Faculty Bibliography

INTRODUCTION: Essential tremor can be treated by thalamic stimulation or ablation of the ventral intermediate nucleus (VIM) with good outcomes [1]. Routine magnetic resonance imaging (MRI) cannot distinguish between thalamic nuclei so targeting is based on anatomic atlas-based coordinates. Diffusion MRI-based track density imaging (TDI) can better depict internal thalamic structure [2], but previously has required high-field MRI or long acquisitions that are not clinically practical. We applied multiband diffusion MRI [3] to enable 3-Tesla (3-T) MRI TDI in patients with essential tremor. METHODS: Six patients with essential tremor underwent standard preoperative MRI with an additional multiband diffusion sequence that used 3-slice acceleration factor, 3-mm isotropic image resolution, whole-brain coverage (45 slices) and 256 diffusion gradient directions (b = 2500 s/mm) acquired in 11 minutes. TDI data post-processing generated track density and direction-encoded color maps at 500-micron isotropic super-resolution [2]. RESULTS: Combining TDI and multiband diffusion acquisitions resulted in high-quality images of the human thalamus in typical elderly essential tremor patients using 3-T MRI and clinically feasible scan times. Results also were consistent for repeat imaging in the 3 volunteers. TDI with or without direction-encoding demonstrated some of the internal anatomy of the thalamus, but fiber-orientation maps derived from these data (Fig. 1) were preferred by the 2 participating functional neurosurgeons. CONCLUSION: Multiband diffusion acquisition makes TDI-based parcellation of the thalamus feasible in elderly patients with essential tremor using 3-T MRI. This approach provides at least equivalent data to previous diffusion tractography or TDI approaches for thalamus parcellation, but without long scan times or a 7-Tesla MRI system [4-6]. While planning for gamma knife ablation of VIM for these initial 6 patients still relied on conventional methods, future efforts will focus on validation and careful introduction of TDI-derived thalamic maps to actual surgical planning.

Reports an error in "Safety and efficacy of peripheral nerve stimulation of the occipital nerves for the management of chronic migraine: Results from a randomized, multicenter, double-blinded, controlled study" by Stephen D. Silberstein, David W. Dodick, Joel Saper, Billy Huh, Konstantin V. Slavin, Ashwini Sharan, Ken Reed, Samer Narouze, Alon Mogilner, Jerome Goldstein, Terrence Trentman, Julien Vaisma, Joseph Ordia, Peter Weber, Timothy Deer, Robert Levy, Roni L. Diaz, Stephanie N. Washburn and Nagy Mekhail (Cephalalgia, 2012[Dec], Vol 32[16], 1165-1179). In the print version of this article, the author name Julien Vaisman was mis-spelled as Julien Vaisma. The correction was present in the erratum. (The following abstract of the original article appeared in record 2012-30866-002). Background: Chronic migraine (CM) is a debilitating neurological disorder with few treatment options. Peripheral nerve stimulation (PNS) of the occipital nerves is a potentially promising therapy for CM patients. Methods: In this randomized, controlled multicenter study, patients diagnosed with CM were implanted with a neurostimulation device near the occipital nerves and randomized 2:1 to active (n = 105) or sham (n = 52) stimulation. The primary endpoint was a difference in the percentage of responders (defined as patients that achieved a >50% reduction in mean daily visual analog scale scores) in each group at 12 weeks. Results: There was not a significant difference in the percentage of responders in the Active compared with the Control group (95% lower confidence bound (LCB) of -0.06; p = 0.55). However, there was a significant difference in the percentage of patients that achieved a 30% reduction (p = 0.01). Importantly, compared with sham-treated patients, there were also significant differences in reduction of number of headache days (Active Group = 6.1, baseline = 22.4; Control Group = 3.0, baseline = 20.1; p = 0.008), migraine-related disability (p = 0.001) and direct reports of pain relief (p = 0.001). The most common adverse event was persistent implant site pain. Conclusion: Although this study failed to meet its primary endpoint, this is the first large-scale study of PNS of the occipital nerves in CM patients that showed significant reductions in pain, headache days, and migraine-related disability. Additional controlled studies using endpoints that have recently been identified and accepted as clinically meaningful are warranted in this highly disabled patient population with a large unmet medical need.

