Mimickers of Cervical Spondylotic Myelopathy
Total Page:16
File Type:pdf, Size:1020Kb
Load more
Recommended publications
-
Clinically Undetected Motor Neuron Disease in Pathologically Proven Frontotemporal Lobar Degeneration with Motor Neuron Disease
ORIGINAL CONTRIBUTION Clinically Undetected Motor Neuron Disease in Pathologically Proven Frontotemporal Lobar Degeneration With Motor Neuron Disease Keith A. Josephs, MST, MD; Joseph E. Parisi, MD; David S. Knopman, MD; Bradley F. Boeve, MD; Ronald C. Petersen, MD, PhD; Dennis W. Dickson, MD Background: Frontotemporal lobar degeneration with evidence of motor neuron disease. Semiquantitative motor neuron disease (FTLD-MND) is a pathological analysis of motor and extramotor pathological findings entity characterized by motor neuron degeneration and revealed a spectrum of pathological changes underlying frontotemporal lobar degeneration. The ability to detect FTLD-MND. Hippocampal sclerosis, predominantly of the clinical signs of dementia and motor neuron disease the subiculum, was a significantly more frequent occur- in pathologically confirmed FTLD-MND has not been rence in the cases without clinical evidence of motor assessed. neuron disease (PϽ.01). In addition, neuronal loss, gliosis, and corticospinal tract degeneration were less Objectives: To determine if all cases of pathologically severe in the other 3 cases without clinical evidence of confirmed FTLD-MND have clinical evidence of fronto- motor neuron disease. temporal dementia and motor neuron disease, and to de- termine the possible reasons for misdiagnosis. Conclusions: Clinical diagnostic sensitivity for the el- ements of FTLD-MND is modest and may be affected by Method: Review of historical records and semiquantita- the fact that FTLD-MND represents a spectrum of patho- tive analysis of the motor and extramotor pathological find- logical findings, rather than a single homogeneous en- ings of all cases of pathologically confirmed FTLD-MND. tity. Detection of signs of clinical motor neuron disease is also difficult when motor neuron degeneration is mild Results: From a total of 17 cases of pathologically con- and in patients with hippocampal sclerosis. -
Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia
brain sciences Review Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia Timothy Fullam and Jeffrey Statland * Department of Neurology, University of Kansas Medical Center, Kansas, KS 66160, USA; [email protected] * Correspondence: [email protected] Abstract: Following the exclusion of potentially reversible causes, the differential for those patients presenting with a predominant upper motor neuron syndrome includes primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), or upper motor neuron dominant ALS (UMNdALS). Differentiation of these disorders in the early phases of disease remains challenging. While no single clinical or diagnostic tests is specific, there are several developing biomarkers and neuroimaging technologies which may help distinguish PLS from HSP and UMNdALS. Recent consensus diagnostic criteria and use of evolving technologies will allow more precise delineation of PLS from other upper motor neuron disorders and aid in the targeting of potentially disease-modifying therapeutics. Keywords: primary lateral sclerosis; amyotrophic lateral sclerosis; hereditary spastic paraplegia Citation: Fullam, T.; Statland, J. Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper 1. Introduction Motor Neuron Dominant Jean-Martin Charcot (1825–1893) and Wilhelm Erb (1840–1921) are credited with first Amyotrophic Lateral Sclerosis, and describing a distinct clinical syndrome of upper motor neuron (UMN) tract degeneration in Hereditary Spastic Paraplegia. Brain isolation with symptoms including spasticity, hyperreflexia, and mild weakness [1,2]. Many Sci. 2021, 11, 611. https:// of the earliest described cases included cases of hereditary spastic paraplegia, amyotrophic doi.org/10.3390/brainsci11050611 lateral sclerosis, and underrecognized structural, infectious, or inflammatory etiologies for upper motor neuron dysfunction which have since become routinely diagnosed with the Academic Editors: P. -
ALS and Other Motor Neuron Diseases Can Represent Diagnostic Challenges
