1 Sensory Disturbances
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Patients and Experiences from the First Danish Flavour Clinic
DANISH MEDICAL JOURNAL Patients and experiences from the first Danish flavour clinic Alexander Fjaeldstad1, 2, 3, Jelena Stankovic2, Mine Onat2, Dovile Stankevice1 & Therese Ovesen1, 2 ABSTRACT duced sense of smell (hyposmia) [1, 2], making olfac INTRODUCTION: Chemosensory dysfunction is common. tory dysfunction a very common disorder. Apart from ORIGINAL ARTICLE Although patients complain of taste loss, the most common these quantitative olfactory disorders, olfactory dis 1) Flavour Clinic, Ear cause of a diminished taste experience is olfactory orders can have a qualitative nature where stimuli are Nose and Throat dysfunction. Department, Holstebro distorted (parosmia) or emerge without apparent stim Regional Hospital, METHODS: Since January 2017, patients with complaints ulation (phantosmia). Around 10% of patients with dis Denmark about smell and/or taste loss have been referred to the torted flavour perception have an actual taste disorder, 2) Flavour Institute, Flavour Clinic by ear, nose and throat (ENT) practitioners. Prior while only a few percent have isolated taste disorders. Department of Clinical to referral, CT, endoscopy of the nasal cavity and allergy Medicine, Aarhus These include loss of taste (ageusia), reduced sense of testing were required. Patients underwent full olfactory and University, Denmark taste (hypogeusia) or distorted sense of taste (parageu 3) Hedonia Research gustatory testing, complete ENT and neurological examination sia). Group, Department of and review of medicine and medical history. Patients also In all cases, the sensory loss can cause a wide range Psychiatry, University of completed different questionnaires such as the Mini Mental Oxford, United Kingdom of complications and consequences for patients. Status Examination, the Sino-Nasal Outcome Test and the Patients often complain of a reduced quality of life due Major Depression Inventory. -
Taste and Smell Disorders in Clinical Neurology
TASTE AND SMELL DISORDERS IN CLINICAL NEUROLOGY OUTLINE A. Anatomy and Physiology of the Taste and Smell System B. Quantifying Chemosensory Disturbances C. Common Neurological and Medical Disorders causing Primary Smell Impairment with Secondary Loss of Food Flavors a. Post Traumatic Anosmia b. Medications (prescribed & over the counter) c. Alcohol Abuse d. Neurodegenerative Disorders e. Multiple Sclerosis f. Migraine g. Chronic Medical Disorders (liver and kidney disease, thyroid deficiency, Diabetes). D. Common Neurological and Medical Disorders Causing a Primary Taste disorder with usually Normal Olfactory Function. a. Medications (prescribed and over the counter), b. Toxins (smoking and Radiation Treatments) c. Chronic medical Disorders ( Liver and Kidney Disease, Hypothyroidism, GERD, Diabetes,) d. Neurological Disorders( Bell’s Palsy, Stroke, MS,) e. Intubation during an emergency or for general anesthesia. E. Abnormal Smells and Tastes (Dysosmia and Dysgeusia): Diagnosis and Treatment F. Morbidity of Smell and Taste Impairment. G. Treatment of Smell and Taste Impairment (Education, Counseling ,Changes in Food Preparation) H. Role of Smell Testing in the Diagnosis of Neurodegenerative Disorders 1 BACKGROUND Disorders of taste and smell play a very important role in many neurological conditions such as; head trauma, facial and trigeminal nerve impairment, and many neurodegenerative disorders such as Alzheimer’s, Parkinson Disorders, Lewy Body Disease and Frontal Temporal Dementia. Impaired smell and taste impairs quality of life such as loss of food enjoyment, weight loss or weight gain, decreased appetite and safety concerns such as inability to smell smoke, gas, spoiled food and one’s body odor. Dysosmia and Dysgeusia are very unpleasant disorders that often accompany smell and taste impairments. -
The Relationship Among Pain, Sensory Loss, and Small Nerve Fibers in Diabetes
