral FS. The ARC thickness measured by ultrasound correlates with that measured by MRI.

Characteristics of Korean Patients with Polymyalgia Rheumatica: a Single Locomotive Pain Clinic Cohort Study.

BACKGROUND: Polymyalgia rheumatica (PMR) is a common systemic inflammatory disease of the elderly; however, the clinical characteristics and therapeutic response of PMR in Korea have been rarely studied. METHODS: We reviewed the medical records of 54 Korean patients diagnosed with PMR between January 2009 and February 2017 in a locomotive pain clinic of one tertiary referral hospital. We analyzed epidemiologic and clinical characteristics, therapeutic responses, and prognostic factors for remission-failure at one-year after oral prednisolone treatment. RESULTS: In 54 patients with PMR, 32 (59.3%) were female. The average age at diagnosis was 65.0 ± 10.5 years. Duration of symptoms before diagnosis was 8.1 ± 8.6 months. All patients had shoulder pain (54 patients, 100.0%); 49 patients (90.7%) had hip girdle pain, while 19 patients (35.2%) had peripheral joint pain. Four patients (7.4%) were accompanied by the giant cell arteritis (GCA). There was no seasonal preference for symptom development. Only 19 patients were diagnosed with PMR at initial symptom presentation. At one-year follow-up after oral prednisolone treatment, the remission rate was 35.3% (12/34). Multivariate analysis showed that history of relapse (odds ratio, 6.81; 95% confidence interval, 1.035-44.804) was a significant predictor of remission-failure. CONCLUSION: The rate of remission (35.3%) after oral prednisolone treatment was similar to previous reports in western countries; and GCA is not a rare condition in Korean PMR patients. Misdiagnosis of PMR is common, and heightened consideration for PMR is needed in elderly patients who present inflammatory features of bilateral shoulder pain.

Early onset polymyalgia rheumatica: two rare cases under age of 50.

Polymyalgia rheumatica (PMR) is almost an exclusive disease of adults over the age of 50, and only a few cases have been reported. Two 46-year-old females visited our locomotor pain clinic with multiple joint pain with increased acute phase reactants. Rheumatologic markers, and HLA-B27 were checked. Serum protein electrophoresis and serum immunofixation electrophoresis, imaging studies including plane image, sonography, and magnetic resonance image was done. 18F-Fludeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) showed characteristic findings of PMR, without evidences of sacroiliitis. Since PMR can develop in mid 40s, a high index of suspicion is necessary in younger patients presenting the bilateral pain in shoulders, hips, and back, with elevated acute phase reactants. Furthermore, in addition to the previous case reports, FDG-PET/CT is helpful in making early differential diagnosis of PMR in patients under the age of 50. Here we present two cases of PMR onset in the mid-40s emphasizing the importance of diagnostic imaging for early differential diagnosis in PMR.

Hypophosphatemic osteomalacia induced by low-dose adefovir therapy: focus on manifestations in the skeletal system and literature review.

Osteomalacia is a metabolic bone disease that leads to softening of the bones and can be caused by hypophosphatemia. Large clinical studies of low-dose adefovir dipivoxil (adefovir) have found no evidence of renal tubular dysfunction leading to hypophosphatemia after 48 weeks of treatment. We report two cases of low-dose adefovir-induced hypophosphatemic osteomalacia that initially presented with diffuse musculoskeletal pain. The first patient was a 62-year-old man with a 2-year history of bone pain involving the dorsal mid-thorax, lower anterior chest wall, right sacroiliac joint area, and both knees. The patient had been receiving adefovir for 5 years before confirmation of hypophosphatemia and urinary phosphate wasting. Bone scintigraphy revealed multifocal lesions including multiple ribs, costochondral junctions, costovertebral junctions, sacrum, both posterior iliac bones, both proximal tibia, right calcaneus, and the left second metatarsophalangeal joint area, which were suggestive of metabolic bone disorder. Bone pain was significantly reduced within 3 months after supplementation with phosphate and calcitriol. The second patient was a 54-year-old male who presented with an 18-month history of severe bone pain of the right medial knee and low back. The patient had been taking adefovir for approximately 40 months before the development of bone pain. Laboratory data revealed hypophosphatemia and vitamin D deficiency. Bone scintigraphy showed increased uptake in bilateral ribs, sternum, both scapulae, both costovertebral junctions, both pelvic bones, medial cortex of the right proximal femur, right proximal tibia, and the left lateral tarsal bone. The symptoms improved by changing the antiviral agent from adefovir to entecavir. Because osteomalacia often presents with diffuse bone pain, non-specific radiologic findings and non-characteristic routine serum biochemical changes, the disease can be confused with various musculoskeletal diseases and a high index of suspicion is necessary for an early diagnosis in patients receiving adefovir therapy.