Review Article Address correspondence to Dr Ezgi Tiryaki, Hennepin ALS and Other Motor County Medical Center, Department of Neurology, 701 Park Avenue P5-200, Neuron Diseases Minneapolis, MN 55415, [email protected]. Ezgi Tiryaki, MD; Holli A. Horak, MD, FAAN Relationship Disclosure: Dr Tiryaki’s institution receives support from The ALS Association. Dr Horak’s ABSTRACT institution receives a grant from the Centers for Disease Purpose of Review: This review describes the most common motor neuron disease, Control and Prevention. ALS. It discusses the diagnosis and evaluation of ALS and the current understanding of its Unlabeled Use of pathophysiology, including new genetic underpinnings of the disease. This article also Products/Investigational covers other motor neuron diseases, reviews how to distinguish them from ALS, and Use Disclosure: Drs Tiryaki and Horak discuss discusses their pathophysiology. the unlabeled use of various Recent Findings: In this article, the spectrum of cognitive involvement in ALS, new concepts drugs for the symptomatic about protein synthesis pathology in the etiology of ALS, and new genetic associations will be management of ALS. * 2014, American Academy covered. This concept has changed over the past 3 to 4 years with the discovery of new of Neurology. genes and genetic processes that may trigger the disease. As of 2014, two-thirds of familial ALS and 10% of sporadic ALS can be explained by genetics. TAR DNA binding protein 43 kDa (TDP-43), for instance, has been shown to cause frontotemporal dementia as well as some cases of familial ALS, and is associated with frontotemporal dysfunction in ALS. Summary: The anterior horn cells control all voluntary movement: motor activity, res- piratory, speech, and swallowing functions are dependent upon signals from the anterior horn cells. -
Syringomyelia in Cervical Spondylosis: a Rare Sequel H
THIEME Editorial 1 Editorial Syringomyelia in Cervical Spondylosis: A Rare Sequel H. S. Bhatoe1 1 Department of Neurosciences, Max Super Specialty Hospital, Patparganj, New Delhi, India Indian J Neurosurg 2016;5:1–2. Neurological involvement in cervical spondylosis usually the buckled hypertrophic ligament flavum compresses the implies radiculopathy or myelopathy. Cervical spondylotic cord. Ischemia due to compromise of microcirculation and myelopathy is the commonest cause of myelopathy in the venous congestion, leading to focal demyelination.3 geriatric age group,1 and often an accompaniment in adult Syringomyelia is an extremely rare sequel of chronic cervical patients manifesting central cord syndrome and spinal cord cord compression due to spondylotic process, and manifests as injury without radiographic abnormality. Myelopathy is the accelerated myelopathy (►Fig. 1). Pathogenesis of result of three factors that often overlap: mechanical factors, syringomyelia is uncertain. Al-Mefty et al4 postulated dynamic-repeated microtrauma, and ischemia of spinal cord occurrence of myelomalacia due to chronic compression of microcirculation.2 Age-related mechanical changes include the cord, followed by phagocytosis, leading to a formation of hypertrophy of the ligamentum flavum, formation of the cavity that extends further. However, Kimura et al5 osteophytic bars, degenerative disc prolapse, all of them disagreed with this hypothesis, and postulated that following contributing to a narrowing of the spinal canal. Degenerative compression of the cord, there is slosh effect cranially and kyphosis and subluxation often aggravates the existing caudally, leading to an extension of the syrinx. It is thus likely compressiononthespinalcord.Flexion–extension that focal cord cavitation due to compression and ischemia movements of the spinal cord places additional, dynamic occurs due to periventricular fluid egress into the cord, the stretch on the cord that is compressed. -
Degenerative Cervical Myelopathy: Clinical Presentation, Assessment, and Natural History
Journal of Clinical Medicine Review Degenerative Cervical Myelopathy: Clinical Presentation, Assessment, and Natural History Melissa Lannon and Edward Kachur * Division of Neurosurgery, McMaster University, Hamilton, ON L8S 4L8, Canada; [email protected] * Correspondence: [email protected] Abstract: Degenerative cervical myelopathy (DCM) is a leading cause of spinal cord injury and a major contributor to morbidity resulting from narrowing of the spinal canal due to osteoarthritic changes. This narrowing produces chronic spinal cord compression and neurologic disability with a variety of symptoms ranging from mild numbness in the upper extremities to quadriparesis and incontinence. Clinicians from all specialties should be familiar with the early signs and symptoms of this prevalent condition to prevent gradual neurologic compromise through surgical consultation, where appropriate. The purpose of this review is to familiarize medical practitioners