Pathophysiology/Complications ORIGINAL ARTICLE The Relationship Among Pain, Sensory Loss, and Small Nerve Fibers in Diabetes 1,2 LEA SORENSEN, RN, BHSC ing our own, have shown this not to be 1 LYNDA MOLYNEAUX, RN the case (9–11). However, in view of the 1,2 DENNIS K. YUE, MD, PHD, FRACP pivotal role played by small nerve fibers in the transmission of pain sensation, fur- ther studies are obviously of importance. OBJECTIVE — Many individuals with diabetes experience neuropathic pain, often without Direct examination of intraepidermal objective signs of large-fiber neuropathy. We examined intraepidermal nerve fibers (IENFs) to nerve fibers (IENF) using skin biopsy evaluate the role of small nerve fibers in the genesis of neuropathic pain. technique is a proven procedure to iden- tify small-fiber abnormalities. Several RESEARCH DESIGN AND METHODS — Twenty-five diabetic subjects with neuro- studies using this technique have shown pathic pain and 13 without were studied. The pain was present for at least 6 months for which the density of IENF to be reduced in id- no other cause could be found. Punch skin biopsies were obtained from the distal leg. IENFs were stained using antibody to protein gene product 9.5 and counted with confocal microscopy. iopathic and nondiabetic neuropathies Neuropathy was graded by vibration perception and cold detection thresholds and the Michigan (12–14). This technique has also shown Neuropathy Screening Instrument. that people with diabetes have reduced IENF and altered nerve morphology RESULTS — In the total cohort, IENF density was significantly lower in those with pain (14,15). However, to our knowledge, no compared with those without (3 [1–6] vs. -
Understanding Perceptions of Variation in Sensory Experience
What do people know about the senses? Understanding perceptions of variation in sensory experience Christine Cuskley*1 and Charalampos Saitis2 1Linguistics and Centre for Behaviour and Evolution, Newcastle University, Newcastle Upon Tyne, UK 2Centre for Digital Music, Queen Mary University London, London, UK *Corresponding author: [email protected] Abstract: Academic disciplines spanning cognitive science, art, and music have made strides in understanding how humans sense and experience the world. We now have a better scientific understanding of how human sensation and perception function both in the brain and in interaction than ever before. However, there is little research on how this high level scientific understanding is translated into knowledge for the public more widely. We present descriptive results from a simple survey and compare how public understanding and perception of sensory experience lines up with scientific understanding. Results show that even in a sample with fairly high educational attainment, many respondents were unaware of fairly common forms of sensory variation. In line with the well- documented under representation of sign languages within linguistics, respondents tended to under- estimate the number of sign languages in the world. We outline how our results represent gaps in public understanding of sensory variation, and argue that filling these gaps can form an important early intervention, acting as a basic foundation for improving acceptance, inclusivity, and accessibility for cognitively diverse populations. Introduction Our senses form our window into the world. Understanding human sensory experience, and how it supports uniquely human endeavours like music and language, is key to a better understanding of what it means to be human. -
Hirayama Disease
VIDEO CASE SOLUTION VIDEO CASE SOLUTION Hirayama Disease BY AZIZ SHAIBANI, MD, FACP, FAAN, FANA Last month’s case presented a 21-year-old shrimp peeler who developed weakness of his fingers five years earlier that pro- gressed to a point where he was unable to perform his job. CPK and 530 U/L and EMG revealed chronic diffuse denerva- tion of the arms and muscles with normal sensory and motor responses. Watch the exam at PracticalNeurology.com. Chronic unilateral or bilateral pure motor weakness of the hand and muscles in a young patient is not common. DIFFERENTIAL DIAGNOSIS • Cervical cord pathology such as Syringomyelia: dissociated sensory loss is typically present. • Brachial plexus pathology: sensory findings are usually present. • Motor neurons disease: ALS, spinal muscular atrophy (SMA). – Cervical Spines are usually investigated before neuromuscular referrals are made. – The lack of pain, radicular or sensory symptoms and normal sensory SNAPS and cervical MRIs ruled out most of the mentioned possibilities except: • distal myopathy (usually not unilaterial) and spinal muscular atrophy. • EMG/NCS demonstration of chronic distal denervation with normal sensory responses and no demyelinating features lim- ited the diagnosis to MND. • Segmental denervation pattern further narrows the diagnosis to Hirayama disease (HD). HIRAYAMA DISEASE • HD is a sporadic and focal form of SMA that affects predominantly males between the ages of 15 and 25 years. • Weakness and atrophy usually starts unilaterally in C8-T1 muscles of the hands and forearm (typically in the dominant hand). – In roughly one-third of cases, the other hand is affected and weakness may spread to the proximal muscles. -
