Autopsy Case of Meningoencephalomyelitis Associated With Glial Fibrillary Acidic Protein Antibody.

BACKGROUND AND OBJECTIVES: To describe the autopsy findings and neuropathologic evaluation of autoimmune meningoencephalomyelitis associated with glial fibrillary acidic protein (GFAP) antibody. METHODS: We reviewed the clinical course, imaging, laboratory, and autopsy findings of a patient with autoimmune meningoencephalomyelitis associated with GFAP antibody who had a refractory course to multiple immunosuppressive therapies. RESULTS: The patient was a 70-year-old man who was diagnosed as GFAP antibody-associated autoimmune meningoencephalomyelitis. MRI of the head showed linear perivascular enhancement in the midbrain and the basal ganglia. Despite treatment with high-dose corticosteroids, plasma exchange, IV immunoglobulins, and cyclophosphamide, he died with devastating neurologic complications. Autopsy revealed a coexistent neuroendocrine tumor in the small intestine and diffuse inflammation in the brain parenchyma, perivascular spaces, and leptomeninges, with predominant T-cells, macrophages, and activated microglia. B-cells and plasma cells were absent. There was no astrocyte involvement with change in GFAP immunostaining. DISCUSSION: This case illustrates autoimmune meningoencephalomyelitis associated with GFAP antibody in the CSF and coexistent neuroendocrine tumor. The autopsy findings were nonspecific and did not demonstrate astrocyte involvement. Further accumulation of cases is warranted to delineate the utility and pathogenic significance of the GFAP autoantibody.

A Comprehensive Practice Guideline for Magnetic Resonance Imaging Compatibility in Implanted Neuromodulation Devices.

OBJECTIVES: The evolution of neuromodulation devices in order to enter magnetic resonance imaging (MRI) scanners has been one of understanding limitations, engineering modifications, and the development of a consensus within the community in which the FDA could safely administer labeling for the devices. In the initial decades of neuromodulation, it has been contraindicated for MRI use with implanted devices. In this review, we take a comprehensive approach to address all the major products currently on the market in order to provide physicians with the ability to determine when an MRI can be performed for each type of device implant. MATERIALS AND METHODS: We have prepared a narrative review of MRI guidelines for currently marketed implanted neuromodulation devices including spinal cord stimulators, intrathecal drug delivery systems, peripheral nerve stimulators, deep brain stimulators, vagal nerve stimulators, and sacral nerve stimulators. Data sources included relevant literature identified through searches of PubMed, MEDLINE/OVID, SCOPUS, and manual searches of the bibliographies of known primary and review articles, as well as manufacturer-provided information. RESULTS: Guidelines and recommendations for each device and their respective guidelines for use in and around MR environments are presented. CONCLUSIONS: This is the first comprehensive guideline with regards to various devices in the market and MRI compatibility from the American Society of Pain and Neuroscience.

Thoracic epidural lipomatosis associated with syrinx: A case report.

Epidural lipomatosis with associated syrinx formation is an exceedingly rare clinical entity. In this case report we present a 48-year old female who initially presented with symptoms of progressive myelopathy and later developed urinary incontinence. Her imaging studies showed upper thoracic epidural lipomatosis and associated spinal stenosis, cord compression and a mildly expansile right paracentral syrinx at C7-T1. She underwent a decompression surgery which provided her with partial symptomatic relief. On MRI, there was a mild decrease in the size of syrinx and some improvement in the degree of spinal stenosis. However, the subsequent clinical improvement supported the possibility that spinal compression caused by epidural lipomatosis could have resulted in syrinx formation. Therefore, such cases can be appropriately treated with spinal decompression surgeries or syrinx shunting procedures.

Unilateral cerebral herniation resulting in combined contralateral superior cerebellar artery territory infarction and mesencephalic injury: Two cases of a severe unrecognized variant of Kernohan notch phenomenon?

A case of unilateral cerebral herniation due to an acute middle cerebral artery territory infarct and a second case of unilateral cerebral herniation due to an acute subdural hematoma are presented in this article. In both instances, the unilateral cerebral herniation resulted in a combined contralateral superior cerebellar artery territory infarction and mesencephalic injury. Unilateral cerebral herniation resulting in a combined contralateral superior cerebellar artery territory infarct and mesencephalic injury is previously undescribed in the literature and likely reflects a severe unrecognized variant of Kernohan notch phenomenon.

Imaging of Intraspinal Tumors.