Incidence of deep vein thrombosis after spinal cord injury in Korean patients at acute rehabilitation unit.

Deep vein thrombosis (DVT) and subsequent pulmonary embolism (PE) remain significant causes of morbidity, mortality in patients with spinal cord injury (SCI). Since incidence of DVT after SCI in Korean population has not been much studied, we retrospectively analyzed the medical records of 185 SCI patients admitted for acute rehabilitation unit to investigate the incidence of DVT. Color Doppler ultrasonography was performed to screen for the occurrence of DVT at the time of initial presentation to acute rehabilitation unit. Primary study outcome was the incidence of DVT. Possible risk factors for DVT including the epidemiologic characteristics, completeness of motor paralysis, cause of injury, spasticity, surgery, and active cancer were analyzed. The incidence of DVT after SCI was 27.6%. In multiple logistic regression analysis, absence of spasticity was a significant independent risk factor (P<0.05) for occurrence of DVT. Symptomatic pulmonary embolism was evident in 7 patients without an episode of sudden death. Therefore, it is concluded that the incidence of DVT after SCI in Korean patients is comparable with that in Western populations. This result suggests that pharmacologic thromboprophylaxis should be considered in Korean patients with SCI.

Adult-onset hypophosphatemic osteomalacia as a cause of widespread musculoskeletal pain: A retrospective case series of single center experience.

BACKGROUND: Osteomalacia (OM) is frequently confused with various musculoskeletal or other rheumatic diseases, especially in patients with adult-onset widespread musculoskeletal pain because of its low prevalence and non-specific manifestations. AIM: To facilitate the early diagnosis and etiology-specific treatment of adult-onset hypophosphatemic OM. METHODS: A retrospective review of medical records was performed to screen adult patients who visited a physiatry locomotive medicine clinic (spine and musculoskeletal pain clinic) primarily presenting with widespread musculoskeletal pain at a single tertiary hospital between January 2011 and December 2019. We enrolled patients with hypophosphatemia, high serum bone-specific alkaline phosphatase levels, and at least one imaging finding suggestive of OM. RESULTS: Eight patients with adult-onset hypophosphatemic OM were included. The back was the most common site of pain. Proximal dominant symmetric muscle weakness was observed in more than half of the patients. Bone scintigraphy was the most useful imaging modality for diagnosing OM because radiotracer uptake in OM showed characteristic patterns. Six patients were diagnosed with adefovir (ADV)-induced Fanconi syndrome, and the other two patients were diagnosed with tumor-induced OM and light-chain nephropathy, respectively. After phosphorus and vitamin D supplementation and treatment for the underlying etiologies, improvements in pain, muscle strength, and gait were observed in all patients. CONCLUSION: Mechanical pain characteristics, hypophosphatemia, and distinctive bone scintigraphy patterns are the initial diagnostic indicators of adult-onset hypophosphatemic OM. ADV-induced Fanconi syndrome is the most common etiology of hypophosphatemic OM in hepatitis B virus-endemic countries.

Therapeutic Efficacy and Prediction of 18F-FDG PET/CT-Assisted Botulinum Toxin Therapy in Patients With Idiopathic Cervical Dystonia.

