Neurology Commons^™

Case Report: Hemorrhage Into An Occult Spinal Ependymoma After Epidural Anesthesia, Peter G. Campbell Md, Ashwini D. Sharan Md, James S. Harrop Md, Alex R. Vaccaro Md, John K. Ratliff Md

JHN Journal

Epidural anesthesia is a procedure which is well tolerated and has a low incidence of adverse events. In performing caesarean sections, regional anesthesia (spinal or epidural) is the preferred modality for anesthetic delivery. Although rare with continuous epidural anesthesia, epidural hematomas have been reported to occur with an incidence between 1:150,000 and 1:190,00010. An underlying bleeding diathesis has been implicated as a causative factor. We present the sixth reported case of hemorrhage into an occult intradural neoplasm after spinal or epidural anesthesia. Similar lesions have not been reported in the recent spine literature.

Endovascular Cure Of A "Locked-In" Patient, Meryl A. Severson Iii Md, Aditya S. Pandey Md, Erol Veznedaroglu Md

JHN Journal

We report the case of a 60 year-old right-handed gentleman who became acutely “Locked-In” several days after a lower extremity orthopedic procedure. He underwent emergent endovascular treatment and had complete resolution of his neurologic findings except for mild dysarthria and dysphagia. Endovascular intervention for posterior circulation thrombosis is highly effective when patients are treated within 24 hours.

Primary Stroke Centers: Their Role And Impact On Acute Stroke Management, Carissa C. Pineda Md, Jeanne Birch Rn, Bsn

JHN Journal

The management of acute ischemic stroke has been recognized as a significant medical problem. Stroke remains to be the third leading cause of death and the leading cause of long-term disability; 80% of all strokes are ischemic (a blood clot disrupts blood flow), and the rest are hemorrhagic (a blood vessel ruptures in the brain). Nearly 1 in 15 deaths in 2003 were the result of a stroke.1 Approximately 750,000 new strokes occur annually, of which 250,000 result in the deaths in the United States alone. The latest estimates for stroke costs total to about $30 billion in direct costs, …

Understanding Neurosurgery Through Experimental And Computer Models, Ciro Randazzo Md, Aditya S. Pandey Md, Erol Veznedaroglu Md

JHN Journal

Endovascular neurosurgery is an evolving field, with the goal of treating neurological disease with minimal invasion of the body. The current approach is to deliver focused therapies via catheters traveling through the vascular tree. Refinement and advancement of these techniques requires not just new ideas, but new ideas that hold up when tested through the scientific method.

Before clinical trials can begin, ethics and law demand that the ideas are tested first under experimental models. One benefit of these pre-clinical tests is the early identification of side effects. Another is the opportunity to practice and polish surgical technique in advance …

Jhn Journal (Download The Full Pdf Of This Issue), Robert H. Rosenwasser Md

JHN Journal

Complete JHN Journal Issue, Vol. 4, Issue 1, June 2008.

Interrelation Of Inflammation And App In Sibm: Il-1 Beta Induces Accumulation Of Beta-Amyloid In Skeletal Muscle., Jens Schmidt, Konstanze Barthel, Arne Wrede, Mohammad Salajegheh, Mathias Bähr, Marinos C Dalakas

Department of Neurology Faculty Papers

Distinct interrelationships between inflammation and beta-amyloid-associated degeneration, the two major hallmarks of the skeletal muscle pathology in sporadic inclusion body myositis (sIBM), have remained elusive. Expression of markers relevant for these pathomechanisms were analysed in biopsies of sIBM, polymyositis (PM), dermatomyositis (DM), dystrophic and non-myopathic muscle as controls, and cultured human myotubes. By quantitative PCR, a higher upregulation was noted for the mRNA-expression of CXCL-9, CCL-3, CCL-4, IFN-gamma, TNF-alpha and IL-1 beta in sIBM muscle compared to PM, DM and controls. All inflammatory myopathies displayed overexpression of degeneration-associated markers, yet only in sIBM, expression of the mRNA of amyloid precursor …

Whiplash: Diagnosis, Treatment, And Associated Injuries., Sanjay Yadla, John K. Ratliff, James Harrop

Department of Neurology Faculty Papers

STUDY DESIGN: Focused review of the current literature.

