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Nearly 90% of patients with glucose transporter type 1 deficiency syndrome (Glut1 DS; figure) have paroxysmal or constant gait abnormalities, including ataxic, spastic, ataxic-spastic, and dystonic gait.1,2 We report 3 cases of genetically proven Glut1 DS (table) demonstrating a distinctive paroxysmal gait disorder triggered by exertion or fasting, herein named “criss-cross gait” (video 1). It is characterized by lower-body choreo-dyskinesia causing the legs to intersect repeatedly, producing irregular, random steps combined with some loss of balance. Compensatory upper-body movements help maintain balance. In the appropriate clinical context, criss-cross gait should prompt evaluation for the treatable Glut1 DS and not be misinterpreted as functional.

A 68-year-old man complained of the subtle onset of postural instability. A detailed investigation revealed the presence of ataxia with pulling to the right, right face and contralateral (left) trunk and limb hypoesthesia, slight dysphagia, right eyelid ptosis, and enophthalmos (figure, A) caused by medullary infarct (figure, B) (Wallenberg syndrome). Due to snoring, the patient underwent polysomnography, which showed central sleep apnea with Biot breathing ataxia (figure, C). Biot periodic breathing is an irregular, gasping respiration caused by respiratory pacemaker lesions, observed in comatose patients with bulbar damage.1 Here, Biot breathing presented in the context of central sleep apnea.2 Medullary breathing control circuit lesions impair autonomic respiration, which is critical for nighttime respiration.

A 68-year-old woman presented with an insidious onset of strictly asymmetric left arm clumsiness (video at [Neurology.org][1]) in absence of any brain lesion but mild right hemisphere hypotrophy (figure). The presence of limb apraxia and bradykinesia fostered the clinical diagnosis of possible corticobasal syndrome, in the context of suspected corticobasal degeneration.1 Bradykinesia and apraxia are 2 possible different faces of hypokinesia; their correct discrimination is not a diagnostic problem if a higher-level disturbance of praxis is present. An isolated limb apraxia is a confounder, and its early recognition allows clinicians to suspect parkinsonism with apraxia as a prominent feature.2 The authors thank the patient and her family for participation and Dr. Matteo Paolucci for technical support on artwork. [1]: http://neurology.org/lookup/doi/10.1212/WNL.0000000000004180

Objective: To estimate the needed workforce of trained neurointerventionalists (NIs) to perform endovascular therapy (ET) for eligible patients with acute ischemic stroke (AIS). Method: Population and ischemic stroke incidence data were extracted with use of US Census and Centers for Disease Control and Prevention 2009 estimates. The annual “demand” is defined as the proportion of AIS patients who would meet inclusion criteria and clinical standards for ET. The “supply” is defined as the number of trained NIs and NIs in training. The “workforce” is the number of NIs needed to meet the demand (the number of eligible AIS patients) within an accessible geographic diameter. Data on NIs and NI fellowships were collected (Society of Neurointerventional Surgery [SNIS], Society of Vascular & Interventional Neurology [SVIN], Concentric Medical, and Penumbra Inc.). Results: The estimated number of NIs is close to 800, practicing within a 50-mile radius of major metropolitan areas in the United States, covering more than 95% of the US population. Approximately 40 NI fellows are graduating yearly from US training programs. In 5 years and 10 years, the number of NIs may reach 1,000 and 1,200, respectively. Currently, there are approximately 14,000 thrombectomy procedures performed in the United States each year. However, the percentage of AIS patients who may be eligible for ET in our estimation is 4% to 14%, or about 25,000 to 95,000 patients. This means that cases will occur at a rate of 26 to 97 per year in 5 years, or 22 to 81 per year in 10 years, for each NI. Providing 24/7 AIS coverage requires 2 to 3 NIs per medical center, adding to the challenge of providing manpower without diluting experience in areas of lower population density. Conclusion: The current and projected number of NIs would adequately supply the future need if the proportion of patients requiring AIS endovascular therapy increases. However, 2 to 3 NIs per comprehensive stroke center would be needed to provide 24/7 AIS therapy with a sufficient number of cases per NI. A tertiary stroke center model similar to the trauma model may provide the manpower solution without compromising the quality of care.

