Ablation or deep brain stimulation in the internal segment of the globus pallidus (GPi) is an effective therapy for the treatment of Parkinson's disease (PD). Yet many patients receive only partial benefit, including varying levels of improvement across different body regions, which may relate to a differential effect of GPi surgery on the different body regions. Unfortunately, our understanding of the somatotopic organization of human GPi is based on a small number of studies with limited sample sizes, including several based upon only a single recording track or plane. To fully address the three-dimensional somatotopic organization of GPi, we examined the receptive field properties of pallidal neurons in a large cohort of patients undergoing stereotactic surgery. The response of neurons to active and passive movements of the limbs and orofacial structures was determined, using a minimum of three tracks across at least two medial-lateral planes. Neurons (3183) were evaluated from 299 patients, of which 1972 (62%) were modulated by sensorimotor manipulation. Of these, 1767 responded to a single, contralateral body region, with the remaining 205 responding to multiple and/or ipsilateral body regions. Leg-related neurons were found dorsal, medial and anterior to arm-related neurons, while arm-related neurons were dorsal and lateral to orofacial-related neurons. This study provides a more detailed map of individual body regions as well as specific joints within each region and provides a potential explanation for the differential effect of lesions or DBS of the GPi on different body parts in patients undergoing surgical treatment of movement disorders.

abbreviatioNs BrM = brain metastasis; GPA = Graded Prognostic Assessment; IORT = intraoperative radiotherapy; KPS = Karnofsky Performance Scale; LMD = leptomeningeal (metastatic) disease; NSCLCa = non-small cell lung cancer; RPA = recursive partitioning analysis; SRS = stereotactic radiosurgery; WBRT = whole-brain radiation therapy; XRS = radiation source. submitted January 30, 2014. accepted November 13, 2014. iNclude wheN citiNg Published online January 23, 2015; DOI: 10.3171/2014.11.JNS1449. disclosure The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. obJect The authors assessed the feasibility of intraoperative radiotherapy (IORT) using a portable radiation source to treat newly diagnosed, surgically resected, solitary brain metastasis (BrM). methods In a nonrandomized prospective study, 23 patients with histologically confirmed BrM were treated with an Intrabeam device that delivered 14 Gy to a 2-mm depth to the resection cavity during surgery. results In a 5-year minimum follow-up period, progression-free survival from the time of surgery with simultaneous IORT averaged (± SD) 22 ± 33 months (range 1-96 months), with survival from the time of BrM treatment with surgery+IORT of 30 ± 32 months (range 1-96 months) and overall survival from the time of first cancer diagnosis of 71 ± 64 months (range 4-197 months). For the Graded Prognostic Assessment (GPA), patients with a score of 1.5-2.0 (n = 12) had an average posttreatment survival of 21 ± 26 months (range 1-96 months), those with a score of 2.5-3.0 (n = 7) had an average posttreatment survival of 52 ± 40 months (range 5-94 months), and those with a score of 3.5-4.0 (n = 4) had an average posttreatment survival of 17 ± 12 months (range 4-28 months). A BrM at the treatment site recurred in 7 patients 9 ± 6 months posttreatment, and 5 patients had new but distant BrM 17 ± 3 months after surgery+IORT. Six patients later received whole-brain radiation therapy, 7 patients received radiosurgery, and 2 patients received both treatments. The median Karnofsky Performance Scale scores before and 1 and 3 months after surgery were 80, 90, and 90, respectively; at the time of this writing, 3 patients remain alive with a CNS progression-free survival of > 90 months without additional BrM treatment. coNclusioNs The results of this study demonstrate the feasibility of resection combined with IORT at a dose of 14 Gy to a 2-mm peripheral margin to treat a solitary BrM. Local control, distant control, and long-term survival were comparable to those of other commonly used modalities. Surgery combined with IORT seems to be a potential adjunct to patient treatment for CNS involvement by systemic cancer. Clinical trial registration no.: NCT00107367 (clinicaltrials.gov)

A review of the literature reveals fewer than ten previous reports of familial CMI+S in the past 30 years. Although rare, the existence of familial cases of CMI+S suggests a genetic component to the pathogenesis of this condition in at least a proportion of patients. Neurosurgeons should be aware of the familial aggregation of CMI+S.

✓ Angiography is often performed to identify the vascular supply of hemangioblastomas prior to resection. Conventional two-dimensional (2D) digital subtraction (DS) angiography and three-dimensional (3D) DS angiography provides high-resolution images of the vascular structures associated with these lesions. However, such 3D DS angiography often does not provide reliable anatomical information about nearby osseous structures, or when it does, resolution of vascular anatomy in the immediate vicinity of bone is sacrificed. A novel angiographic reconstruction algorithm was recently developed at The Johns Hopkins University to overcome these inadequacies. By combining two separate sequences of images of bone and blood vessels in a single 3D representation, 3D fusion DS (FDS) angiography provides precise topographic information about vascular lesions in relation to the osseous environment, without a loss of resolution. In this paper, the authors present the cases of two patients with cervical spine hemangioblastomas who underwent preoperative evaluation with FDS angiography and then successful gross-total resection of their tumors. In both cases, FDS angiography provided high-resolution 3D images of the hemangioblastoma anatomy, including each tumor’s topographic relationship with adjacent osseous structures and the location and size of feeding arteries and draining veins. These cases provide evidence that FDS angiography represents a useful adjunct to magnetic resonance imaging and 2D DS angiography in the preoperative evaluation and surgical planning of patients with vascular lesions in an osseous environment, such as hemangioblastomas in the spinal cord.

Progression to paraparesis was consistent in all the VX2-injected animals, with predictable onset of paraparesis occurring approximately 17 days postinjection. Histopathological and radiological characteristics of the VX2 intramedullary tumor are comparable with those of aggressive primary human IMSCTs. Establishment of this novel animal tumor model will facilitate the testing of new therapeutic paradigms for the treatment of IMSCTs.