Nonosseous spinal tumors are rare and encompass a wide range of benign and malignant masses. Compartmental localization of the mass to the intramedullary, intradural extramedullary, or extradural spaces can narrow the differential of possibilities. Ependymomas and astrocytomas are the most common intramedullary masses. Nerve sheath tumors and meningiomas are the most common intradural extramedullary tumors, and nerve sheath tumors dominate the nonosseous extradural tumors. These tumors and other less common masses are described in this article through a space-based approach.

Radiographic and CT Evaluation of Recombinant Human Bone Morphogenetic Protein-2-assisted Cervical Spinal Interbody Fusion.

STUDY DESIGN: This was a retrospective study. OBJECTIVE: To radiographically demonstrate the distinct fusion pattern of recombinant human bone morphogenetic protein-2 (rhBMP-2) in the setting of anterior cervical discectomy and fusion. SUMMARY OF BACKGROUND DATA: Studies investigating spinal fusion assisted with rhBMP-2 have yielded promising results, suggesting rhBMP-2 is an efficacious alternative to iliac crest autografts. rhBMP-2-assisted spinal fusion both hastens healing and eliminates patient morbidity from iliac crest autograft. Unique to rhBMP-assisted spinal fusion is its distinct radiographic fusion pattern as fusion is achieved. Despite promising results and increased clinical use of rhBMP-2, there remains a paucity of literature documenting this radiographic process. MATERIALS AND METHODS: This study included 26 patients who underwent single-level anterior cervical discectomy and fusion using rhBMP-2. All data used for this study was collected from a prior FDA Investigational Device Exemption study. RESULTS: A polyetheretherketone cage was used as an interbody disk spacer in all 26 patients. Patients were evaluated between 2 and 6 weeks after surgery and subsequently at 3, 6, 12, and 24 months postoperative. All patients underwent plain radiography at every follow-up visit, and computed tomograhy evaluation was performed at 3, 6, 12, and 24 months as part of the study protocol. Earliest fusion was observed at 3 months in 38% of patients. Likely fusion was observed in all patients by 12 months postoperative. CONCLUSIONS: rhBMP-2 leads to both successful interbody fusion and an enhanced fusion rate with unique imaging characteristics. Additional characteristics of BMP observed in 100% of patients included prevertebral soft-tissue swelling and early endplate resorption. Other common features included polyetheretherketone cage migration, heterotopic bone formation and cage subsidence.

Case report of male child with elevated lipoprotein (a) leading to acute ischemic stroke.

Acute ischemic stroke (AIS) in children is rare with almost 40% diagnosed as cryptogenic. One possible mechanism associated with AIS is an elevated Lipoprotein (a) [Lp(a)] level. Here, we discuss the case of an 11-year old boy who presented with multiple thrombotic strokes secondary to elevated Lp(a), which was identified as the only risk factor and immediately treated with lipoprotein apheresis (LA). Eighteen months post-AIS, he is still receiving LA treatments and has made remarkable progress in his recovery without another cerebrovascular event.

Meningioma consistency prediction utilizing tumor to cerebellar peduncle intensity on T2-weighted magnetic resonance imaging sequences: TCTI ratio.

OBJECTIVE Meningioma consistency, firmness or softness as it relates to resectability, affects the difficulty of surgery and, to some degree, the extent of resection. Preoperative knowledge of tumor consistency would affect preoperative planning and instrumentation. Several methods of prediction have been proposed, but the majority lack objectivity and reproducibility or generalizability to other surgeons. In a previous pilot study of 20 patients the authors proposed a new method of prediction based on tumor/cerebellar peduncle T2-weighted imaging intensity (TCTI) ratios in comparison with objective intraoperative findings. In the present study they sought validation of this method. METHODS Magnetic resonance images from 100 consecutive patients undergoing craniotomy for meningioma resection were evaluated preoperatively. During surgery a consistency grade was prospectively applied to lesions by the operating surgeon, as determined by suction and/or cavitron ultrasonic surgical aspirator (CUSA) intensity. Consistency grades were A, soft; B, intermediate; and C, fibrous. Using T2-weighted MRI sequences, TCTI ratios were calculated. Analysis of the TCTI ratios and intraoperative tumor consistency was completed with ANOVA and receiver operating characteristic curves. RESULTS Of the 100 tumors evaluated, 50 were classified as soft, 29 as intermediate, and 21 as firm. The median TCTI ratio for firm tumors was 0.88; for intermediate tumors, 1.5; and for soft tumors, 1.84. One-way ANOVA comparing TCTI ratios for these groups was statistically significant (p < 0.0001). A single cutoff TCTI value of 1.41 for soft versus firm tumors was found to be 81.9% sensitive and 84.8% specific. CONCLUSIONS The authors propose this T2-based method of tumor consistency prediction with correlation to objective intraoperative consistency. This method is quantifiable and reproducible, which expands its usability. Additionally, it places tumor consistency on a graded continuum in a clinically meaningful way that could affect preoperative surgical planning.