OBJECTIVE: To identify and synthesize the most current data pertaining to the diagnosis and treatment of whiplash and whiplash-associated disorders (WAD), and to report on whiplash-related injuries.

METHODS: A search of OVID Medline (1996-January 2007) and the Cochrane database of systematic reviews was performed using the keywords whiplash and WAD. Articles under subheadings for pathology, diagnosis, treatment, and epidemiology were chosen for review after identification by the authors.

RESULTS: A total of 485 articles in the English language literature were identified. Thirty-six articles pertained to the diagnosis, treatment, epidemiology of whiplash, and …

Rehabilitation Of Neuromyelitis Optica (Devic’S Syndrome): 3 Case Reports, Adam L. Schreiber, Guy W. Fried, Christopher S. Formal, Bryan X. Desouza

Department of Rehabilitation Medicine Faculty Papers

We describe the inpatient clinical rehabilitation course of three patients with neuromyelitis optica (NMO; Devic’s Syndrome). These patients had varying functional deficits. Each patient improved in several functional independence measures (FIM domains), but had minimal to no progress in other domains after acute rehabilitation stays between 1 to 1.5 months. NMO is a severe central nervous system demyelinating syndrome distinct from MS, characterized by optic neuritis, myelitis, and at least two of three criteria: longitudinally extensive cord lesion, MRI nondiagnostic for multiple sclerosis, or NMO-IgG seropositivity. Persons with NMO may demonstrate improved function with rehabilitation efforts; though gains may be …

A Novel And Generalizable Organotypic Slice Platform To Evaluate Stem Cell Potential For Targeting Pediatric Brain Tumors., Shengwen Calvin Li, William Gunter Loudon

Department of Cancer Biology Faculty Papers

Brain tumors are now the leading cause of cancer-related deaths in children under age 15. Malignant gliomas are, for all practical purposes, incurable and new therapeutic approaches are desperately needed. One emerging strategy is to use the tumor tracking capacity inherent in many stem cell populations to deliver therapeutic agents to the brain cancer cells. Current limitations of the stem cell therapy strategy include that stem cells are treated as a single entity and lack of uniform technology is adopted for selection of clinically relevant sub-populations of stem cells. Specifically, therapeutic success relies on the selection of a clinically competent …

Elucidating A Normal Function Of Huntingtin By Functional And Microarray Analysis Of Huntingtin-Null Mouse Embryonic Fibroblasts., Hua Zhang, Sudipto Das, Quan-Zhen Li, Ioannis Dragatsis, Joyce Repa, Scott Zeitlin, György Hajnóczky, Ilya Bezprozvanny

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

BACKGROUND: The polyglutamine expansion in huntingtin (Htt) protein is a cause of Huntington's disease (HD). Htt is an essential gene as deletion of the mouse Htt gene homolog (Hdh) is embryonic lethal in mice. Therefore, in addition to elucidating the mechanisms responsible for polyQ-mediated pathology, it is also important to understand the normal function of Htt protein for both basic biology and for HD. RESULTS: To systematically search for a mouse Htt function, we took advantage of the Hdh +/- and Hdh-floxed mice and generated four mouse embryonic fibroblast (MEF) cells lines which contain a single copy of the Hdh …

Spinal Cord Stimulation: An Update., Steven Falowski, Amanda Celii, Ashwini Sharan

Department of Neurosurgery Faculty Papers

Spinal cord stimulation has been used in the treatment of many chronic pain disorders since 1967. In this update, the indications for spinal cord stimulation are reviewed with attention to recent publications. A focused review of the literature on abdominal and visceral pain syndromes is also provided. Furthermore, the technology has evolved from the use of monopolar electrodes to complex electrode arrays. Similarly, the power source has changed from a radio frequency-driven system to a rechargeable impulse generator. These topics are covered, along with a short discussion of implant technique. Finally, we include a review of complications of such therapy. …