PURPOSE: This study aimed to investigate the therapeutic efficacy of 18F-FDG PET/CT-assisted botulinum toxin (BTX) injection therapy and predictive PET findings in relation to a good response in patients with idiopathic cervical dystonia (ICD). MATERIALS AND METHODS: A total of 78 patients was enrolled from November 2007 to July 2018. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) score was determined at baseline and 4 weeks after BTX injection guided by electromyography and PET/CT. The number of hypermetabolic muscles, the highest SUVmax among hypermetabolic muscles, and the total SUVmax of hypermetabolic muscles were evaluated as pretreatment PET parameters. A good response was defined as a reduction rate ≥30% and a point decrease ≥15 of the TWSTRS total score. RESULTS: Half of the subjects showed a good response. Good responders had significantly higher baseline TWSTRS scores than poor responders (total score, P < 0.001; severity, P < 0.001; disability, P < 0.001; pain, P = 0.026). Good responders also had significantly higher numbers of hypermetabolic muscles and BTX-injected hypermetabolic muscles (P < 0.001, both). In multivariable analysis, the baseline TWSTRS disability subscale score and the number of BTX-injected hypermetabolic muscles were significant predictors for good response (P = 0.001 and P = 0.028). The aforementioned 3 PET parameters were positively correlated with the baseline TWSTRS scores. In addition, PET/CT well detected dystonic deep cervical muscles. CONCLUSIONS: FDG PET/CT-assisted BTX injection therapy showed good therapeutic efficacy in ICD patients. The numbers of hypermetabolic cervical muscles and BTX-injected hypermetabolic muscles may be helpful in predicting a good response.

Hereditary Spastic Paraplegia in Koreans: Clinical Characteristics and Factors Influencing the Disease Severity.

BACKGROUND AND PURPOSE: Hereditary spastic paraplegia (HSP) progresses over time and is associated with locomotive dysfunction. Understanding the factors affecting disease severity and locomotive function is important in HSP. This study investigated the factors influencing disease severity and ambulation status of HSP. METHODS: We consecutively enrolled 109 Korean patients (64 males, and 45 females)from 84 families with a clinical diagnosis of HSP. HSP was primarily diagnosed based on clinical criteria including clinical findings, family history, and supported by genetic studies. Epidemiological and clinical features of the patients were analyzed, and the Spastic Paraplegia Rating Scale (SPRS) score and ambulatory status were used to evaluate disease severity. RESULTS: Ninety-two (84.4%) patients had pure HSP, and 55 (50.4%) had a dominant family history. Thirty-one (28.4%) patients required a mobility aid for locomotion. A Kaplan-Meier analysis showed that HSP patients lost their independent gait ability after a median disease duration of 34 years. Those with an age at onset of ≤18 years had a longer median independent walking time. Pure HSP is characterized by predominant bilateral lower extremity weakness and spasticity, whereas complicated HSP presents more complex neurological findings such as ocular and bulbar symptoms, ataxia, and cognitive impairment. Complicated HSP was significantly correlated with the SPRS mobility score (ß=3.70, 95% confidence interval=0.45-6.94). The age at onset and disease duration were significantly correlated with disease severity, and they were significant predictors of the use of a mobility aid (p<0.05). CONCLUSIONS: These findings suggest that a later age at onset and longer disease duration are significant factors affecting the disease severity and ambulatory function in patients with HSP. These findings can help clinicians to identify subjects at risk of locomotive impairment.

Deep vein thrombosis of the common iliac vein caused by neurogenic heterotopic ossification in the anterior lower lumbar spine of a patient with complete paraplegia due to radiation-induced myelopathy.

