Reversible Focal Dystonia Secondary to Giant Perivascular Spaces.

Giant perivascular spaces (PVS) are rare, CSF-filled, dilated cavities lined by the pia mater that are most often asymptomatic radiological findings but can sometimes cause neurological symptoms. We present two exceptional cases of secondary focal dystonia induced by the mass effect on the basal ganglia caused by giant diencephalic PVSs. In both cases, we chose a surgical stereotactic drainage approach as a treatment strategy that allowed controlling the movement disorders. The beneficial therapeutic effect was sustained at long-term follow-up. We believe that similar cases could be addressed successfully with this method.

Pulse generator battery life in deep brain stimulation: out with the old in with the less durable?

BACKGROUND: Battery life of the most commonly used implantable pulse generators in deep brain stimulation is limited. Device replacement is costly and may expose patients to additional risks. Driven by the observation that in our experience newer generation devices seemed to need earlier replacement than the older generation, we aimed to retrospectively analyze the battery life of two generations of non-rechargeable devices, manufactured by a single company (Medtronic, USA). METHODS: Battery life of 281 devices in 165 patients was taken into account for data analysis. This represented 243 older generation devices (Kinetra and Soletra) and 38 newer generation devices (Activa). RESULTS: The battery life of older generation stimulators was 2-fold longer than the newer generation. CONCLUSIONS: Newer devices are more versatile than the older generation. Their battery life is however significantly shorter. Development of next-generation devices needs to address this issue in order to limit health risks and reduce financial costs.

Delayed presentation of deep penetrating trauma to the subaxial cervical spine.

PURPOSE: To present a rare case of deep penetrating neck trauma in which a retained foreign body in the cervical spine (a broken knife blade) resulted in delayed radicular injury. We describe the surgical management using a retrojugular approach. CASE REPORT: Our patient sustained a stab wound to the supraclavicular triangle from a small pocketknife. He was initially managed in a local hospital by simple primary wound closure without any radiological examinations, and was discharged home. The patient re-consulted in a delayed fashion with mild local persistent neck pain. Subsequent radiological investigations revealed a foreign body (the broken blade of a pocket knife) embedded in the left neural foramen between the C6 and C7 vertebrae penetrating the disc space. The blade was lying between the left C7 nerve root and the ipsilateral vertebral artery (VA) at the transition of V1 and V2 segments. Initial neurological evaluation was normal. Some days later, the patient developed a delayed left C7 radicular deficit. We undertook urgent exploration along the wound corridor through a retrojugular, transforaminal approach with successful removal of the blade. DISCUSSION: To our knowledge, this is a unique case where a retained foreign body penetrated the soft tissues of the neck, embedding deep in the vertebral column without vascular, aerodigestive or significant primary neurological injury, while causing delayed neck pain and delayed onset radicular injury. We describe our surgical management for removal of the retained blade. The retrojugular approach gives excellent access to all of the important anatomical structures of the neck from an anterolateral approach.

Rupture of a concealed aneurysm after intravenous thrombolysis of a thrombus in the parent middle cerebral artery.

BACKGROUND: Management of ischemic stroke in the presence of aneurysmal brain disease is controversial. Recent retrospective evidence suggests that in selected patients, intravenous thrombolysis (IVT) remains a safe approach for reperfusion. METHODS: We document a case of post-thrombolysis aneurysmal rupture. Supported by additional scientific literature we postulate that acute aneurysmal thrombosis leading to stroke in the culprit artery may be an ominous sign of rupture and should be considered separately from fortuitously discovered distant aneurysmal disease. RESULTS: A 71-year-old female presented with an acute right middle cerebral artery stroke syndrome. IVT allowed vessel reperfusion and revealed a previously concealed, juxtaposed non-giant M1 segment saccular aneurysm. Secondary aneurysmal rupture ensued. The aneurysm was secured by surgical clipping. Postoperative course was uneventful. CONCLUSIONS: This case shows that despite reports of thrombolysis safety in the presence of brain aneurysms, thrombolysis remains potentially hazardous and hints toward an increased risk when the stroke arises on the parent vessel itself.

A Comprehensive Training Model for Simulation of Intracranial Aneurysm Surgery Using a Human Placenta and a Cadaveric Head.