Short-Wavelength Light Sensitivity Of Circadian, Pupillary, And Visual Awareness In Humans Lacking An Outer Retina, Farhan H. Zaidi, Joseph T. Hull, Stuart N. Peirson, Katharina Wulff, Daniel Aeschbach, Joshua J. Gooley, George C. Brainard, Kevin Gregory-Evans, Joseph F. Rizzo Iii, Charles A. Czeisler, Russell G. Foster, Merrick J. Moseley, Steven W. Lockley

Department of Neurology Faculty Papers

As the ear has dual functions for audition and balance, the eye has a dual role in detecting light for a wide range of behavioral and physiological functions separate from sight. These responses are driven primarily by stimulation of photosensitive retinal ganglion cells (pRGCs) that are most sensitive to short-wavelength ( approximately 480 nm) blue light and remain functional in the absence of rods and cones. We examined the spectral sensitivity of non-image-forming responses in two profoundly blind subjects lacking functional rods and cones (one male, 56 yr old; one female, 87 yr old). In the male subject, we found …

Developing "Human Functioning And Rehabilitation Research" From The Comprehensive Perspective., Gerold Stucki, Jan Dietrich Reinhardt, Gunnar Grimby, John Melvin

Department of Rehabilitation Medicine Faculty Papers

With the International Classification of Functioning, Disability and Health (ICF) the World Health Organization (WHO) has prepared the ground for a comprehensive understanding of Human Functioning and Rehabilitation Research, integrating the biomedical perspective on impairment with the social model of disability. This poses a number of old and new challenges regarding the enhancement of adequate research capacity. Here we will summarize approaches to address these challenges with respect to 3 areas: the organization of Human Functioning and Rehabilitation Research into distinct scientific fields, the development of suitable academic training programmes and the building of university centres and collaboration networks.

Teaching Case: Occipital Neuralgia In A Young Patient - Expert Commentary, Avi Ashkenazi

Department of Neurology Faculty Papers

Expert commentary on Carayannopoulos, AG. Teaching case: Occipital neuralgia in a young patient, 47(9):1367-1368, October 2007.

Regional Variability In Use Of A Novel Assessment Of Thoracolumbar Spine Fractures: United States Versus International Surgeons., John Ratliff, Neel Anand, Alexander R Vaccaro, Moe R Lim, Joon Y Lee, Paul Arnold, James Harrop, Raja Rampersaud, Christopher M Bono, Ralf H Gahr

Department of Neurosurgery Faculty Papers

BACKGROUND: Considerable variability exists in clinical approaches to thoracolumbar fractures. Controversy in evaluation and nomenclature contribute to this confusion, with significant differences found between physicians, between different specialties, and in different geographic regions. A new classification system for thoracolumbar injuries, the Thoracolumbar Injury Severity Score (TLISS), was recently described by Vaccaro. No assessment of regional differences has been described. We report regional variability in use of the TLISS system between United States and non-US surgeons.

METHODS: Twenty-eight spine surgeons (8 neurosurgeons and 20 orthopedic surgeons) reviewed 56 clinical thoracolumbar injury case histories, which included pertinent imaging studies. Cases were classified …

Teaching Case: Headache Stigmata, Michael Marmura, Seymour Solomon

Department of Neurology Faculty Papers

A 38-year-old man with past medical history of hypertension and obesity presented to our clinic for evaluation of severe headaches and multiple episodes of discoloration of the forehead on the same side as the headache.

