Neurology Commons^™

Case Study: Effects Of Psychosocial Factors On A Geriatric Patient With Guillain Barré Syndrome, Keane Evangelista, Mary Pent Hunter, Jessica Phung, Aum Tejani, Lee Anne H. Robotta

San Marcos, Summer 2019

The purpose of this case report is to determine how a change in intrinsic motivation and environmental factors help physical and functional improvements in a 77-year-old male patient with Guillain Barré Syndrome (GBS) in the geriatric population. Interventions to promote this change included patient education about lifestyle choices along with endurance and balance exercises.

Patient psychological state, community access, and general education about interventions/dosing had an effect on the patient's participation in his prescribed HEP. His comorbidities hindered his overall progress over the course of his treatment and should be addressed to improve patient outcomes. Activities that were more salient …

Effect Of Pregnancy On Growth Of Intracranial Meningiomas, Ayesha Quddusi, Muhammad Shahzad Shamim, Quratulain Virani

Section of Neurosurgery

Meningiomas are the most common benign primary intracranial tumours in adults. Their rapid growth during pregnancy has been well reported. Moreover, the relationship of increased progesterone expression during pregnancy and extensive tumour growth has been debated. In this review, we discuss not only the effect of progesterone on meningiomal growth but also other mechanisms involved in gestational meningiomas.

Ossification Of The Cruciform Ligament Of Atlas; A Rare Cause Of Cervical Myelopathy: Case Report And Review Of Literature, Muhammad Waqas Saeed Baqai, Gohar Javed, Mirza Zain Baig

Section of Neurosurgery

We present a case of cervical myelopathy secondary to ossification of the cruciform ligament (also known as cruciate ligament). This is a rare phenomenon that, to the best of our knowledge, has only been reported 16 times previously in literature. We have added a review of literature after our case presentation. We hope that by doing so, we may aid clinicians reach early diagnosis so as to be able to better manage this rare disease.

Perianal Necrotizing Fasciitis With Retroperitoneal Extension: A Case Report From Pakistan, Mirza Zain Baig, Abeer Aziz, Umme E Hani Abdullah, Muhammad Salman Khalil, Sumiya Abbasi

Section of Neurosurgery

Necrotizing fasciitis is a rare but potentially fatal condition. It is defined as a rapidly spreading infection of the subcutaneous soft tissue. Extension into the retroperitoneum may further complicate this deadly condition. We report a case of a 45-year-old gentleman who presented to our institute with perianal necrotizing fasciitis with extension into the retroperitoneum. He was managed with antibiotics and prompt surgical debridement. Our patient had a positive outcome which may be due to the fact that we had a high clinical suspicion, on the basis of which we opted for early operative management rather than delaying definitive treatment by …

Invasive Cryptococcal Meningitis Presenting As A Skull Base Mass In An Immunocompetent Host: A Case Report., Joshua Prickett, Juan J Altafulla, Anna Knisely, Zachary Litvack

Articles, Abstracts, and Reports

No abstract provided.

Global Spinal Deformity From The Upper Cervical Perspective. What Is "Abnormal" In The Upper Cervical Spine?, Peter G Passias, Haddy Alas, Renaud Lafage, Bassel G Diebo, Irene Chern, Christopher P Ames, Paul Park, Khoi D Than, Alan H Daniels, D Kojo Hamilton, Douglas C Burton, Robert A Hart, Shay Bess, Breton G Line, Eric O Klineberg, Christopher I Shaffrey, Justin S Smith, Frank J Schwab, Virginie Lafage

Articles, Abstracts, and Reports

Hypothesis: Reciprocal changes in the upper cervical spine correlate with adult TL deformity modifiers.

Design: This was a retrospective review.

Introduction: The upper cervical spine has remarkable adaptability to wide ranges of thoracolumbar (TL) deformity.