with the pathophysiology, common presentations, diagnosis, and management (conservative and surgical) for DCM to develop informed discussions with patients and recognize those in need of early surgical referral to prevent severe neurologic deterioration. Keywords: degenerative cervical myelopathy; cervical spondylotic myelopathy; cervical decompres- sion Citation: Lannon, M.; Kachur, E. Degenerative Cervical Myelopathy: Clinical Presentation, Assessment, 1. Introduction and Natural History. J. Clin. Med. Degenerative cervical myelopathy (DCM) is now the leading cause of spinal cord in- 2021, 10, 3626. https://doi.org/ jury [1,2], resulting in major disability and reduced quality of life. While precise prevalence 10.3390/jcm10163626 is not well described, a 2017 Canadian study estimated a prevalence of 1120 per million [3]. DCM results from narrowing of the spinal canal due to osteoarthritic changes. This Academic Editors: Allan R. -
A Histopathological and Immunohistochemical Study of Acute and Chronic Human Compressive Myelopathy
Cellular Pathology and Apoptosis in Experimental and Human Acute and Chronic Compressive Myelopathy ROWENA ELIZABETH ANNE NEWCOMBE M.B.B.S. B.Med Sci. (Hons.) Discipline of Pathology, School of Medical Sciences University of Adelaide June 2010 A thesis submitted in partial fulfilment of the requirements for the degree of Doctor of Philosophy CHAPTER 1 INTRODUCTION 1 The term “compressive myelopathy” describes a spectrum of spinal cord injury secondary to compressive forces of varying magnitude and duration. The compressive forces may act over a short period of time, continuously, intermittently or in varied combination and depending on their magnitude may produce a spectrum varying from mild to severe injury. In humans, spinal cord compression may be due to various causes including sudden fracture/dislocation and subluxation of the vertebral column, chronic spondylosis, disc herniation and various neoplasms involving the vertebral column and spinal canal. Neoplasms may impinge on the spinal cord and arise from extramedullary or intramedullary sites. Intramedullary expansion producing a type of internal compression can be due to masses created by neoplasms or fluid such as the cystic cavitation seen in syringomyelia. Acute compression involves an immediate compression of the spinal cord from lesions such as direct trauma. Chronic compression may develop over weeks to months or years from conditions such as cervical spondylosis which may involve osteophytosis or hypertrophy of the adjacent ligamentum flavum. Compressive myelopathies include the pathological changes from direct mechanical compression at one or multiple levels and changes in the cord extending multiple segments above and below the site of compression. Evidence over the past decade suggests that apoptotic cell death in neurons and glia, in particular of oligodendrocytes, may play an important role in the pathophysiology and functional outcome of human chronic compressive myelopathy. -
Myelopathy—Paresis and Paralysis in Cats
Myelopathy—Paresis and Paralysis in Cats (Disorder of the Spinal Cord Leading to Weakness and Paralysis in Cats) Basics OVERVIEW • “Myelopathy”—any disorder or disease affecting the spinal cord; a myelopathy can cause weakness or partial paralysis (known as “paresis”) or complete loss of voluntary movements (known as “paralysis”) • Paresis or paralysis may affect all four limbs (known as “tetraparesis” or “tetraplegia,” respectively), may affect only the rear legs (known as “paraparesis” or “paraplegia,” respectively), the front and rear leg on the same side (known as “hemiparesis” or “hemiplegia,” respectively) or only one limb (known as “monoparesis” or “monoplegia,” respectively) • Paresis and paralysis also can be caused by disorders of the nerves and/or muscles to the legs (known as “peripheral neuromuscular disorders”) • The spine is composed of multiple bones with disks (intervertebral disks) located in between adjacent bones (vertebrae); the disks act as shock absorbers and allow movement of the spine; the vertebrae are named according to their location—cervical vertebrae are located in the neck and are numbered as cervical vertebrae one through seven or C1–C7; thoracic vertebrae are located from the area of the shoulders to the end of the ribs and are numbered as thoracic vertebrae one through thirteen or T1–T13; lumbar vertebrae start at the end of the ribs and continue to the pelvis and are numbered as lumbar vertebrae one through seven or L1–L7; the remaining vertebrae are the sacral and coccygeal (tail) vertebrae • The brain -