BACKGROUND AND OBJECTIVES: Aneurysmal surgery is technically complex, and surgeon experience is an important factor in therapeutic success, but training young vascular neurosurgeons has become a complex paradigm. Despite new technologies and simulation models, cadaveric studies still offer an incomparable training tool with perfect anatomic accuracy, especially in neurosurgery. The use of human placenta for learning and improving microsurgical skills has been previously described. In this article, we present a comprehensive simulation model with both realistic craniotomy exposure and vascular handling consisting of a previously prepared and perfused human placenta encased in a human cadaveric specimen. METHODS: Humans' placentas from the maternity and cadaveric heads from the body donation program of the anatomy laboratory were used. Placentas were prepared according to the established protocol, and aneurysms were created by catheterization of a placental artery. Ten participants, including senior residents or young attendees, completed an evaluation questionnaire after completing the simulation of conventional unruptured middle artery aneurysm clipping surgery from opening to closure. RESULTS: The skin incision, muscle dissection, and craniotomy were assessed as very similar to reality. Brain tissue emulation and dissection of the lateral fissure were judged to be less realistic. Vascular management was evaluated as similar to reality as closure. Participants uniformly agreed that this method could be implemented as a standard part of their training. CONCLUSION: This model could provide a good model for unruptured aneurysm clipping training.

Endoscopic Transfontanellar Approach of a Cyst Anterior to the Brainstem Crossing the Foramen Magnum-Part II: Success of Multiple Fenestrations: Two-Dimensional Operative Video.

Premature infants with severe germinal matrix/intraventricular hemorrhage are at high risk of posthemorrhagic ventricular dilation and hydrocephalus (Video 1). We report a 4-month-old premature infant with grade III intraventricular hemorrhage and compartmentalized hydrocephalus with an unusual craniospinal cyst. The cyst extended anteriorly from the mesencephalon to the posterior wall of C6, causing severe compression of the brainstem and spinal cord. An endoscopic procedure was performed first to achieve a unique cranial fenestration, which is detailed in "Endoscopic Transfontanellar Approach of a Cyst Anterior to the Brainstem Crossing the Foramen Magnum-Part I: Failure of Unique Fenestration." One month later the clinical picture recurred. Magnetic resonance imaging revealed hydrocephalus and cyst recurrence. A second procedure with endoscopic fenestration and shunt revision was needed. The same right transfontanellar approach was chosen, using a straight 30° endoscope. The procedure and surgical technique are explained in a step-by-step fashion. Extreme care was taken to align the head and the cervical spine. This allowed access to the extreme caudal cyst membrane posterior to C6 to create multiple transfixing fenestrations of the cyst. At 18-month follow-up, the child demonstrated almost normal neurological and psychomotor development with no cyst recurrence or hydrocephalus. Our report underlines the importance of performing multiple fenestrations of such cysts. We believe that performing a transfixing fenestration through the cyst allows cerebrospinal fluid flow and prevents recurrence, as, for instance, has been shown for suprasellar arachnoid cysts.

Endoscopic Transfontanellar Approach of a Cyst Anterior to the Brainstem Crossing the Foramen Magnum-Part I: Failure of Unique Fenestration: 2-Dimensional Operative Video.

Premature infants with severe germinal matrix intraventricular hemorrhage (IVH) are at high risk of posthemorrhagic ventricular dilation and hydrocephalus (Video 1). We report the case of a 3-month-old premature infant referred for posthemorrhagic multilocated hydrocephalus. He presented with somnolence, hypotonia, and a bulging fontanelle. His past medical history included an IVH grade III with hydrocephalus initially treated by temporary ventriculosubgaleal shunting. Magnetic resonance imaging (MRI) showed persistent hydrocephalus associated to a cyst extending anteriorly from the mesencephalon to the posterior wall of the sixth cervical vertebra, causing severe brainstem and spinal cord compression. A two-step surgery was performed, consisting of an endoscopic procedure and a ventriculo peritoneal shunt placement, to achieve hydrocephalus treatment and cyst fenestration. A right transfontanellar approach with the head flexed was chosen, using a straight 30-degree endoscope. The procedure and surgical technique are explained in a step-by-step fashion in the original conditions. A large unique fenestration was performed with no postoperative complications and improvement of the neurological status. One month later the patient presented again with symptomatic intracranial hypertension with compatible clinical and imagery findings. MRI showed cyst recurrence and hydrocephalus. We therefore revised our strategy and performed an additional procedure, which is detailed in the second part of the video (Part II: Success of Multiple Fenestrations). Our report underlines the high risk of recurrence of such cysts in post-hemorrhagic arachnoiditis. Cyst recurrence may be explained by the absence of crossing flow when a unique perforation is made.