Accessibility Guide for Sensory Loss
A simple touch leads to endless possibilities Accessibility Guidelines for Sensory Loss 2020 3rd Edition Table of Contents Introduction .............................................................................................................................. 1 DeafBlind Ontario Services .................................................................................................. 2 About the Accessibility Guidelines for Sensory Loss ....................................................... 3 Acknowledgements .............................................................................................................. 3 Copyright ............................................................................................................................. 3 Accessibility Guidelines ....................................................................................................... 3 Quick Design Tips ................................................................................................................ 4 DIY Orientation and Accessibility Enhancements ................................................................ 4 Deafblindness ....................................................................................................................... 5 What is Deafblindness? ....................................................................................................... 5 Deafblindness and Communication ..................................................................................... 5 Types of Deafblindness ...................................................................................................... -
Olfactory Dysfunction in Neurodegenerative Diseases
Eur J Gen Med 2004; 1(3): 1-11 REVIEW ARTICLE OLFACTORY DYSFUNCTION IN NEURODEGENERATIVE DISEASES M. Hakan Özdener, Nancy E. Rawson Monell Chemical Senses Center, University City Science Center Interest in olfactory dysfunction has increased tremendously in the past decade, in part due to observations that chemosensory impairment is an early symptom of many neurodegenerative diseases. Several features of the olfactory system make it particularly relevant to understanding neurodegeneration/regeneration. First, while being vulnerable to environmental and infectious exposure, the olfactory system has the unique property of ongoing replacement of the olfactory sensory neurons under physiological conditions and following injury. Second, the receptor neurons residing in the periphery are developmentally related to the central nervous system, yet are accessible relatively noninvasively via biopsy from living subjects. Third, olfactory performance can easily be tested in a variety of ways that provide insight into the function of brain areas involved in detection, identification and memory. In addition to providing insights into the etiology or diagnosis of disease, a better understanding of olfactory neurobiology is needed to develop ways to treat olfactory dysfunction, which affects both quality of life and personal safety. At least 3,000,000 Americans suffer from chemosensory disorders and that is likely to grow as the aging segment of the population increases. Olfactory impairment represents a danger to the individuals resulting from inability to detect hazards as natural gas and spoiled food and threatens quality of life through the loss of enjoyment of foods and fragrances. The neurological systems responsible for olfactory function represent perhaps the most diverse, complex and adaptable components of the nervous system. -
Table of Contents