Prediction of Consistency of Pituitary Adenomas by Magnetic Resonance Imaging.

Objective Pituitary adenomas are typically soft. The prevalence of fibrous adenomas is ∼ 5 to 13%. Firm tumors are difficult to remove by curettage or suction. Predicting fibrous adenomas by magnetic resonance (MR) imaging is typically difficult and unreliable. We propose a new prediction method based on MR T2-sequence intensity. Methods The MRIs of 36 consecutive patients with nonsecreting macroadenomas were evaluated preoperatively by a blinded radiologist. Using an MR T2-weighted sequence, regions of interest were sampled from the adenoma and cerebellar peduncle, and the ratio was calculated. Intraoperatively, tumors were graded prospectively for their consistency by the operating surgeon. Results There were 28 soft and 6 fibrous tumors. Unpaired t test for these ratios was found to be statistically significant (p < 0.0240; 95% confidence interval, -0.8229 to -0.06207). Mean values for soft tumors were found to be 1.918 (standard error of the mean [SEM] = 0.08212); firm tumors, 1.475 (SEM = 0.1179). Soft tumors were associated with ratios > 1.5 (sensitivity 100%; specificity 66.7%); firm tumors were associated with ratios < 1.8 (sensitivity 100%; specificity 42.9%). Conclusion Fibrous adenomas are typically diagnosed intraoperatively. However, their resection can be technically challenging. Using T2 intensity ratios on routine preoperative MRI allows identification of these challenging cases. The surgeon can then be better prepared for the surgical resection.

Compartmentalized Histoplasma capsulatum Infection of the Central Nervous System.

Background. Histoplasmosis is a common fungal infection in the southeastern, mid-Atlantic, and central states; however, its presentation can be atypical. Case Presentation. We report a case of Histoplasma capsulatum infection presenting as slowly progressive weakness in the lower extremities, followed by the development of numbness below the midthoracic area, urinary incontinence, and slurred speech. Brain MRI showed leptomeningeal enhancement, predominantly linear, involving the basal cisterns, the brainstem, and spinal cord. Cerebrospinal fluid analysis showed lymphocytic pleocytosis. Discussion. CNS histoplasmosis is usually seen in patients with disseminated histoplasmosis. Isolated CNS histoplasmosis is rarely seen, especially in immunocompetent patients. Conclusions. Histoplasmosis should be considered in the differential diagnosis of patients experiencing slowly progressive neurological deficit.

Predicting Consistency of Meningioma by Magnetic Resonance Imaging.

Objective Meningioma consistency is important because it affects the difficulty of surgery. To predict preoperative consistency, several methods have been proposed; however, they lack objectivity and reproducibility. We propose a new method for prediction based on tumor to cerebellar peduncle T2-weighted imaging intensity (TCTI) ratios. Design The magnetic resonance (MR) images of 20 consecutive patients were evaluated preoperatively. An intraoperative consistency scale was applied to these lesions prospectively by the operating surgeon based on Cavitron Ultrasonic Surgical Aspirator (Valleylab, Boulder, Colorado, United States) intensity. Tumors were classified as A, very soft; B, soft/intermediate; or C, fibrous. Using T2-weighted MR sequence, the TCTI ratio was calculated. Tumor consistency grades and TCTI ratios were then correlated. Results Of the 20 tumors evaluated prospectively, 7 were classified as very soft, 9 as soft/intermediate, and 4 as fibrous. TCTI ratios for fibrous tumors were all ≤ 1; very soft tumors were ≥ 1.8, except for one outlier of 1.66; and soft/intermediate tumors were > 1 to < 1.8. Conclusion We propose a method using quantifiable region-of-interest TCTIs as a uniform and reproducible way to predict tumor consistency. The intraoperative consistency was graded in an objective and clinically significant way and could lead to more efficient tumor resection.