Background: Recanalization and angiographic reperfusion are key elements to successful endovascular and interventional acute ischemic stroke (AIS) therapy. Intravenous recombinant tissue plasminogen activator (rt-PA), the only established revascularization therapy approved by the US Food & Drug Administration for AIS, may be less effective for large artery occlusion. Thus, there is enthusiasm for endovascular revascularization therapies, which likely provide higher recanalization rates, and trials are ongoing to determine clinical efficacy and compare various methods. It is anticipated that clinical efficacy will be well correlated with revascularization of viable tissue in a timely manner. Method: Reporting, interpretation, and comparison of the various revascularization grading methods require agreement on measurement criteria, reproducibility, ease of use, and correlation with clinical outcome. These parameters were reviewed by performing a Medline literature search from 1965 to 2011. This review critically evaluates current revascularization grading systems. Results and Conclusion: The most commonly used revascularization grading methods in AIS interventional therapy trials are the thrombolysis in cerebral ischemia (TICI, pronounced “tissy”) and thrombolysis in myocardial ischemia (TIMI) scores. Until further technical and imaging advances can incorporate real-time reliable perfusion studies in the angio-suite to delineate regional perfusion more accurately, the TICI grading system is the best defined and most widely used scheme. Other grading systems may be used for research and correlation purposes. A new scale that combines primary site occlusion, lesion location, and perfusion should be explored in the future.

Over the past decade, endovascular therapy has emerged as a promising therapeutic approach for select patients with acute ischemic stroke. However, the morbidity, mortality, and complication rates in intra-arterial recanalization trials are higher than in the National Institute of Neurological Disorders and Stroke trial of IV tissue plasminogen activator. This review discusses common complications associated with endovascular therapy for acute ischemic stroke, avoidance of complications, and management of some of the common complications.

Background: A recently devised test of motor cortex excitability (short latency afferent inhibition) was shown to be sensitive to the blockade of muscarinic acetylcholine receptors in healthy subjects. The authors used this test to assess cholinergic transmission in the motor cortex of patients with AD. Methods: The authors evaluated short latency afferent inhibition in 15 patients with AD and compared the data with those of 12 age-matched healthy controls. Results: Afferent inhibition was reduced in the patients (mean responses ± SD reduced to 85.7% ± 15.8% of the test size) compared with controls (mean responses ± SD reduced to 45.3% ± 16.2% of the test size; p t -test). Administration of a single oral dose of rivastigmine improved afferent inhibition in a subgroup of six patients. Conclusions: The findings suggest that this method can be used as a noninvasive test of cholinergic pathways in AD. Future studies are required to evaluate whether short latency afferent inhibition measurements have any consistent clinical correlates.

Dorsal column spinal stimulation in dopamine-depleted rodents was recently reported to disrupt pathologic corticostriatal synchronization, alleviate akinesia, and restore locomotion.1 This claim has prompted consideration that spinal stimulation “might become an efficient and less invasive alternative for treatment of Parkinson disease (PD) in the future.” In this study, we investigated whether dorsal column stimulation was of therapeutic benefit in 2 patients with PD. ### Level of evidence. This study provides Class II evidence that for patients with moderate to severe motor impairment from PD, high-frequency epidural cervical spinal cord stimulation does not significantly improve motor function as measured by the motor subsection of the Unified Parkinson's Disease Rating Scale (UPDRS). ### Methods. Two patients with PD had spinal stimulators (Medtronic models 3487a or 3898) implanted surgically into the high cervical epidural space without complication, as described previously.2 Patient 1, 75 years old, had moderate motor impairment (off/on medication motor UPDRS = 30/18). Patient 2, 77 years old, had more severe motor impairment (off/on medication UPDRS = 51/38) and was unable to walk without dopaminergic medication. Both patients met UK Brain Bank criteria for PD.3 Ten days postoperatively, both patients participated in a double-blind crossover study of the motor effects of spinal stimulation. In an initial exploratory phase, an antiparkinsonian effect of spinal stimulation was sought over a range of frequencies (30–300 Hz) and intensities (up to 4.0 V and 240 μs). Having failed to establish a clear benefit for any particular set of parameters, for the purposes of the study, patient 1 was …

