Spinner, Robert J.

AbstractNeurolymphomatosis (NL) is a rare manifestation of lymphoma, with limited evidence for optimal management. The largest patient series, 50 cases of lymphoma and leukemia, was published in 2010 with limited rituximab exposure. This study aims to evaluate the clinical presentation, diagnostic testing, and outcomes of NL in the rituximab era. Forty biopsy-proven cases of NL, in association with non-Hodgkin lymphoma (NHL), at the Mayo Clinic were retrospectively evaluated. B-cell NHL was associated with 97% of NL cases, of which diffuse large B-cell lymphoma (DLBCL) was the most common (68%). Primary NL, defined as neural involvement present at the time of diagnosis of lymphoma, was noted in 52% cases. Seventy percent of patients presented with sensorimotor weakness and neuropathic pain. Magnetic resonance imaging (MRI) was positive in 100% patients. Overall survival (OS) was significantly better for primary NL and NL associated with indolent lymphomas. Relapses were seen in 60% (24/40) of patients; 75% involved the peripheral or central nervous system at relapse. The use of rituximab in the frontline setting significantly impacted progression-free survival (PFS). Transplant consolidation was noted to be associated with improved OS. This study adds to the available literature on NL in the rituximab era. The overall outcomes have improved in recent years. In our experience, MRI and positron emission tomography/computed tomography may be required for accurate assessment of the extent of disease involvement and identification of an optimal biopsy site. The use of rituximab was associated with improvement in PFS, and autologous stem cell transplant was associated with OS.

OBJECTIVE The aim of this study was to examine the role of intraoperative neuromonitoring (IONM) during resection of benign peripheral nerve sheath tumors in achieving gross-total resection (GTR) and in reducing postoperative neurological complications. METHODS Data from consecutive adult patients who underwent resection of a benign peripheral nerve sheath tumor at 7 participating institutions were combined. Propensity score matching was used to balance covariates. The primary outcomes of interest were the association between IONM and GTR and the association of IONM and the development of a permanent postoperative neurological complication. The secondary outcomes of interest were the association between IONM and GTR and the association between IONM and the development of a permanent postoperative neurological complication in the subgroup of patients with tumors involving a motor or mixed nerve. Univariate and multivariate logistic regression were then performed on the propensity score–matched samples to assess the ability of the independent variables to predict the outcomes of interest. RESULTS A total of 337 patients who underwent resection of benign nerve sheath tumors were included. In multivariate analysis, the use of IONM (OR 0.460, 95% CI 0.199–0.978; p = 0.047) was a significant negative predictor of GTR, whereas none of the variables, including IONM, were associated with the occurrence of a permanent postoperative neurological complication. Within the subgroup of motor/mixed nerve tumors, in the multivariate analysis, IONM (OR 0.263, 95% CI 0.096–0.723; p = 0.010) was a significant negative predictor of a GTR, whereas IONM (OR 3.800, 95% CI 1.925–7.502; p < 0.001) was a significant positive predictor of a permanent postoperative motor deficit. CONCLUSIONS Overall, 12% of the cohort had a permanent neurological complication, with new or worsened paresthesias most common, followed by pain and then weakness. The authors found that formal IONM was associated with a reduced likelihood of GTR and had no association with neurological complications. The authors believe that these data argue against IONM being considered standard of care but do not believe that these data should be used to universally argue against IONM during resection of benign nerve sheath tumors.

Background: The preponderance of literature on the repair of proximal hamstring tendon tears focuses on the acute phase (<4 weeks). As such, there is a paucity of data reporting on the outcomes of chronic proximal hamstring tears. Purpose: To report minimum 2-year postoperative patient-reported outcome (PRO) scores, visual analog scale (VAS) for pain, and patient satisfaction from patients who underwent open or endoscopic repair of partial- and full-thickness chronic proximal hamstring tendon tears. Study Design: Case series study; Level of evidence, 4. Methods: Between April 2002 and May 2017, prospectively collected data from 3 tertiary care institutions were retrospectively reviewed for patients who underwent open and endoscopic repair of partial- and full-thickness chronic proximal hamstring tendon tears. Patients were included only if they had a chronic proximal hamstring tear (defined as ≥4 weeks from symptom onset to surgery). Patients were excluded if they had a tear treated <4 weeks after injury, underwent hamstring reconstruction, or claimed workers’ compensation. Patients who reported minimum 2-year follow-up for VAS, patient satisfaction, and the following PROs had their outcomes analyzed: the modified Hip Harris Score, Non-arthritic Hip Score, iHOT-12 (International Hip Outcome Tool), and Hip Outcome Score–Sports Specific Subscale. Results: Fifty patients (34 females and 16 males) were included in this study. There were 19 endoscopic repairs and 31 open repairs. Within the cohort, 52.0% had a full-thickness tendon tear on magnetic resonance imaging, and 48.0% had a partial tear. Average follow-up time was 58.07 ± 37.27 months (mean ± SD; range, 24-220 months). The mean age and body mass index of the group were 46.13 ± 13 years and 25.43 ± 5.14. The average time from injury to surgery was 66.73 weeks (range, 5.14-215.14 weeks). Average postoperative PROs were as follows: modified Hip Harris Score, 91.94 ± 9.96; Non-arthritic Hip Score, 91.33 ± 9.99; iHOT-12, 87.17 ± 17.54; Hip Outcome Score–Sports Specific Subscale, 87.15 ± 18.10; and VAS, 1.16 ± 1.92. Patient satisfaction was 8.22 ± 1.20. Conclusion: Patients who underwent open and endoscopic repairs for chronic partial- and full-thickness proximal hamstring tendon tears reported high PROs and satisfaction at a minimum 2-year follow-up with low rates of complications.