TABLE OF CONTENTS 1. INTRODUCTION . Faculty . Program 2. GUIDELINES . Fellowship Appointments . Fellow Selection 3. REQUIREMENTS . UAB Child Neurology Training Requirements . Call Requirements . Moonlighting Policy . ACGME Duty Hours . Policy on Fatigue . ABPN Training Requirements 4. GOALS AND OBJECTIVES . Overall Program Goals and Objectives . Adult Year PG3-PG4 . Inpatient PG4 . Inpatient PG5 . Outpatient . Neuromuscular . Epilepsy . Neuropathology . Psychiatry . Conferences . Required Research Survey Course 5. CURRICULUM IN CHILD NEUROLOGY . Purpose . Structure . Content 6. SALARY AND BENEFITS 7. CRITERIA FOR ADVANCEMENT . Educational Expectations . Disciplinary Procedures . Grievance Procedure 8. EVALUATIONS . Supervision of Fellows Policy . Fellow Evaluations . Attending Evaluations . Program Evaluations . Peer and Self Evaluations . Healthcare Professional Evaluations INTRODUCTION: Child Neurology is a specialty of both pediatrics and neurology which focuses on the nervous system of the pediatric population. The practice of child neurology requires competence and training in both pediatrics and neurology in order to understand and treat disorders of the pediatric nervous system. This manual outlines the goals and expectations as well as logistics of the Child Neurology training program at The University of Alabama at Birmingham. A. Clinical competence requires: 1. A solid fund of basic and clinical knowledge and the ability to maintain it at current levels for a lifetime of continuous education. 2. The ability to perform an adequate history and physical examination. 3. The ability to appropriately order and interpret diagnostic tests. 4. Adequate technical skills to carry out selected diagnostic procedures. 5. Clinical judgment to critically apply the above data to individual patients. 6. Attitudes conducive to the practice of neurology, including appropriate interpersonal interactions with patients and families, professional colleagues, supervisory faculty and all paramedical personnel. -
EM Guidemap - Myopathy and Myoglobulinuria
myopathy EM guidemap - Myopathy and myoglobulinuria Click on any of the headings or subheadings to rapidly navigate to the relevant section of the guidemap Introduction General principles ● endocrine myopathy ● toxic myopathy ● periodic paralyses ● myoglobinuria Introduction - this short guidemap supplements the neuromuscular weakness guidemap and offers the reader supplementary information on myopathies, and a short section on myoglobulinuria - this guidemap only consists of a few brief checklists of "causes of the different types of myopathy" that an emergency physician may encounter in clinical practice when dealing with a patient with acute/subacute muscular weakness General principles - a myopathy is suggested when generalized muscle weakness involves large proximal muscle groups, especially around the shoulder and proximal girdle, and when the diffuse muscle weakness is associated with normal tendon reflexes and no sensory findings - a simple classification of myopathy:- Hereditary ● muscular dystrophies ● congenital myopathies http://www.homestead.com/emguidemaps/files/myopathy.html (1 of 13)8/20/2004 5:14:27 PM myopathy ● myotonias ● channelopathies (periodic paralysis syndromes) ● metabolic myopathies ● mitochondrial myopathies Acquired ● inflammatory myopathy ● endocrine myopathies ● drug-induced/toxic myopathies ● myopathy associated with systemic illness - a myopathy can present with fixed weakness (muscular dystrophy, inflammatory myopathy) or episodic weakness (periodic paralysis due to a channelopathy, metabolic myopathy -
Paraneoplastic Neurological and Muscular Syndromes
Paraneoplastic neurological and muscular syndromes Short compendium Version 4.5, April 2016 By Finn E. Somnier, M.D., D.Sc. (Med.), copyright ® Department of Autoimmunology and Biomarkers, Statens Serum Institut, Copenhagen, Denmark 30/01/2016, Copyright, Finn E. Somnier, MD., D.S. (Med.) Table of contents PARANEOPLASTIC NEUROLOGICAL SYNDROMES .................................................... 4 DEFINITION, SPECIAL FEATURES, IMMUNE MECHANISMS ................................................................ 4 SHORT INTRODUCTION TO THE IMMUNE SYSTEM .................................................. 7 DIAGNOSTIC STRATEGY ..................................................................................................... 12 THERAPEUTIC CONSIDERATIONS .................................................................................. 18 SYNDROMES OF THE CENTRAL NERVOUS SYSTEM ................................................ 22 MORVAN’S FIBRILLARY CHOREA ................................................................................................ 22 PARANEOPLASTIC CEREBELLAR DEGENERATION (PCD) ...................................................... 24 Anti-Hu syndrome .................................................................................................................. 25 Anti-Yo syndrome ................................................................................................................... 26 Anti-CV2 / CRMP5 syndrome ............................................................................................ -