ABSTRACT Introduction: Intracranial hypotension is a neurologic syndrome characterized by orthostatic headaches and, radiographically, by dural thickening and enhancement as well as subdural collections. Several of etiologies exist, including surgical dural violations, lumbar puncture, or spontaneous cerebrospinal fluid leak. Current management includes conservative management consisting of bed rest, caffeine, and hydration. When conservative management fails, open surgical or percutaneous options are considered. Currently, the gold standard in percutaneous management of intracranial hypotension involves the epidural injection of autologous blood. Recently, some therapies for intracranial hypotension have employed the use of epidural fibrin glue. Case Presentation: Two cases of patients with persistent postdural puncture headaches are presented. Epidural fibrin glue injection alleviated the orthostatic headaches of two patients with intracranial hypotension. Conclusion: Although consideration must be afforded for the potential risks of viral transmission and aseptic meningitis, the utilization of epidural fibrin glue injection as an alternative or adjunct to the epidural blood patch in the treatment of intracranial hypotension should be further investigated.

BACKGROUND: Deep brain stimulation (DBS) remains an experimental but promising treatment for patients with severe refractory Gilles de la Tourette syndrome (TS). Controversial issues include the selection of patients (age and clinical presentation), the choice of brain targets to obtain optimal patient-specific outcomes, and the risk of surgery- and stimulation-related serious adverse events. METHODS: This report describes our open-label experience with eight patients with severe refractory malignant TS treated with DBS. The electrodes were placed in the midline thalamic nuclei or globus pallidus, pars internus, or both. Tics were clinically assessed in all patients pre- and postoperatively using the Modified Rush Video Protocol and the Yale Global Tic Severity Scale (YGTSS). RESULTS: Although three patients had marked postoperative improvement in their tics (>50% improvement on the YGTSS), the majority did not reach this level of clinical improvement. Two patients had to have their DBS leads removed (one because of postoperative infection and another because of lack of benefit). DISCUSSION: Our clinical experience supports the urgent need for more data and refinements in interventions and outcome measurements for severe, malignant, and medication-refractory TS. Because TS is not an etiologically homogenous clinical entity, the inclusion criteria for DBS patients and the choice of brain targets will require more refinement.

OBJECTIVE: The clinical entity of thoracic radiculopathy following spinal cord stimulator (SCS) placement has not been previously described. MATERIALS AND METHODS: A retrospective review of prospectively acquired data on 172 patients, having undergone thoracic SCS placement at our institution, was performed. In addition, four patients were implanted at outside institutions, and were referred for revision. We examine our early experience with placement of thoracic SCS in surgically treated patients with chronic pain and 15 associated specific postoperative radicular pain complications along respective thoracic dermatomes. We postulate that preexisting thoracic spinal pathology affords less compliance in the placement of larger paddles, and subsequent radicular pain in a band-like abdominal fashion. RESULTS: A syndrome of thoracic radiculopathy, presenting as intractable lower thoracic or abdominal wall pain occurring in the immediate postoperative period, was identified in 15 patients. These patients subsequently underwent revision surgery, with either a more extensive laminectomy to further decompress the dorsal nerve roots or lead removal, both of which resulted in near immediate relief of symptoms. CONCLUSIONS: Thoracic radiculopathy may occur following SCS paddle lead placement. This clinical syndrome is characterized by its immediate postoperative development, band-like thoracic or abdominal pain pattern, severe pain that both overwhelms the incisional pain and is refractory to medications, and absence of motor deficit. The lateral placement of paddle leads increases the risk of radicular symptoms. Preoperative thoracic spine magnetic resonance imaging may be helpful in identifying patients who may be susceptible to this syndrome.

An 84-year-old man underwent deep brain stimulation (DBS) for mixed rest-action tremors. One year later, he developed a keratoma where the DBS wire emerged from the insertion cap. It was suspected to be a foreign body reaction and removed by a dermatologist but returned and grew conically over the next 4 years, reaching a height of 4 cm (figure). The surrounding skin began to break down with protrusion of the adjacent extension wire. We recommended removal of the lead but due to continued efficacy and advanced age, he decided to continue with the stimulator in place under observation.

BACKGROUND: Abetalipoproteinemia is a rare disorder of fat absorption, characterized by vitamin deficiency, acanthocytosis, and neurologic symptoms including ataxia and tremor. CASE REPORT: A 41-year-old male with abetalipoproteinemia is presented. He underwent staged bilateral thalamic deep brain stimulation (DBS) for the treatment of his tremors. After DBS, the patient achieved significant improvements in his tremors, ataxia, and quality of life. DISCUSSION: Thalamic DBS proved to be both safe and efficacious in the management of ataxia and tremors in a patient with abetalipoproteinemia. This is the first report of DBS in abetalipoproteinemia in the literature.