OBJECTIVE Meralgia paresthetica is caused by entrapment of the lateral femoral cutaneous nerve (LFCN) and often presents with pain. Multiple treatment options targeting the LFCN can be pursued to treat the pain should conservative measures fail, with the most common options being injection, neurolysis, and neurectomy. However, their efficacy in causing pain relief and their clinical outcomes have yet to be directly compared. The aim of this study was to interrogate the contemporary literature and quantitatively define how these options compare. METHODS The electronic databases Ovid Embase, PubMed, SCOPUS, and the Cochrane Library were interrogated from inception to May 2020 following the PRISMA guidelines. Candidate articles were screened against prespecified criteria. Outcome data were abstracted and pooled by random-effects meta-analysis of proportions. RESULTS There were 25 articles that satisfied all criteria, reporting outcomes for a total of 670 meralgia paresthetica patients, with 78 (12%) treated by injection, 496 (74%) by neurolysis, and 96 (14%) by neurectomy. The incidence of complete pain relief was 85% (95% CI 71%–96%) after neurectomy, 63% (95% CI 56%–71%) after neurolysis, and 22% (95% CI 13%–33%) after injection, which were all statistically different (p < 0.01). The incidence of revision procedures was 12% (95% CI 4%–22%) after neurolysis and 0% (95% CI 0%–2%) after neurectomy, which were significantly lower than 81% (95% CI 64%–94%) after injection (p < 0.01). The incidences of treatment complications were statistically comparable across all three treatments, ranging from 0% to 5% (p = 0.34). CONCLUSIONS There are multiple treatment options to target pain in meralgia paresthetica. The incidence of complete pain relief appears to be the greatest among the 3 interventions after neurectomy, accompanied by the lowest incidence of revision procedures. These findings should help inform patient preference and expectations. Greater exploration of the anatomical rationale for incomplete pain relief after surgical intervention will assist in optimizing further surgical treatment for meralgia paresthetica.

Abstract BACKGROUND Neuromuscular choristoma (NMC) is a peripheral nerve malformation frequently associated with a fibromatosis (NMC-DTF) that mimics sporadic desmoid-type fibromatosis (DTF). Sporadic DTF is often managed conservatively but its clinical behavior varies. CTNNB1 mutational subtypes in sporadic DTF have prognostic value. We have previously identified CTNNB1 mutations in NMC, and 3 paired NMC-DTF but the clinical behavior of NMC-DTF is poorly understood. OBJECTIVE To evaluate patients with NMC-DTF to determine (1) CTNNB1 mutational subtypes in NMC-DTF, and (2) associated clinical behavior and response to treatment. METHODS Retrospective review of clinical, imaging, and pathologic features of patients with NMC and NMC-DTF, and molecular testing for CTNNB1 mutations. RESULTS Among 7 patients with NMC of the sciatic nerve (median age: 18 yr), NMC-DTF (mean size 10.7 cm) developed shortly following NMC biopsy (N = 5) or spontaneously (N = 2): 6 NMC-DTF had CTNNB1 p.S45X mutations and 1 NMC-DTF had a p.T41A mutation. All patients with CTNNB1 -p.S45-mutated NMC-DTF developed local progression after wide local excision or active surveillance, including one distal metachronous NMC-DTF. No patient had spontaneous disease stabilization. Following adjuvant radiation or systemic therapy, disease stabilization was achieved in 4 (of 6) patients. One patient progressed on sorafenib treatment. CONCLUSION NMC-DTF frequently contain CTNNB1 p.S45 mutations, behave aggressively, and require adjuvant therapies for disease stabilization. We now use imaging alone to diagnose NMC, and routinely surveille the NMC-affected nerve segment to identify early NMC-DTF. In contrast to sporadic DTF, earlier adoption of systemic therapeutic strategies may be required for optimal disease management of NMC-DTF.

AbstractBackgroundMultiple mononeuropathy is a rare presentation of primary (AL) amyloidosis and nerve biopsy is usually needed for diagnosis. Conventional imaging is useful to identify proximal nerve involvement but may be inadequate. We report a patient with multiple mononeuropathy whose presentation was suggestive of AL amyloid neuropathy and in whom repeated tissue biopsies were negative for amyloid (including two sensory nerves and one muscle).MethodsThe patient underwent magnetic resonance imaging (MRI) and whole body 18F‐florbetapir positron emission tomography (PET)/MRI.ResultsWhole body 18F‐florbetapir PET/MRI revealed abnormal low‐level florbetapir uptake in the right proximal tibial and peroneal nerves, which provided a target for a sciatic bifurcation fascicular nerve biopsy that was diagnostic of AL amyloidosis.Conclusions 18F‐florbetapir PET/MRI imaging is a promising diagnostic tool for patients with suspected peripheral nerve amyloidosis (including multiple mononeuropathy) in whom conventional imaging and nerve and muscle biopsies miss the pathology.

Abstract Objectives: The objective of the study is to distinguish the mechanisms of disease for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN), which we believe to be fundamentally different. However, distinguishing the mechanisms is more difficult when the presentation of CIDP is motor-predominant, focal, or asymmetric. Methods: We describe 3 focal, motor-predominant, representative cases that could be interpreted on clinical and/or electrophysiological grounds as either MMN or focal CIDP, and present pathological findings. Results: We highlight pathological differences in these cases, and provide an argument that CIDP and MMN are distinct entities with different pathophysiological mechanisms—chronic demyelination for CIDP, and an immune-mediated attack on paranodal motor axons for MMN. Conclusions: Based on clinical evaluation, electrophysiology, and nerve biopsy pathology, we can divide the conditions into inflammatory demyelinating neuropathy (focal CIDP) versus chronic axonal neuropathy (MMN). The divergent pathological findings provide further evidence that CIDP and MMN are fundamentally different disorders.