Hereditary Sensory and Autonomic Neuropathies: Adding More to the Classification
Current Neurology and Neuroscience Reports (2019) 19: 52 https://doi.org/10.1007/s11910-019-0974-3 AUTONOMIC DYSFUNCTION (L.H. WEIMER, SECTION EDITOR) Hereditary Sensory and Autonomic Neuropathies: Adding More to the Classification Coreen Schwartzlow1 & Mohamed Kazamel1 Published online: 20 June 2019 # Springer Science+Business Media, LLC, part of Springer Nature 2019 Abstract Purpose of Review Hereditary sensory and autonomic neuropathies (HSANs) are a clinically heterogeneous group of inherited neuropathies featuring prominent sensory and autonomic involvement. Classification of HSAN is based on mode of inheritance, genetic mutation, and phenotype. In this review, we discuss the recent additions to this classification and the important updates on management with a special focus on the recently investigated disease-modifying agents. Recent Findings In this past decade, three more HSAN types were added to the classification creating even more diversity in the genotype–phenotype. Clinical trials are underway for disease-modifying and symptomatic therapeutics, targeting mainly HSAN type III. Summary Obtaining genetic testing leads to accurate diagnosis and guides focused management in the setting of such a diverse and continuously growing phenotype. It also increases the wealth of knowledge on HSAN pathophysiologies which paves the way toward development of targeted genetic treatments in the era of precision medicine. Keywords Congenital insensitivity to pain . Familial dysautonomia . Hereditary sensory and autonomic neuropathies . IKBKAP/ ELP1 . L-Serine Introduction kinships [2]. This prompted further investigations into the underlying etiologies of the variable phenotypes, including The hereditary sensory and autonomic neuropathies (HSANs) genetic causes, guiding a classification system that initially are a group of heterogeneous genetic disorders that predomi- recognized these neuropathies as hereditary sensory neuropa- nantly feature slowly progressive loss of multimodal sensation thies (HSNs). -
Sensory Functions in Chronic Neuralgia
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.42.5.422 on 1 May 1979. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry, 1979, 42, 422435 Sensory functions in chronic neuralgia ULF LINDBLOM AND RONALD T. VERRILLO From the Department of Neurology, Huddinge University Hospital, Huddinge, Sweden SUMMARY Eleven patients with sustained neuralgia, in most cases after traumatic nerve lesion, were subjected to quantitative sensory testing with thermal and non-noxious mechanical stimuli. Measurements were made in the pain area and at a homologous site on the contralateral normal side. All patients were hypoaesthetic with raised thresholds for warm and cold or touch, or both. Thermal pain thresholds were also raised in some patients but lowered in others indicat- ing hypersensitivity of the nociceptor system or dysaesthesia for thermal input. In six patients single mechanical stimuli produced a painful response above the touch detection threshold. Reaction time measurements indicated that this painful response was the result of a central dysfunction rather than of peripheral sensitisation. The response to suprathreshold mechanical pulses was measured by magnitude estimation as a function of stimulus amplitude. The results were fitted by power functions, as in normal skin, but with steeper slopes on the abnormal side. Suprathreshold hyperaesthesia (recruitment) may exist in the presence of normal threshold functioning. Protected by copyright. The symptom of neuralgia which is the centre of be observed and measured on patients and extra- concern for both the patient and the physician is, polate from experimental physiological data. of course, the pain itself. Of secondary importance Among conceivable peripheral mechanisms, which is the hyperalgesia and other sensory abnormalities have been reviewed recently by Wall and Devor which often occur in the painful area.