Motor Neuron Disease and the Elderly
Neurology 61 Motor neuron disease and the elderly Motor neuron disease is a devastating condition characterised by degeneration of motor nerves. Many of the presenting symptoms, such as fatigue, muscle weakness and difficulty in swallowing have a broad differential diagnoses in the elderly population. Dr Sheba Azam and Professor PN Leigh explain how ensuring quality of life for patients requires preventing unnecessary delay in diagnosis and early referral to an appropriate multidisciplinary team. myotrophic lateral sclerosis (ALS) also cognitive changes. Thus, misdiagnosis as well as known as motor neuron disease (MND) under-investigation has been suggested as possible (the terms are used interchangeably), was causes of an apparent decrease in the incidence of A 4 fi rst described in 1869 by the French neurologist MND in later life . Jean-Martin Charcot1. It is a progressive, fatal neurological disease characterised by degeneration of motor nerve cells in the motor cortex, Prognostic factors corticospinal tract and the spinal cord anterior horn Although the average survival in MND is around cells. The degeneration of motor nerve cells results 36 months, some patients live for 10 years or more. in progressive muscle wasting leading to signifi cant Certain phenotypic variants appear to determine disability and ultimately death. Death usually survival rates. Using information held in a tertiary results from respiratory failure due to weakness of referral MND database, a group of researchers the respiratory muscles. analysed data on onset of disease, site of onset and duration of survival5. The authors concluded that typical MND with bulbar onset, onset later in life Incidence and prevalence or in the defi nite category of El Escorial (where The worldwide incidence of MND is approximately the World Federation of Neurologist meet to a professor of clinical two per 100,000 and the prevalence is four to seven decide diagnostic criteria) at presentation, per 100,000. -
Upper Extremity Function in Persons with Tetraplegia: Relationships
Neurorehabilitation and Research Articles Neural Repair Volume 23 Number 5 June 2009 413-421 © 2009 The Author(s) 10.1177/1545968308331143 Upper Extremity Function in Persons with http://nnr.sagepub.com Tetraplegia: Relationships Between Strength, Capacity, and the Spinal Cord Independence Measure Claudia Rudhe, OT, MSc, and Hubertus J. A. van Hedel, PT, PhD Objective. To quantify the relationship between the Spinal Cord Independence Measure III (SCIM III), arm and hand muscle strength, and hand function tests in persons with tetraplegia. Methods. A total of 29 individuals with tetraplegia (motor level between cervical 4 and thoracic 1; sensory-motor complete and incomplete) participated. The total score, category scores, and separate items of the SCIM III were compared to the upper extremity motor score (UEMS), an extended manual muscle test (MMT) for 11 upper extremity muscles, and 6 functional capacity tests of the hand. Spearman’s correlation coefficients (rs) and regression analyses were performed. Results. The SCIM III sum score correlated well with the sum scores of the 3 tests (rs ≥ .76). The SCIM III self-care category correlated better with the tests (rs ≥ .80) compared to the other categories (rs ≤ .72). The SCIM III self-care item “grooming” highly correlated with muscle strength and hand capacity items (rs ≥ .80). A combination of hand muscle tests and the key grasping task explained over 90% of the variability in the self-care category scores. Conclusions. The SCIM III self-care category reflects upper extremity performance as it contains especially useful and valid items that relate to upper extremity function and capacity tests. -
Exacerbation of Motor Neuron Disease by Chronic Stimulation of Innate Immunity in a Mouse Model of Amyotrophic Lateral Sclerosis