Short latency trigemino-cervical reflexes can be recorded from sternocleidomastoid muscle after stimulation of the infraorbital branch of the trigeminal nerve. We studied the trigemino-cervical reflexes and the conventional blink reflex in three patients with an isolated lesion in the medulla oblongata, eight patients with multiple sclerosis, and two patients with supratentorial ischemic lesion. The trigemino-cervical response was abnormal in the patients with an isolated lesion in the medulla oblongata and in all multiple sclerosis patients, whereas both components of the blink reflex were preserved in the patients with a lesion in the medulla oblongata and in half of the patients with multiple sclerosis. The trigemino-cervical reflex was preserved in patients with supratentorial lesions, whereas the late component of the blink reflex was abnormal. These findings suggest that central pathways generating the trigemino-cervical reflex are confined to the medulla oblongata and that they are independent from those generating the long latency (R2) component of the blink reflex. The trigemino-cervical reflex may help in disclosing and localizing brainstem lesions.

Guidelines have been established for the management of acute ischemic stroke; however, specific recommendations for endovascular revascularization therapy are lacking. Burgeoning investigation of endovascular revascularization therapies for acute ischemic stroke, rapid device development, and a diverse training background of the providers performing the procedures underscore the need for practice recommendations. This review provides a concise summary of the Society of Vascular and Interventional Neurology endovascular acute ischemic stroke roundtable meeting. This document was developed to review current clinical efficacy of pharmacologic and mechanical revascularization therapy, selection criteria, periprocedure management, and endovascular time metrics and to highlight current practice patterns. It therefore provides an outline for the future development of multisociety guidelines and recommendations to improve patient selection, procedural management, and organizational strategies for revascularization therapies in acute ischemic stroke.

A 64-year-old man presented with a 5-month history of right neck and arm pain followed by progressive hand weakness. Cervical X-rays and MRI were normal. EMG disclosed lower trunk brachial plexopathy. Magnetic resonance (MR) neurography revealed right brachial plexus distortion with thickening of

Pharmacologic and mechanical endovascular acute ischemic stroke therapy aims to recanalize occluded cerebral vessels to achieve improved clinical outcome. Several limitations to achieving this goal have been identified; one of the least discussed challenges in the literature is reocclusion. The intraprocedure and periprocedural roles of anticoagulation and antiplatelet agents in preventing reocclusion are unclear. In this review, the role of antiplatelet and anticoagulation agents as an adjunctive to acute ischemic stroke endovascular revascularization therapy is discussed.

The glossopharyngeal, vagus, and accessory cranial nerves pass through the jugular foramen and may be involved by transforaminal tumors.1,2 Weakness of the muscles supplied by these cranial nerves may simulate the bulbar variant of ALS or myasthenia gravis. Here we report a 94-year-old woman with a schwannoma of the left jugular foramen (figure, …

A healthy 68-year-old woman presented with 2 years of recurrent episodes of right ptosis, constantly present after waking from sleep (figure 1). Clinical examination, brain MRI, EMG, and polysomnography were unremarkable (figure 2). In particular, there was no cranial nerve impairment. Few cases of idiopathic ptosis on awakening have been described, and they have been attributed to eyelid opening apraxia.1 This disorder needs to be differentiated from myasthenia gravis and other neuromuscular disorders.2 We propose that the term apraxia is not suitable to describe this clinical phenomenon, which could result from an abnormal, transient persistence of focal sleep-related muscular atonia.

