Faculty Bibliography

Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by heterotopic ossification of soft connective and muscle tissues, often as the result of minor trauma. The sequelae include joint fusion, accumulation of calcified foci within soft tissues, thoracic insufficiency syndrome, and progressive immobility. The authors report on a patient with FOP who developed severe spinal canal stenosis in the thoracic spine causing substantial myelopathy. He underwent a thoracic laminectomy and resection of a large posterior osteophyte. Unique considerations are required in treating patients with FOP, including steroid administration to prevent ossification and anesthetic technique. The nuances of neurosurgical and medical management as they pertain to this disease are discussed.

There has been increasing experience in the utilization of intraoperative magnetic resonance imaging (iMRI) for intracranial surgery. Despite this trend, only a few U.S centers have examined the use of this technology for transsphenoidal resection of tumors of the sella. We present the largest series in North America examining the role of iMRI for pituitary adenoma resection. We retrospectively reviewed our institutional experience of 59-patients who underwent transsphenoidal procedures for sellar and suprasellar tumors with iMRI guidance. Of these, 52 patients had a histological diagnosis of pituitary adenoma. The technical results of this subgroup were examined. A 1.5-T iMRI was integrated with the BrainLAB (Feldkirchen, Germany) neuronavigation system. The majority (94%) of tumors in our series were macroadenomas. Seventeen percent of tumors were confined to the sella, 49% had suprasellar extensions without involvement of the cavernous sinus, 34% had frank cavernous sinus invasion. All patients underwent at least one iMRI, and 19% required one or more additional sets of intraoperative imaging. In 58% of patients, iMRI led to the surgeon attempting more resection. A gross total resection was obtained in 67% of the patients with planned total resections. There was one case of permanent postoperative diabetes insipidus and no other instances of new hormone replacement. In summary, iMRI was most useful for tumors of the sella with and without suprasellar extension where the information from the iMRI extended the complete resection rate from 40 to 72% and 55 to 88%, respectively. As one would expect, it did not substantially increase the rate of resection of tumors with cavernous sinus invasion. Overall, iMRI was particularly useful in guiding resection safely, aiding in clinical decision making, and allowing identification and preservation of the pituitary stalk and normal pituitary gland. Limitations of the iMRI include a need for additional personnel and training as well as additional operative time, which diminishes over time as personnel learn to optimize workflow efficiency. Additional costs are mitigated in part by using the iMRI as an immediate postoperative scan. Other data emerging from our experience suggest that preservation of normal gland and thus avoidance of hypopituitarism may be improved by iMRI use, but longer follow-up periods are required to test this conclusion. iMRI can detect unsuspected complications sooner than routine postoperative imaging, potentially leading to improved outcomes. However, larger studies are needed.

BACKGROUND AND IMPORTANCE: Mucinous eccrine carcinoma (MEC) is a rare but distinct type of sweat gland tumor. MECs tend to recur locally, and their spread to distant organs is very uncommon. This article describes the first case of MEC metastasizing to the brain and the spine. CLINICAL PRESENTATION: A 45-year-old female presented with a 2-year history of a scalp mass in the occipital area with lymph node spread. She underwent excision of the mass and neck lymph node dissection. Pathology confirmed the diagnosis of MEC. Postoperatively, she received radiation to the involved areas. Four years later the patient presented with left hemiparesis and underwent craniotomy for gross total resection of the metastasis. This recurred after 2.5 years and she underwent another craniotomy for gross total resection followed by whole brain radiation. In addition, the patient had metastases to T11 vertebral body and the left C6 to 7 neural foramen. Moreover, the patient developed leptomeningeal disease in the spine. The metastases to the spine were treated with radiation therapy. The patient died 1.5 years later. CONCLUSION: Even though it is rare for MEC to spread to distant organs, physicians should be aware of the risk of metastatic invasion of the brain and spine and be vigilant about surveillance of these sites. MEC metastases to the brain should be treated aggressively with surgical resection followed by stereotactic radiosurgery to the tumor bed. Spine metastases should be treated with a combination of surgery and image-guided radiation therapy, depending on the degree of cord compression from epidural metastatic disease