Methods: Patients >18 years with adult spinal deformity (ASD) and complete radiographic data at baseline (BL) and 1 year were identified. Patients were grouped into component types of the Roussouly classification system (Type 1: Pelvic incidence [PI]

Results: A total of 343 ASD patients were analyzed. When grouped by BL Schwab and Roussouly, Group

Conclusions: Our study suggests that upper cervical alignment remains relatively stable …

The Impact Of Osteotomy Grade And Location On Regional And Global Alignment Following Cervical Deformity Surgery., Peter G Passias, Samantha R Horn, Tina Raman, Avery E Brown, Virginie Lafage, Renaud Lafage, Justin S Smith, Cole A Bortz, Frank A Segreto, Katherine E Pierce, Haddy Alas, Breton G Line, Bassel G Diebo, Alan H Daniels, Han Jo Kim, Alex Soroceanu, Gregory M Mundis, Themistocles S Protopsaltis, Eric O Klineberg, Douglas C Burton, Robert A Hart, Frank J Schwab, Shay Bess, Christopher I Shaffrey, Christopher P Ames

Articles, Abstracts, and Reports

Introduction: Correction of cervical deformity (CD) often involves different types of osteotomies to address sagittal malalignment. This study assessed the relationship between osteotomy grade and vertebral level on alignment and clinical outcomes.

Methods: Retrospective review of a multi-center prospectively collected CD database. CD was defined as at least one of the following: C2-C7 Cobb >10°, cervical lordosis (CL) >10°, C2-C7 sagittal vertical axis (cSVA) >4 cm, and chin-brow vertical angle > 25°. Patients were evaluated for level and type of cervical osteotomy.

Results: 86 CD patients were included (61.4 ± 10.6 years, 66.3% female, body mass index 29.1 kg/m

Conclusions: CD …

A Technique For Approximating Transition Rates From Published Survival Analyses, Markian A. Pahuta, Joel Werier, Eugene K. Wai, Roy A. Patchell, Doug Coyle

Neurology Faculty Publications

Background: Quality-adjusted-life-years (QALYs) are used to concurrently quantify morbidity and mortality within a single parameter. For this reason, QALYs can facilitate the discussion of risks and benefits during patient counseling regarding treatment options. QALYs are often calculated using partitioned-survival modelling. Alternatively, QALYs can be calculated using more flexible and informative state-transition models populated with transition rates estimated using multistate modelling (MSM) techniques. Unfortunately the latter approach is considered not possible when only progression-free survival (PFS) and overall survival (OS) analyses are reported.

Methods: We have developed a method that can be used to estimate approximate transition rates from published PFS …

Trial Of Canakinumab, An Il-1Β Receptor Antagonist, In Patients With Inclusion Body Myositis., Michalis L Kosmidis, Dimitris Pikazis, Panayotis Vlachoyiannopoulos, Athanasios G Tzioufas, Marinos C. Dalakas

Department of Neurology Faculty Papers

Objective: To assess whether canakinumab, a monoclonal antibody against IL-1β approved for autoinflammatory diseases, is effective as target-specific therapy in patients with sporadic inclusion body myositis (sIBM).

Methods: Because in sIBM IL-1β colocalizes with amyloid precursor protein and upregulates amyloid aggregates enhancing degeneration, targeting IL-1β with canakinumab may arrest disease progression. On this basis, 5 ambulatory patients with sIBM participated in an institutional review board--approved open-labeled study with 150 mg canakinumab [4 bimonthly, then monthly subcutaneous injections] for a mean period of 15.8 months. Patients were assessed bimonthly with a manual dynamometer in 12 proximal and distal muscles and with …

Gangliosides: Treatment Avenues In Neurodegenerative Disease., Pierre J. Magistretti, Fred H. Geisler, Jay S. Schneider, P. Andy Li, Hubert Fiumelli, Simonetta Sipione

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Gangliosides are cell membrane components, most abundantly in the central nervous system (CNS) where they exert among others neuro-protective and -restorative functions. Clinical development of ganglioside replacement therapy for several neurodegenerative diseases was impeded by the BSE crisis in Europe during the 1990s. Nowadays, gangliosides are produced bovine-free and new pre-clinical and clinical data justify a reevaluation of their therapeutic potential in neurodegenerative diseases. Clinical experience is greatest with monosialo-tetrahexosyl-ganglioside (GM1) in the treatment of stroke. Fourteen randomized controlled trials (RCTs) in overall >2,000 patients revealed no difference in survival, but consistently superior neurological outcomes vs. placebo. GM1 was shown …

Commentary: The History Of Neurosurgery At Albany Medical College And Albany Medical Center Hospital, Albany, New York., Pouya Entezami, Michael R. Gooch, Adedamola Adepoju, Julie Pilitsis, Tyler J. Kenning, Alan Boulos, David L. Semenoff

