Amrami, Kimberly K.

Abstract Amyotrophic lateral sclerosis is a neurodegenerative disorder, which is characterized by progressive upper and lower motor neuron signs and symptoms, resulting in progressive muscle weakness. There are very rare reports of magnetic resonance imaging (MRI) abnormalities within the nerve roots or plexus reported in amyotrophic lateral sclerosis. Here, we report a patient who presented with progressive left arm weakness and was found to have bilateral MRI plexus abnormalities. This report illustrates that MRI plexus abnormalities can be seen in amyotrophic lateral sclerosis, possibly before symptoms, and may have clinical value in the diagnostic process.

Abstract Neuromuscular choristoma (NMC) is a rare congenital nerve lesion that is classified by the presence of heterotopic muscle fibers intercalated within nerve fascicles. 1 The presence of NMC typically causes progressive neuropathy and bony/soft tissue undergrowth, often leading to a shortened atrophic limb. 2,3 The typical appearance of NMC on magnetic resonance imaging is an enlarged fusiform nerve that exhibits minimal gadolinium enhancement. 4 While there is increasing documentation of pathognomonic clinical and radiographic features for various diffuse fusiform peripheral nerve lesions, nerve biopsy is still occasionally required for some patients given the rarity of these diseases and progressive neuropathic features. The intraoperative appearance of NMC and its unique electrical stimulation properties are quite interesting. Intrafascicular replacement of nerve by skeletal muscle gives the nerve a red beefy color and causes the fascicles to contract with electrical stimulation. 5 In this video, we demonstrate the intraoperative appearance and contractile properties of NMC in a 60-yr-old woman with progressive sciatic neuropathy. Histologic and immunohistochemical studies identified a gradient of intralesional mature skeletal muscle within the NMC, with substantially fewer desmin-positive skeletal muscle fibers in the distal aspect. A gradient of contractility with intraoperative stimulation seemed to correlate with this pathology. These findings suggest that the lesional extent of NMC may not be clinically or grossly evident, reflecting a varying distribution of skeletal muscle within the affected nerve. The Mayo Clinic Institutional Review Board Office for Human Research Protection does not require approval for single participant case studies. The subject involved in this study was not identifiable and the general surgical consent form included permission for intraoperative photos and video.

AbstractObjectivesBoth projection and dual‐energy (DE)‐based methods have been used for metal artifact reduction (MAR) in CT. The two methods can also be combined. The purpose of this work was to evaluate these three MAR methods using phantom experiments for five types of metal implants.Materials and MethodsFive phantoms representing spine, dental, hip, shoulder, and knee were constructed with metal implants. These phantoms were scanned using both single‐energy (SE) and DE protocols with matched radiation output. The SE data were processed using a projection‐based MAR (iMAR, Siemens) algorithm, while the DE data were processed to generate virtual monochromatic images at high keV (Mono+, Siemens). In addition, the DE images after iMAR were used to generate Mono+ images (DE iMAR Mono+). Artifacts were quantitatively evaluated using CT numbers at different regions of interest. Iodine contrast‐to‐noise ratio (CNR) was evaluated in the spine phantom. Three musculoskeletal radiologists and two neuro‐radiologists independently ranked the artifact reduction.ResultsThe DE Mono+ at high keV resulted in reduced artifacts but also lower iodine CNR. The iMAR method alone caused missing tissue artifacts in dental phantom. DE iMAR Mono+ caused wrong CT numbers in close proximity to the metal prostheses in knee and hip phantoms. All musculoskeletal radiologists ranked SE iMAR > DE iMAR Mono+ > DE Mono+ for knee and hip, while DE iMAR Mono+ > SE iMAR > DE Mono+ for shoulder. Both neuro‐radiologists ranked DE iMAR Mono+ > DE Mono+ > SE iMAR for spine and DE Mono+ > DE iMAR Mono+ > SE iMAR for dental.ConclusionsThe SE iMAR was the best choice for the hip and knee prostheses, while DE Mono+ at high keV was best for dental implants and DE iMAR Mono+ was best for spine and shoulder prostheses. Artifacts were also introduced by MAR algorithms.