OBJECTIVE: Medically refractory epilepsy is amenable to neurosurgical intervention if the epileptogenic focus is accurately localized. If the scalp video-electroencephalography (EEG) and magnetic resonance imaging are nonlateralizing, yet a single focus is suspected, video-EEG monitoring with bilateral intracranial electrode placement is helpful to lateralize the ictal onset zone. We describe the indications, risks, and utility of such bilateral surveys at our institution. METHODS: We retrospectively reviewed 26 patients with medically refractory seizures who were treated over a 5-year period and underwent bilateral placement of intracranial electrodes. Subdural strips were used in all cases, and additional stereotactic implantation of depth electrodes into mesial temporal lobes occurred in 50%. The mean patient age was 37.7 years, and 65.4% of patients were male. RESULTS: The most common indication for bilateral invasive monitoring was bilateral ictal onsets on surface video-EEG (76.9%), followed by frequent interictal spikes contralateral to a single ictal focus (7.7%). Intracranial monitoring lasted an average of 8.2 days, with ictal events recorded in all cases. Ten patients (38.5%) subsequently underwent more extensive unilateral monitoring via implantation of subdural and depth electrodes through a craniotomy. A therapeutic procedure was performed in 17 patients (65.4%), whereas 1 patient underwent a palliative corpus callosotomy (3.8%). Nine patients underwent a resection without unilateral invasive mapping. Reasons for no therapeutic surgery (n = 8) included multifocal onsets, failing the Wada test, refusal of further treatment, and negative intraoperative electrocorticogram. There was 1 surgical complication, involving a retained electrode fragment that was removed in a separate minor procedure. Of the 26 patients, 15 (57.7%) are now seizure-free or have seizure disorders that have substantially improved (modified Engel classes I and II). Of the 17 patients who underwent a potentially curative surgery, 13 (76.5%) were Engel classes I and II. CONCLUSION: Bilateral placement of subdural strip and depth electrodes for epilepsy monitoring in patients with nonlateralizing scalp EEG and/or discordant imaging studies but clinical suspicion for focal seizure origin is both safe and effective. Given the safety and efficacy of this procedure, epileptologists should have a low threshold to consider bilateral implants for suitable patients

Pharmacologic therapy represents the first line of treatment of epilepsy and is effective in most patients. However, about 20% to 30% of cases develop intractable seizures that cannot be controlled by medication alone. In such cases, surgical intervention is considered for therapeutic, often curative purposes. Dynamic spatiotemporal variability in the epileptic focus renders seizure localization a challenge to the clinician. Many diagnostic modalities have been developed to identify different aspects of the epileptic focus. Older techniques are being increasingly supplemented by a variety of anatomic and functional imaging modalities that can help clarify discrepancies. Invasive electroencephalography remains the gold standard for identifying epileptic foci and guiding the surgeon to successful resections

Human embryonic stem cells (hESCs) have enormous potential for applications in basic biology and regenerative medicine. However, harnessing the potential of hESCs toward generating homogeneous populations of specialized cells remains challenging. Here we describe a novel technology for the genetic identification of defined hESC-derived neural cell types using bacterial artificial chromosome (BAC) transgenesis. We generated hESC lines stably expressing Hes5::GFP, Dll1::GFP, and HB9::GFP BACs that yield green fluorescent protein (GFP)(+) neural stem cells, neuroblasts, and motor neurons, respectively. Faithful reporter expression was confirmed by cell fate analysis and appropriate transgene regulation. Prospective isolation of HB9::GFP(+) cells yielded purified human motor neurons with proper marker expression and electrophysiological activity. Global mRNA and microRNA analyses of Hes5::GFP(+) and HB9::GFP(+) populations revealed highly specific expression signatures, suggesting that BAC transgenesis will be a powerful tool for establishing expression libraries that define the human neural lineage and for accessing defined cell types in applications of human disease

OBJECT: In this retrospective analysis the authors describe the assessment and outcomes of 90 patients who underwent placement of posterior instrumentation at the cervicothoracic junction following the resection of a primary or metastatic tumor during a 10-year period. METHODS: All patients underwent a posterolateral laminectomy including uni- or bilateral facetectomy, and 44 patients additionally required vertebral body resection and reconstruction. In patients who underwent C-6 or C-7 decompression, the posterior instrumentation strategies changed from the use of lateral mass plate systems (LMPSs) to lateral mass screw/rod systems (LMSRSs). Similarly, for T1-3 tumor decompression, the strategy shifted from sublaminar hook/rod systems (SHRSs) to the use of pedicle screw systems (PSSs) in which the surgeon used either a 6.25-mm rod or dual-diameter rods with or without a connector. RESULTS: The overall surgical complication rate was 19% including fixation failure in 11 patients (12%), 6 of whom required reoperation. Fixation failure rates for cervical decompression decreased from 2 (29%) of 7 patients in the LMPS group to 0 (0%) of 8 in the LMSRS group (p = 0.2). The fixation failure rates for thoracic decompression were 7 (15%) of 48 patients in the SHRS group, and there was a decrease to 2 (7%) of 27 in the PSS group (p = 0.48). Neurological and functional outcomes including American Spinal Injury Association, Eastern Cooperative Oncology Group, and Medical Research Council muscle strength and pain scores remained stable or improved in 94, 96, 100, and 96% of patients, respectively. CONCLUSIONS: Current posterior instrumentation strategies involving LMSRSs and PSSs provide excellent and safe stabilization of the cervicothoracic junction following resection of primary or metastatic tumors