A 71-year-old man presented recurrent transient episodes of anterograde and retrograde amnesia, since age 60 years, with a frequency of 10 per month. The attacks, preceded by vague abdominal discomfort, lasted from a few minutes to 2–3 hours. History was unremarkable except for a head trauma; he took no drugs. MRI showed a left meningoencephalocele, likely posttraumatic, in the …

Objective: To report feasibilty, safety and efficacy of endovascular embolization for middle cerebral artery (MCA) aneurysms. Background Middle cerebral artery (MCA) aneurysms can be treated by both craniotomy and endovascular coil embolization. However, given the complex aneurysm neck anatomy and location; it was felt that surgical approach may be more suitable. We report a feasibility, safety and efficacy of MCA aneurysms treated with endovascular embolization as our primary treatment modality. Design/Methods:We retrospectively reviewed the Neurointerventional database from July 2005 through October 2011, identifying patients who underwent endovascular embolization for MCA aneurysms with detachable coils. Data was collected on demographics, periprocedural complications and follow up imaging. Peri-procedural complications were reported as intra operative perforation (IOP), thromboembolic event (TEE), both were considered symptomatic if patient develop new neurological deficit lasting for ≥24 hours with increase in NIHSS of ≥2 compare to baseline not contributing to other etiology. Results:Total of 161/850(19%) patients underwent endovascular embolization for MCA aneurysms with average age of 55, 108(76%) were females, 105(73%) were Caucasians. There were 53(33%) patients presented with subarachnoid hemorrhage (SAH) with an average aneurysm maximum dome size of 6.74 ±3.24 and neck size of 3.6±1.85. Fifty four (34%) underwent stent assisted coil embolization, 14(9%) balloon assist coil embolization and 14(9%) required Y stent configuration. We had 100% success in treating all the aneurysms with IOP rate of 8(5%), and TEE rate of 23(14%) from which 3(2%) were symptomatic in each group. There were total of 138(86%) aneurysms who had follow up angiogram from which 20(12%) required retreatment for aneurysm recanalization. Conclusions: We report a case series of MCA aneurysm treated with EAE with 100% success rate with significant peri-procedural complication rate of 4%. We conclude that endovascular embolization can be performed safely and effectively for MCA aneurysms. Disclosure: Dr. Pandya has nothing to disclose. Dr. Lazzaro has nothing to disclose. Dr. Fitzsimmons has nothing to disclose. Dr. Lynch has nothing to disclose. Dr. Zaidat has received personal compensation for activities with Boston Scientific Corporation and Micrus Inc.

Objective: To present a case of Superficial Siderosis of the CNS in a patient with bilateral internal jugular vein narrowing from an external compression of elongated styloid processes. Background Superficial Siderosis of CNS is an uncommon disorder that results from hemosiderin deposition in leptomeninges, subpial layers of brain, and spinal cord from chronic subarachnoid hemorrhage (SAH). The source of SAH is not identified in half of the cases. We present a case of superficial siderosis in a patient with bilateral jugular vein narrowing causing venous congestion from an external compression of styloid processes. Design/Methods: Case report from a tertiary medical center. Results: A 59 year old female presented with slowly progressive gait ataxia, sensorineural hearing loss, and episodic headaches for 6 months. MRI of brain and spinal cord revealed a pituitary adenoma and T2 hypointensity involving the cerebellum, brainstem, and spinal cord consistent with superficial siderosis. CSF studies demonstrated elevated protein without xanthochromia. A dynamic cerebral venogram with extension and flexion views revealed severe bilateral internal jugular vein narrowing at the level of C1-C2, more severe with neck flexion, with considerable congestion of the paravertebral veins and spinal epidural venous plexus. Manometry study revealed an increased pressure gradient across the narrowing between the sigmoid sinuses and internal jugular veins, which was worse with neck flexion compared to neutral position. Computerized tomography angiography of the neck demonstrated elongated styloid processes with external compression of the internal jugular veins at the level of the narrowing. The patient was referred to surgery for styloidectomy. Conclusions: We present a case of superficial siderosis with possible etiology related to bilateral internal jugular vein narrowing leading to significant venous congestion from an external compression of elongated syloid processes. Disclosure: Dr. Gupta has nothing to disclose. Dr. Pandya has nothing to disclose. Dr. Lazzaro has nothing to disclose. Dr. Fitzsimmons has nothing to disclose.