We report a case of low‐grade fibromyxoid sarcoma arising within the median nerve. A 31‐year‐old woman presented with symptoms of carpal tunnel syndrome and an enlarging mass in her right palm over 1 year. MRI demonstrated a mass associated with the right median nerve with solid and cystic components. At surgery, the mass was located within the epineurium, could be bluntly dissected from the nerve fascicles, and was suspected to be a schwannoma. A 3.4 cm, tan‐pink, glistening, smooth, homogenous mass was submitted to pathology. Microscopically, the tumor was a solid and cystic circumscribed nodule with a dense fibrous pseudocapsule. The tumor cells were uniformly bland and spindle‐shaped, with small, hyperchromatic oval nuclei and were embedded in an alternating fibrous and myxoid stroma with a prominent curvilinear vasculature and perivascular sclerosis. The differential diagnosis for this lesion included myxoid neurofibroma, schwannoma, soft tissue perineurioma, low‐grade malignant peripheral nerve sheath tumor and low‐grade fibromyxoid sarcoma. The tumor cells expressed MUC4, GLUT‐1, and vimentin and were negative for S‐100 protein, epithelial membrane antigen, smooth muscle actin, desmin, claudin‐1, neurofilament and SOX10. Fluorescence in situ hybridization, with a break‐apart probe strategy, demonstrated FUS rearrangement, consistent in this morphological context with the low‐grade fibromyxoid sarcoma‐associated FUS‐CREB3L2 or FUS‐CREB3L1 fusions. Low‐grade fibromyxoid sarcoma is exceptionally rare in the peripheral nerve, with only a single previously reported case. Nonetheless, as our case illustrates, this entity must be included in the differential diagnosis of unusual intraneural mesenchymal tumors. As in all other locations, intraneural low‐grade fibromyxoid sarcomas should be excised with negative margins. Patients with this disease require long‐term clinical follow‐up, given this tumor's propensity for very late distant metastases to the lungs and other sites.

OBJECTIVEThis study aimed to define a set of clinicoradiological parameters with a high specificity for the diagnosis of intraneural perineurioma, obviating the need for operative tissue diagnosis.METHODSThe authors retrospectively reviewed MR images obtained in a large cohort of patients who underwent targeted fascicular biopsy and included only those patients for whom the biopsy yielded a diagnosis. Clinical and radiological findings were then tested for their ability to predict a tissue diagnosis of intraneural perineurioma. The authors propose a new set of diagnostic criteria, referred to as the Perineurioma Diagnostic Criteria. The sensitivity, specificity, positive predictive value, and negative predictive value of several clinicoradiological methods of diagnosis were compared.RESULTSA total of 195 patients who underwent targeted fascicular biopsy were included in the cohort, of whom 51 had a tissue diagnosis of intraneural perineurioma. When the clinicoradiological methods used in this study were compared, the highest sensitivity (0.86), negative predictive value (0.95), and F1 score (0.88) were observed for the decision trees generated in C5.0 and rPart, whereas the highest specificity (1.0) and positive predictive value (1.0) were observed for the Perineurioma Diagnostic Criteria.CONCLUSIONSThis study identified clinical and radiological features that are associated with a diagnosis of perineurioma. The Perineurioma Diagnostic Criteria were determined to be the following: 1) no cancer history, 2) unifocal disease, 3) moderate to severe hyperintensity on T2-weighted MR images, 4) moderate to severe contrast enhancement, 5) homogeneous contrast enhancement, 6) fusiform shape, 7) enlargement of the involved nerves, and 8) age ≤ 40 years. Use of the Perineurioma Diagnostic Criteria obviates the need for tissue diagnosis when all of the criteria are satisfied.

Background An anteroinferior osseous defect of the glenoid rim is sometimes encountered in patients with recurrent anterior dislocations of the shoulder. A defect of more than 21% of the glenoid length is reported to cause instability after Bankart repair. Hypothesis We can estimate the critical size of glenoid defects by using radiography or computed tomography. Study Design A controlled laboratory study. Methods Osseous defects of 0%, 9%, 21%, 34%, and 46% of the glenoid length were created stepwise in 12 cadaveric scapulae, and plain radiographs simulating the axillary and West Point views and computed tomographic images were obtained. The maximum width of the remnant glenoid was measured under each condition and expressed as a percentage of the width of the intact glenoid. Results A 21% defect appeared to be 18.6% of the intact glenoid on the West Point view. With computed tomography, a 21% defect resulted in loss of 50% of the width on a single slice across the lower one-fourth of the glenoid. Conclusions We can estimate the size of a glenoid defect by using the West Point radiographic view or computed tomogram. Clinical Relevance These images gave decisive information as to whether an osseous glenoid defect required bone grafting to achieve stability after Bankart repair.