Meeting Report: The Role Of Environmental Lighting And Circadian Disruption In Cancer And Other Diseases, Richard G. Stevens, David E. Blask, George C. Brainard, Johnni Hansen, Steven W. Lockley, Ignacio Provencio, Mark S. Rea, Leslie Reinlib

Department of Neurology Faculty Papers

Light, including artificial light, has a range of effects on human physiology and behavior and can therefore alter human physiology when inappropriately timed. One example of potential light-induced disruption is the effect of light on circadian organization, including the production of several hormone rhythms. Changes in light-dark exposure (e.g., by nonday occupation or transmeridian travel) shift the timing of the circadian system such that internal rhythms can become desynchronized from both the external environment and internally with each other, impairing our ability to sleep and wake at the appropriate times and compromising physiologic and metabolic processes. Light can also have …

Cephalalgiaphobia: A Possible Specific Phobia Of Illness., Mario F P Peres, Juliane P P Mercante, Vera Z Guendler, Felipe Corchs, Marcio A Bernik, Eliova Zukerman, Stephen Silberstein

Department of Jefferson Headache Center Papers and Presentations

Psychiatric comorbidity, mainly anxiety and depression, are common in chronic migraine (CM). Phobias are reported by half of CM patients. Phobic avoidance associated with fear of headache or migraine attack has never been adequately described. We describe 12 migraine patients with particular phobic-avoidant behaviours related to their headache attacks, which we classified as a specific illness phobia, coined as cephalalgiaphobia. All patients were women, mean age 42, and all had a migraine diagnosis (11 CM, all overused acute medications). Patients had either a phobia of a headache attack during a pain-free state or a phobia of pain worsening during mild …

Evidence Against Roles For Phorbol Binding Protein Munc13-1, Adam Adaptor Eve-1, Or Vesicle Trafficking Phosphoproteins Munc18 Or Nsf As Phospho-State-Sensitive Modulators Of Phorbol/Pkc-Activated Alzheimer App Ectodomain Shedding., Annat F Ikin, Mirsada Causevic, Steve Pedrini, Lyndsey S Benson, Joseph D Buxbaum, Toshiharu Suzuki, Simon Lovestone, Shigeki Higashiyama, Tomas Mustelin, Robert D Burgoyne, Sam Gandy

Farber Institute for Neuroscience Faculty Papers

ABSTRACT: BACKGROUND: Shedding of the Alzheimer amyloid precursor protein (APP) ectodomain can be accelerated by phorbol esters, compounds that act via protein kinase C (PKC) or through unconventional phorbol-binding proteins such as Munc13-1. We have previously demonstrated that application of phorbol esters or purified PKC potentiates budding of APP-bearing secretory vesicles at the trans-Golgi network (TGN) and toward the plasma membrane where APP becomes a substrate for enzymes responsible for shedding, known collectively as alpha-secretase(s). However, molecular identification of the presumptive "phospho-state-sensitive modulators of ectodomain shedding" (PMES) responsible for regulated shedding has been challenging. Here, we examined the effects on …

A Turner Syndrome Neurocognitive Phenotype Maps To Xp22.3., Andrew R Zinn, David Roeltgen, Gerry Stefanatos, Purita Ramos, Frederick F Elder, Harvey Kushner, Karen Kowal, Judith L Ross

Department of Pediatrics Faculty Papers

BACKGROUND: Turner syndrome (TS) is associated with a neurocognitive phenotype that includes selective nonverbal deficits, e.g., impaired visual-spatial abilities. We previously reported evidence that this phenotype results from haploinsufficiency of one or more genes on distal Xp. This inference was based on genotype/phenotype comparisons of individual girls and women with partial Xp deletions, with the neurocognitive phenotype considered a dichotomous trait. We sought to confirm our findings in a large cohort (n = 47) of adult women with partial deletions of Xp or Xq, enriched for subjects with distal Xp deletions. METHODS: Subjects were recruited from North American genetics and …

Hemicrania Continua-Like Headache Associated With Carotid Dissection May Respond To Indomethacin, Avi Ashkenazi, Muhammad A. Abbas, Dinesh K. Sharma, Stephen D. Silberstein

Department of Neurology Faculty Papers

Hemicrania continua (HC) is an idiopathic, chronic disorder characterized by a continuous, strictly unilateral headache associated with ipsilateral cranial autonomic symptoms. The symptoms of HC typically respond dramatically to indomethacin therapy. We describe a patient with traumatic internal carotid artery (ICA) dissection, who presented with a clinical picture mimicking HC that initially responded to indomethacin. Patients with a clinical picture similar to HC should be managed with a high index of suspicion for a possible cervical arterial dissection.