Context: Deep vein thrombosis (DVT), a frequent complication of spinal cord injury, is occasionally caused by neurogenic heterotopic ossification (NHO). In most cases of NHO, the hip joint is affected. Herein, we present a case of paraplegia following radiation-induced myelopathy that presented with left leg swelling due to DVT in the common iliac vein (CIV) caused by venous compression by NHO on the anterior lower lumbar spine.Findings: A 28-year-old man with complete paraplegia due to radiation-induced myelopathy presented with left lower extremity swelling 6 years after the onset of paraplegia. DVT in the left CIV was observed on computed tomography venography. The left CIV was significantly compressed between the NHO at the anterior longitudinal ligament of the lumbar spine and the right common iliac artery, suggestive of May-Thurner syndrome. Slightly distal to that compressed area, the left CIV was significantly compressed by the large NHO at the anterior longitudinal ligament of the lumbar spine.Conclusions: We believe that such compression of the left CIV would have contributed to the development of DVT. This case shows that DVT might be caused by NHO at the anterior aspect of the lumbar vertebral body, and this may help clinicians identify the main cause of DVT in the leg.

TREX1 Deficiency Induces ER Stress-Mediated Neuronal Cell Death by Disrupting Ca2+ Homeostasis.

TREX1 is an exonuclease that degrades extranuclear DNA species in mammalian cells. Herein, we show a novel mechanism by which TREX1 interacts with the BiP/GRP78 and TREX1 deficiency triggers ER stress through the accumulation of single-stranded DNA and activates unfolded protein response (UPR) signaling via the disruption of the TREX1-BiP/GRP78 interaction. In TREX1 knockdown cells, the activation of ER stress signaling disrupted ER Ca2+ homeostasis via the ERO1α-IP3R1-CaMKII pathway, leading to neuronal cell death. Moreover, TREX1 knockdown dysregulated the Golgi-microtubule network through Golgi fragmentation and decreased Ac-α-tubulin levels, contributing to neuronal injury. These alterations were also observed in neuronal cells harboring a TREX1 mutation (V91M) that has been identified in hereditary spastic paraplegia (HSP) patients in Korea. Notably, this mutation leads to defects in the TREX1-BiP/GRP78 interaction and mislocalization of TREX1 from the ER and possible disruption of the Golgi-microtubule network. In summary, the current study reveals TREX1 as a novel regulator of the BiP/GRP78 interaction and shows that TREX1 deficiency promotes ER stress-mediated neuronal cell death, which indicates that TREX1 may hold promise as a therapeutic target for neurodegenerative diseases such as HSP.

Case Report: Neurogenic Thoracic Outlet Syndrome Without Electrophysiologic Abnormality.

Neurogenic thoracic outlet syndrome (N-TOS) is a chronic compressive brachial plexopathy that involves the C8, T1 roots, and/or lower trunk. Medial antebrachial cutaneous (MABC) nerve conduction study (NCS) abnormality is reportedly one of the most sensitive findings among the features of N-TOS. The aim of the present study was to report clinical features, imaging findings, treatment, and prognoses of two N-TOS patients with no abnormalities in electrophysiological studies. Both patients presented with paresthesia of unilateral arm, and examination revealed no neurologic deficits. Electrophysiologic studies including MABC NCS were normal. Computed tomography (CT) angiography and brachial plexus magnetic resonance imaging (MRI) of the patients showed compression and displacement of the neurovascular bundle in the thoracic outlet by causative structures. Due to their sensory symptoms and CT angiography and brachial plexus MRI findings, after excluding other diseases, we diagnosed them with N-TOS. With the development of imaging techniques, more patients presenting with clinical features of lower trunk brachial plexopathy and anomalous structures compressing the neurovascular bundle on imaging studies can be diagnosed with N-TOS, even if electrophysiologic studies including MABC NCS do not show abnormalities.

Neurolymphomatosis: a single-center experience of neuromuscular manifestations, treatments, and outcomes.