Using Smartphones for Clinical Assessment in Cervical Spondylotic Myelopathy a Feasibility Study.

Degenerative cervical myelopathy (DCM) is characterized by a progressive deterioration in spinal cord function. Its evaluation requires subjective clinical examination with wide interobserver variability. Objective quantification of spinal cord function remains imprecise, even though validated myelopathy-grading scales have emerged and are now widely used. We created a Smartphone Application, the N-Outcome App, with the aim of quantifying accurately and reliably spinal cord dysfunction using a 5-minute Test. A patient suffering from DCM was clinically evaluated before surgery, at 3 and 6 months follow-up after surgical decompression of the cervical spinal cord. Standard scores (Nurick grade, modified Japanese Orthopedic Association (mJOA) score) were documented at these time points. A 5-minute motor and proprioceptive performance test aided by a smartphone with the N-outcome App was also performed. Motor performance in rapid alternating movements and finger tapping improved in correlation with improvements in standard grading scale scores. Clinical improvements were seen in maximum reflex acceleration and in Romberg testing which showed less closed/open eyes variation, suggesting pyramidal and proprioceptive function recovery. We demonstrate that using the N-Outcome App as an adjunct to clinical evaluation of compressive myelopathy is feasible and potentially useful. The results correlate with the results of clinical assessment obtained by standard validated myelopathy scores.

Foot Drop After Iliocaval Vein Stenting: Radicular Syndrome from Stent Misplacement in Spinal Canal.

BACKGROUND: Endovascular procedures have become more and more prevalent in both general vascular and neurosurgical practices. Because these procedures rely on real-time bidimensional control through fluoroscopic guidance, they can be prone to spatial misplacement in the third dimension when not controlled in 2 different radiologic planes. CASE DESCRIPTION: We report a unique complication of an iliocaval vessel stenting procedure with misplacement of a venous stent in the spinal canal. This case illustrates the close vascular relationship between the large venous vessels of the abdomen and pelvis and epidural plexus of the spinal canal. CONCLUSIONS: The complex venous anatomy of the lumbar region and lumbar epidural space is illustrated in this unique case. We believe this may serve both vascular surgeons and neurosurgeons in their daily practice.

Indications, Techniques, and Outcomes of Robot-Assisted Insular Stereo-Electro-Encephalography: A Review.

Stereo-electro-encephalography (SEEG) is an invasive, surgical, and electrophysiological method for three-dimensional registration and mapping of seizure activity in drug-resistant epilepsy. It allows the accurate analysis of spatio-temporal seizure activity by multiple intraparenchymal depth electrodes. The technique requires rigorous non-invasive pre-SEEG evaluation (clinical, video-EEG, and neuroimaging investigations) in order to plan the insertion of the SEEG electrodes with minimal risk and maximal recording accuracy. The resulting recordings are used to precisely define the surgical limits of resection of the epileptogenic zone in relation to adjacent eloquent structures. Since the initial description of the technique by Talairach and Bancaud in the 1950's, several techniques of electrode insertion have been used with accuracy and relatively few complications. In the last decade, robot-assisted surgery has emerged as a safe, accurate, and time-saving electrode insertion technique due to its unparalleled potential for orthogonal and oblique insertion trajectories, guided by rigorous computer-assisted planning. SEEG exploration of the insular cortex remains difficult due to its anatomical location, hidden by the temporal and frontoparietal opercula. Furthermore, the close vicinity of Sylvian vessels makes surgical electrode insertion challenging. Some epilepsy surgery teams remain cautious about insular exploration due to the potential of neurovascular injury. However, several authors have published encouraging results regarding the technique's accuracy and safety in both children and adults. We will review the indications, techniques, and outcomes of insular SEEG exploration with emphasis on robot-assisted implantation.