Liver Transplant Recipient With Calcineurin-Inhibitor Induced Pain Syndrome: A Case Report, Nethra S. Ankam, Md, Stanley R. Jacobs, Md

Department of Rehabilitation Medicine Faculty Papers

Setting: University Hospital

Patient: 65-year-old female status-post liver transplant secondary to cirrhosis.

Case Description: Prior to the rehabilitation consult on post-operative day 42, she had an episode of acute rejection requiring rapid escalation of cyclosporine dosage, later changed to high dose tacrolimus for immunosuppression, resulting in high blood levels of both calcineurin inhibitors. She then complained of paroxysms of 10/10 pain over her entire body not relieved by opioids despite escalation in medication by the acute pain service. She was not participating in a rehabilitation program because of pain. Examination revealed an anxious woman for whom any tactile stimulation …

Rehabilitation Of A Patient With Diabetic Myonecrosis: A Case Report, Nethra S. Ankam, Md, Vishal Kancherla, Do, Stanley R. Jacobs, Md

Department of Rehabilitation Medicine Faculty Papers

Setting: Inpatient rehabilitation unit at a university hospital

Patient: 37-year-old male with diabetic myonecrosis.

Case Description: The patient had a long-standing history of uncontrolled diabetes mellitus with multiple comorbidities, including end-stage renal disease on dialysis and diabetic myonecrosis of the left biceps femoris diagnosed by biopsy and magnetic resonance imaging (MRI.) On this admission, he presented with right leg pain and swelling, found to be a reoccurrence of diabetic myonecrosis in the vastus lateralis, medialis, and intermedius, diagnosed by MRI only. Prior to admission, he lived alone in a wheelchair inaccessible duplex and required minimal assistance with housekeeping.

Assessment/Results: …

Preventive Treatment Of Migraine, Stephen Silberstein

Department of Neurology Faculty Papers

Migraine is a common episodic pain disorder, the treatment of which can be acute to stop an attack or preventive to reduce the frequency, duration or severity of attacks. Preventive treatment is used when attacks are frequent or disabling. Many different medication groups are used for preventive treatment, including β-blockers, antidepressants and antiepileptic drugs. Their mechanisms of action include raising the threshold to migraine activation, enhancing antinociception, inhibiting cortical spreading depression, inhibiting peripheral and central sensitization, blocking neurogenic inflammation and modulating sympathetic, parasympathetic or 5-HT tone. In this article, I review evidence of the effectiveness of migraine preventive drugs. I …

Botulinum Toxin Type A In The Prophylactic Treatment Of Chronic Tension-Type Headache: A Multicentre, Double-Blind, Randomized, Placebo-Controlled, Parallel-Group Study, Stephen D. Silberstein, H. Göbel, R. Jensen, A. H. Elkind, R. Degryse, J. M.C.M. Walcott, C. Turkel

Department of Neurology Faculty Papers

We studied the safety and efficacy of 0 U, 50 U, 100 U, 150 U (five sites), 86 Usub and 100 Usub (three sites) botulinum toxin type A (BoNTA; BOTOX); Allergan, Inc., Irvine, CA, USA) for the prophylaxis of chronic tension-type headache (CTTH). Three hundred patients (62.3% female; mean age 42.6 years) enrolled. For the primary endpoint, the mean change from baseline in the number of TTH-free days per month, there was no statistically significant difference between placebo and four BoNTA groups, but a significant difference favouring placebo vs. BoNTA 150 was observed (4.5 vs. 2.8 tension headache-free days/month; P …

Comparison Of Dynamic (Brush) And Static (Pressure) Mechanical Allodynia In Migraine, C. Lopinto, W. B. Young, Avi Ashkenazi