OBJECTIVES: Neurolymphomatosis (NL) is a disease characterized by the infiltration of malignant lymphocytes into the peripheral nervous system. We report clinical features, radiographic findings, modes of treatment, and outcomes of patients with NL. METHODS: We retrospectively investigated patients with NL. We extracted data, including clinical features, magnetic resolution imaging (MRI), 18F-fludeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) scans, cerebrospinal fluid cytology findings, the results of electrodiagnostic studies, as well as patient treatments and outcomes. RESULTS: Ten NL patients were identified. All patients reported pain/paresthesia and weakness in the affected area. The MRI scans were abnormal in eight out of nine patients with an enhancement of the spinal nerve root, plexus, peripheral nerve trunk, and cranial nerve. The FDG PET/CT scans were positive in all patients. Radiculopathy or radiculoplexopathy was the most common electrodiagnostic finding. Neurological improvement was observed in only three patients. The condition of the nine patients who underwent multimodality treatments for cancer eventually deteriorated and the patients died. CONCLUSIONS: NL should be considered in the differential diagnosis of any type of neuropathy in patients with lymphoma. Because it could be confused with other neuropathies in lymphoma and various musculoskeletal diseases, a high index of suspicion and familiarity with clinical manifestation of NL are key. FDG PET/CT was the most sensitive diagnostic imaging modality to detect relevant neural invasion. The root within the spinal neural foramen was the most frequently affected neural structure. Early diagnosis of this rare neurologic manifestation of lymphoma may improve treatment outcomes.

Comparison of Shoulder Ultrasonographic Assessments between Polymyalgia Rheumatica and Frozen Shoulder in Patients with Bilateral Shoulder Pain: A Comparative Retrospective Study.

This study aimed to assess and compare the ultrasonographic (US) pathologic findings in patients with polymyalgia rheumatica (PMR) and bilateral frozen shoulder (FS). We included 19 patients with clinically diagnosed PMR and 19 patients with stage II bilateral FS. The US evaluation included the assessment of subacromial-subdeltoid (SASD) bursitis, long head of biceps (LHB) tenosynovitis, and posterior and inferior glenohumeral (GH) synovitis. Unilateral SASD bursitis was noted significantly more frequently in PMR patients than in bilateral FS patients (p = 0.001). There were no significant differences in the incidence of unilateral LHB tenosynovitis and posterior GH synovitis between PMR and bilateral FS patients (p = 0.108 and p = 0.304, respectively). Unilateral inferior GH synovitis was more common among bilateral FS patients than among PMR patients (p < 0.001). Bilateral SASD bursitis and LHB tenosynovitis were noted significantly more frequently in PMR patients than in bilateral FS patients (p < 0.001 and 0.049, respectively). Significant differences were not observed in the incidence of bilateral posterior GH synovitis between PMR and bilateral FS patients (p = 0.426). Bilateral inferior GH synovitis was more common among bilateral FS patients than among PMR patients (p = 0.044). The US evidence for bilateral inferior GH synovitis without bilateral SASD showed high specificity (94.7%) with sensitivity (78.9%) for the diagnosis of bilateral FS. SASD bursitis, representing periarticular synovial inflammation, was more common among the patients with PMR than among the patients with bilateral FS. Inferior GH synovitis without SASD bursitis suggests FS rather than PMR in patients with bilateral shoulder pain.

Brachial Plexus Injury Associated with Median Sternotomy during Cardiac Surgery: Three Cases of C8 Radiculopathy Due to the Fracture of the First Rib.

In cardiac surgery, median sternotomy is often necessary during certain surgical processes and it can cause the rare complication of brachial plexus injury. Retraction of the rib cage during median sternotomy may produce a fracture of the first thoracic rib at the costovertebral junction which might penetrate or irritate the lower root of the brachial plexus. Because the C8 ventral root is located immediately superior to the first thoracic rib, the extraforaminal C8 root is thought to be the key location of brachial plexus injury by the first rib fracture. This report describes three cases of brachial plexus injury after median sternotomy in a single center. In our cases, fracture of the first rib and consequent brachial plexus injury is confirmed with imaging and electrophysiologic studies. The fracture of the first rib is not detected with standard plain images and it is confirmed only with CT or MRI studies. Advanced imaging tools are recommended to assess the first rib fracture when brachial plexus injury is suspected after median sternotomy.