Department of Neurosurgery Faculty Papers

The origins of the Department of Neurosurgery at Albany Medical College closely parallel the development of early America and the establishment of modern health care.The tales of Washington Irving, the works of the Hudson River School of painters, and summers in the Catskill Mountains or Adirondacks are the stories that color the history of Upstate New York (Figure1). As a social, industrial, and political hub of the American colonies, New England’s need for centers providing structured medicine led to the creation of Albany Medical College in1839, one of the earliest such institutions in the young nation.1 Rapid progress in nearly …

Initiating The Ketogenic Diet In Infants With Treatment Refractory Epilepsy While Maintaining A Breast Milk Diet, Jean-Baptist Lepichon, Lindsey M. Thompson, Megan Gustafson, Ahmed Abdelmoity

Manuscripts, Articles, Book Chapters and Other Papers

© 2019 The Authors

Purpose: The ketogenic diet has been found to be safe and effective in the treatment of drug resistant epilepsy in childhood. The age range of children undergoing this treatment has steadily been going down. There is strong evidence that it is a safe alternative in infants with drug resistant seizures. The American Academy of Pediatrics strongly supports continuing a breast milk diet until infants are at least six months of age. The purpose of this study is to evaluate the safety and efficacy of the ketogenic diet in infants while maintaining a breast milk diet.

Method: …

Biochemical And Clinical Response After Umbilical Cord Blood Transplant In A Boy With Early Childhood-Onset Beta-Mannosidosis., Troy C. Lund, Weston P. Miller, Julie B. Eisengart, Katrina Simmons, Laura Pollard, Deborah L. Renaud, David A. Wenger, Marc C. Patterson, Paul J. Orchard

Department of Neurology Faculty Papers

BACKGROUND: Deficiency in the enzyme β-mannosidase was described over three decades ago. Although rare in occurrence, the presentation of childhood-onset β-mannosidase deficiency consists of hypotonia in the newborn period followed by global development delay, behavior problems, and intellectual disability. No effective pharmacologic treatments have been available.

METHODS: We report 2-year outcomes following the first umbilical cord blood transplant in a 4-year-old boy with early childhood-onset disease.

RESULTS: We show restoration of leukocyte β-mannosidase activity which remained normal at 2 years posttransplant, and a simultaneous increase in plasma β-mannosidase activity and dramatic decrease in urine-free oligosaccharides were also observed. MRI of …

Guillain-Barre Syndrome Following A Snakebite: A Case Report And Review Of Literature, Sajid Hameed, Mubashar Memon, Sara Khan

Section of Neurology

Guillain-Barre syndrome (GBS) is preceded by a respiratory or gastrointestinal illness in more than 50% of the patients. A rare association of GBS with a preceding snakebite is reported in the literature in only four previous cases. We present a case report of a patient diagnosed with GBS following the bite of a yellow-bellied sea snake (Hydrophis platurus) and a brief review of the available literature.

Yield Of Muscle Biopsy In Patients With Findings Of Myopathy On Electrodiagnostic Testing, Sarah H. Siddiqui, Raheel Ahmed, Safia Awan, Ambreen Zain, Sara Khan

Section of Neurology

Background: The evaluation of neuromuscular diseases includes detailed clinical assessment, blood testing, electrodiagnostic studies (EDS), biopsy, and genetic tests. EDS alone cannot provide a specific diagnosis. Further testing in the form of genetic tests or muscle biopsy (MB) is required. Objective The objective of the study is to evaluate the yield of MB in patients with findings of myopathy on electrodiagnostic testing and assess the factors affecting an abnormal biopsy outcome.

Methods: Electromyography (EMG)/nerve conduction studies (NCS) performed for suspected myopathy over 5 years from 2011 to 2016, at the neurophysiology department of a tertiary care center in Pakistan, were …

Galcanezumab In Episodic Migraine: Subgroup Analyses Of Efficacy By High Versus Low Frequency Of Migraine Headaches In Phase 3 Studies (Evolve-1 & Evolve-2)., Stephen D. Silberstein, Virginia L. Stauffer, Katie A. Day, Sarah Lipsius, Maria-Carmen Wilson

Department of Neurology Faculty Papers

BACKGROUND: Patients with high-frequency episodic migraine (HFEM) have a greater disease burden than those with low-frequency episodic migraine (LFEM). Acute treatment overuse increases the risk of migraine chronification in patients with HFEM. Galcanezumab, a humanized monoclonal antibody binding calcitonin gene-related peptide (CGRP), is effective for migraine prevention with a favorable safety profile. Here, we investigate whether there are differences in galcanezumab efficacy in patients with LFEM or with HFEM.