Multilevel Pedicular Osteotomies for En Bloc Resection of a Primary Ewing Sarcoma of the Subaxial Cervical Spine with Pedicle Screw Reconstruction.

BACKGROUND: Primary Ewing sarcoma of the mobile spine is a rare disease. Its management requires careful surgical planning, because radical, margin-free excision is directly correlated with prognosis. Extensive bone removal in the cervical spine can lead to instability and cause postoperative iatrogenic cervical deformity. Thus, spinal instrumentation plays an important role in restoring postresection spinal stability and improving quality of life. METHODS: We present a novel technique that allows successful removal of a large Ewing sarcoma of the subaxial cervical spine, infiltrating and traversing the posterior bone elements, and extending into the paraspinal muscles. This technique involves radical en bloc resection of posteriorly located cervical tumors via multilevel pediculotomy, with terminal vertebrae pedicle screw reconstruction. RESULTS: Terminal vertebrae cervical pedicle screw reconstruction allowed wide surgical excision with satisfactory oncologic and mechanical results. CONCLUSIONS: This technique enables 1-stage total tumor resection and stabilization and may be a viable alternative to radical en bloc resection of posteriorly located epidural malignant lesions of the cervical spine in selected cases.

MicroRNA-22 (miR-22) overexpression is neuroprotective via general anti-apoptotic effects and may also target specific Huntington's disease-related mechanisms.

BACKGROUND: Whereas many causes and mechanisms of neurodegenerative diseases have been identified, very few therapeutic strategies have emerged in parallel. One possible explanation is that successful treatment strategy may require simultaneous targeting of more than one molecule of pathway. A new therapeutic approach to have emerged recently is the engagement of microRNAs (miRNAs), which affords the opportunity to target multiple cellular pathways simultaneously using a single sequence. METHODOLOGY/PRINCIPAL FINDINGS: We identified miR-22 as a potentially neuroprotective miRNA based on its predicted regulation of several targets implicated in Huntington's disease (histone deacetylase 4 (HDAC4), REST corepresor 1 (Rcor1) and regulator of G-protein signaling 2 (Rgs2)) and its diminished expression in Huntington's and Alzheimer's disease brains. We then tested the hypothesis that increasing cellular levels of miRNA-22 would achieve neuroprotection in in vitro models of neurodegeneration. As predicted, overexpression of miR-22 inhibited neurodegeneration in primary striatal and cortical cultures exposed to a mutated human huntingtin fragment (Htt171-82Q). Overexpression of miR-22 also decreased neurodegeneration in primary neuronal cultures exposed to 3-nitropropionic acid (3-NP), a mitochondrial complex II/III inhibitor. In addition, miR-22 improved neuronal viability in an in vitro model of brain aging. The mechanisms underlying the effects of miR-22 included a reduction in caspase activation, consistent with miR-22's targeting the pro-apoptotic activities of mitogen-activated protein kinase 14/p38 (MAPK14/p38) and tumor protein p53-inducible nuclear protein 1 (Tp53inp1). Moreover, HD-specific effects comprised not only targeting HDAC4, Rcor1 and Rgs2 mRNAs, but also decreasing focal accumulation of mutant Htt-positive foci, which occurred via an unknown mechanism. CONCLUSIONS: These data show that miR-22 has multipartite anti-neurodegenerative activities including the inhibition of apoptosis and the targeting of mRNAs implicated in the etiology of HD. These results motivate additional studies to evaluate the feasibility and therapeutic efficacy of manipulating miR-22 in vivo.

Highlights of the Keystone Symposium: sirtuins in metabolism, aging and disease.

From February 12-16, 2012, leading members of the sirtuin scientific community assembled in Tahoe, CA to attend the Keystone Symposium "Sirtuins in Aging, Metabolism, and Disease." It was a vibrant and lively meeting, and in the spirit of Keystone Symposia, both established sirtuin researchers and those new to the field enjoyed a unique opportunity to interact and exchange ideas.