Department of Neurology Faculty Papers

Allodynia has been described in migraine but has not been fully investigated for the different sensory modalities. The aim of this study was to compare the prevalence of dynamic (brush) and static (pressure) mechanical allodynia in migraine patients and to suggest a practical method of testing them in a clinical setting. Patients with International Headache Society-defined episodic migraine (EM) or with transformed migraine (TM) as defined by Silberstein and Lipton were prospectively recruited from the Jefferson Headache Center out-patient clinic. A questionnaire of migraine features and symptoms of allodynia was administered. Brush allodynia (BA) was tested by cutaneous stimulation with …

Light During Darkness And Cancer: Relationships In Circadian Photoreception And Tumor Biology, Samar A. Jasser, David E. Blask, George C. Brainard

Department of Neurology Faculty Papers

The relationship between circadian phototransduction and circadian-regulated processes is poorly understood. Melatonin, commonly a circadian phase marker, may play a direct role in a myriad of physiologic processes. The circadian rhythm for pineal melatonin secretion is regulated by the hypothalamic suprachiasmatic nucleus (SCN). Its neural source of light input is a unique subset of intrinsically photosensitive retinal ganglion cells expressing melanopsin, the primary circadian photopigment in rodents and primates. Action spectra of melatonin suppression by light have shown that light in the 446–477 nm range, distinct from the visual system’s peak sensitivity, is optimal for stimulating the human circadian system. …

Zonisamide For Migraine Prophylaxis In Refractory Patients, Avi Ashkenazi, Adam Benlifer, Jason Korenblit, Stephen D. Silberstein

Department of Neurology Faculty Papers

Zonisamide, a new antiepileptic drug, has been approved in the US as adjunctive therapy for the treatment of partial seizures in adults.^1,2 Chemically a sulfonamide analogue, zonisamide is thought to have several mechanisms of action, including a rate-dependent blockade of voltage-gated sodium channels and reduction of ion flow through T-type calcium channels.^3-5 It is also a weak carbonic anhydrase inhibitor. Zonisamide has a favorable pharmacokinetic profile that includes high oral bioavailability and a long half life (63 hours), permitting a once- or twice-daily dosing regimen.⁶

There are only a limited number of current migraine preventive medications that …

Measuring Sustained Attention After Traumatic Brain Injury: Differences In Key Findings From The Sustained Attention To Response Task (Sart), John Whyte, Patricia Grieb-Neff, Christopher Gantz, Marcia Polansky

Department of Rehabilitation Medicine Faculty Papers

Clinical reports after traumatic brain injury (TBI) suggest frequent difficulties with sustained attention, but their objective measurement has proved difficult. In 1997, Robertson and colleagues reported on a new sustained attention assessment tool, the sustained attention to response task (SART). Individuals with TBI were reported to produce more errors of commission on the SART than control participants, and both groups showed a relationship between SART errors and everyday lapses of attention as measured by the cognitive failures questionnaire (CFQ). Although few direct replications of these findings have been reported, the SART has been used widely as a measure of sustained …

Continuous Asl Perfusion Fmri Investigation Of Higher Cognition: Quantification Of Tonic Cbf Changes During Sustained Attention And Working Memory Tasks, Junghoon Kim, John Whyte, Jiongjiong Wang, Hengyi Rao, Kathy Z. Tang, John A. Detre

Department of Rehabilitation Medicine Faculty Papers

Arterial spin labeling (ASL) perfusion fMRI is an emerging method in clinical neuroimaging. Its non-invasiveness, absence of low frequency noise, and ability to quantify the absolute level of cerebral blood flow (CBF) make the method ideal for longitudinal designs or low frequency paradigms. Despite the usefulness in the study of cognitive dysfunctions in clinical populations, perfusion activation studies to date have been conducted for simple sensorimotor paradigms or with single-slice acquisition, mainly due to technical challenges. Using our recently developed amplitude-modulated continuous ASL (CASL) perfusion fMRI protocol, we assessed the feasibility of a higher level cognitive activation study in twelve …