METHODS: Data were pooled from two double-blind, placebo-controlled phase 3 trials; EVOLVE-1 and EVOLVE-2. Patients were 18-65 years old, experienced 4-14 monthly migraine headache days (MHDs) for ≥1 year prior, with …

Astragaloside Iv Protects Neurons From Microglia-Mediated Cell Damage Through Promoting Microglia Polarization., Jingwen Yu, Minfang Guo, Yanhua Li, Huiyu Zhang, Zhi Chai, Qing Wang, Yuqing Yan, Jiezhong Yu, Chunyun Liu, Guang-Xian Zhang, Ma Cungen

Department of Neurology Faculty Papers

Astragaloside IV (AST-IV) is a major active ingredient of astragalus, with a neuroprotective effect. The current study is aimed to investigate the impact of AST-IV on the M1/M2 microglial activation in response to lipopolysaccharide (LPS) stimulation, how AST-IV attenuated microglia-mediated neuronal damage, and the molecular mechanisms underlying AST-IV's protection of neurons against microglia-mediated neuronal damage. Our results showed that AST-IV partially protected microglia from death evoked by LPS and downregulated the release of pro-inflammatory (M1) mediators including interleukin (IL)-1β, IL-6, tumour necrosis factor α (TNF-α) and nitric oxide, as well as the expression of Toll-like receptors 4 (TLR4), MyD88, and …

A 24-Week Double-Blind Placebo-Controlled Study Of The Efficacy And Safety Of The Ampa Modulator S47445 In Patients With Mild To Moderate Alzheimer's Disease And Depressive Symptoms, Eileen M. Bulger, Sylvie Gouttefangeas, Sylvie Bretin, Stéphanie Galtier, Philippe Robert, Vjera Holthoff-Detto, Jeffrey Cummings, Maria Pueyo

School of Medicine Faculty Research

Introduction S47445 is a novel positive allosteric modulator of alpha-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid receptors that may emerge as a favorable candidate for the symptomatic treatment of cognitive and depressive disorders in patients suffering from Alzheimer's disease (AD) of mild to moderate severity and with depressive symptoms. Methods For this double-blind, placebo-controlled 24-week phase II trial, 520 outpatients aged between 55 and 85 years, with probable AD at mild to moderate stages (a Mini-Mental State Examination score of 24-15 inclusive) and exhibiting depressive symptoms (Cornell Scale for Depression in Dementia [CSDD] ≥ 8) were recruited in twelve countries and randomized to 3 doses …

Stat3 Genotypic Variant Rs744166 And Increased Tyrosine Phosphorylation Of Stat3 In Il-23 Responsive Innate Lymphoid Cells During Pathogenesis Of Crohn's Disease, Ying Tang, Sanda A. Tan, Atif Iqbal, Jian Li, Sarah C. Glover

Department of Neurology Faculty Papers

Crohn's disease (CD) results from dysregulated immune responses to gut microbiota in genetically susceptible individuals, affecting multiple areas of the gastrointestinal tract. Innate lymphoid cells (ILCs) are tissue-resident innate effector lymphocytes which play crucial roles in mucosal immune defense, tissue repair, and maintenance of homeostasis. The accumulation of IFN-γ-producing ILC1s and increased level of proinflammatory cytokines produced by ILCs has been observed in the inflamed terminal ileum of CD patients. To date, the precise mechanisms of ILC plasticity and gene regulatory pathways in ILCs remain unclear. Signal transducer and activator of transcription 3 (STAT3) regulates gene expression in …

Testosterone Levels In Men With Chronic Migraine, Lisa B. E. Shields, Tad Seifert, Brent J. Shelton, Brian M. Plato

Markey Cancer Center Faculty Publications

Chronic migraine is a frequent and debilitating condition affecting 14% of the general population. This prospective observational pilot study investigated whether men with chronic migraine have lower than expected total serum testosterone levels. We identified 14 men ages 26-51 at our Institution who fulfilled the ICHD-3b criteria for chronic migraine and obtained serum total testosterone levels. The mean total testosterone level in our 14 patients was 322 ng/dL (range: 120-542 ng/dL) which is in the lower 5% of the reference range for our laboratory (300-1080 ng/dL). Men with chronic migraine had lower total testosterone levels compared to published agematched normative …