1340 • The Journal of Neuroscience, February 11, 2004 • 24(6):1340–1349 Neurobiology of Disease Exacerbation of Motor Neuron Disease by Chronic Stimulation of Innate Immunity in a Mouse Model of Amyotrophic Lateral Sclerosis Minh Dang Nguyen,1 Thierry D’Aigle,2 Genevie`ve Gowing,1,2 Jean-Pierre Julien,1,2 and Serge Rivest2 1McGill University Health Center, Centre for Research in Neurosciences, McGill University, The Montreal General Hospital Research Institute, Montre´al, Que´bec H3G 1A4, Canada, and 2Laboratory of Molecular Endocrinology, Laval University Medical Center Research Center and Department of Anatomy and Physiology, Laval University, Sainte-Foy, Que´bec G1V 4G2, Canada Innate immunity is a specific and organized immunological program engaged by peripheral organs and the CNS to maintain homeostasis after stress and injury. In neurodegenerative disorders, its putative deregulation, featured by inflammation and activation of glial cells resulting from inherited mutations or viral/bacterial infections, likely contributes to neuronal death. However, it remains unclear to what extent environmental factors and innate immunity cooperate to modulate the interactions between the neuronal and non-neuronal elements in the perturbed CNS. In the present study, we addressed the effects of acute and chronic administration of lipopolysaccharide (LPS), a Gram-negative bacterial wall component, in a genetic model of neurodegeneration. Transgenic mice expressing a mutant form of the superoxide dismutase 1 (SOD1 G37R) linked to familial amyotrophic lateral sclerosis were challenged intraperitoneally with a single nontoxic or repeated injections of LPS (1 mg/kg). At different ages, SOD1 G37R mice responded normally to acute endotoxemia. Remark- ably, only a chronic challenge with LPS in presymptomatic 6-month-old SOD1 G37R mice exacerbated disease progression by 3 weeks and motor axon degeneration. -
Cerebellar Disease in the Dog and Cat
CEREBELLAR DISEASE IN THE DOG AND CAT: A LITERATURE REVIEW AND CLINICAL CASE STUDY (1996-1998) b y Diane Dali-An Lu BVetMed A thesis submitted for the degree of Master of Veterinary Medicine (M.V.M.) In the Faculty of Veterinary Medicine University of Glasgow Department of Veterinary Clinical Studies Division of Small Animal Clinical Studies University of Glasgow Veterinary School A p ril 1 9 9 9 © Diane Dali-An Lu 1999 ProQuest Number: 13815577 All rights reserved INFORMATION TO ALL USERS The quality of this reproduction is dependent upon the quality of the copy submitted. In the unlikely event that the author did not send a com plete manuscript and there are missing pages, these will be noted. Also, if material had to be removed, a note will indicate the deletion. uest ProQuest 13815577 Published by ProQuest LLC(2018). Copyright of the Dissertation is held by the Author. All rights reserved. This work is protected against unauthorized copying under Title 17, United States C ode Microform Edition © ProQuest LLC. ProQuest LLC. 789 East Eisenhower Parkway P.O. Box 1346 Ann Arbor, Ml 48106- 1346 GLASGOW UNIVERSITY lib ra ry ll5X C C ^ Summary SUMMARY________________________________ The aim of this thesis is to detail the history, clinical findings, ancillary investigations and, in some cases, pathological findings in 25 cases of cerebellar disease in dogs and cats which were presented to Glasgow University Veterinary School and Hospital during the period October 1996 to June 1998. Clinical findings were usually characteristic, although the signs could range from mild tremor and ataxia to severe generalised ataxia causing frequent falling over and difficulty in locomotion. -
Motor Neuron Disease Motor Neuron Disease
Motor Neuron Disease Motor Neuron Disease • Incidence: 2-4 per 100 000 • Onset: usually 50-70 years • Pathology: – Degenerative condition – anterior horn cells and upper motor neurons in spinal cord, resulting in mixed upper and lower motor neuron signs • Cause unknown – 10% familial (SOD-1 mutation) – ? Related to athleticism Presentation • Several variations in onset, but progress to the same endpoint • Motor nerves only affected • May be just UMN or just LMN at onset, but other features will appear over time • Main patterns: – Amyotrophic lateral sclerosis – Bulbar presentaion – Primary lateral sclerosis (UMN onset) – Progressive muscular atrophy (LMN onset) Questions Wasting Classification • Amyotrophic Lateral Sclerosis • Progressive Bulbar Palsy • Progressive Muscular Atrophy • Primary Lateral Sclerosis • Multifocal Motor Neuropathy • Spinal Muscular Atrophy • Kennedy’s Disease • Monomelic Amyotrophy • Brachial Amyotrophic Diplegia El Escorial Criteria for Diagnosis Tongue fasiculations Amyotrophic lateral sclerosis • ‘Typical’ presentation (60%+) • Usually one limb initially – Foot drop – Clumsy weak hand – May complain of cramps • Gradual progression over months • May be some wasting at presentation • Usually fasiculations (often more widespread) • Brisk reflexes, extensor plantars • No sensory signs; MAY occasionally be mild symptoms • Relentless progression, noticable over weeks/ months Bulbar MND • Approximately 30% of cases • Onset with dysarthria, dysphagia • Bulbar and pseudobulbar symptoms • On examination – Dysarthria –