Essential Elements for Assessment of Persons with Severe Neurological Impairments for Computer Access Using Assistive Technology Devices: a Delphi Study
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Detrusor Sphincter Dyssynergia: a Review of Physiology, Diagnosis, and Treatment Strategies
Review Article Detrusor sphincter dyssynergia: a review of physiology, diagnosis, and treatment strategies John T. Stoffel Department of Urology, University of Michigan, Ann Arbor, MI, USA Correspondence to: John T. Stoffel, MD. Department of Urology, University of Michigan, Ann Arbor, MI, USA. Email: [email protected]. Abstract: Detrusor sphincter dyssynergia (DSD) is the urodynamic description of bladder outlet obstruction from detrusor muscle contraction with concomitant involuntary urethral sphincter activation. DSD is associated with neurologic conditions such as spinal cord injury, multiple sclerosis, and spina bifida and some of these neurogenic bladder patients with DSD may be at risk for autonomic dysreflexia, recurrent urinary tract infections, or upper tract compromise if the condition is not followed and treated appropriately. It is diagnosed most commonly during the voiding phase of urodynamic studies using EMG recordings and voiding cystourethrograms, although urethral pressure monitoring could also potentially be used. DSD can be sub-classified as either continuous or intermittent, although adoption of this terminology is not widespread. There are few validated oral pharmacologic treatment options for this condition but transurethral botulinum toxin injection have shown temporary efficacy in reducing bladder outlet obstruction. Urinary sphincterotomy has also demonstrated reproducible long term benefits in several studies, but the morbidity associated with this procedure can be high. Keywords: Detrusor sphincter dyssynergia (DSD); neurogenic bladder; urodynamics; external urinary sphincter (EUS) Submitted Nov 30, 2015. Accepted for publication Jan 05, 2016. doi: 10.3978/j.issn.2223-4683.2016.01.08 View this article at: http://dx.doi.org/10.3978/j.issn.2223-4683.2016.01.08 Introduction Physiology The human bladder has two functions—to store and During storage of urine, afferent nerves carry information empty urine. -
Acute Cerebellar Ataxia Associated with Intermittent ECG Pattern Similar to Wellens Syndrome and Transient Prominent QRS Anterior Forces
Acute cerebellar ataxia associated with intermittent ECG pattern similar to Wellens syndrome and transient prominent QRS anterior forces Andrés Ricardo Pérez-Riera, MD, PhD. Post-Graduates Advisor at Design of Studies and Scientific Writing Laboratory in the ABC Faculty of Medicine - ABC Foundation - Santo André – São Paulo – Brazil Raimundo Barbosa-Barros, MD. Specialist in Cardiology by the Brazilian Society of Cardiology (SBC) Specialist in Intensive Care by the Sociedade Brasileira de Terapia Intensiva Chief of the Coronary Center of the Hospital de Messejana Dr. Carlos Alberto Studart Gomes. Fortaleza - Brazil Adrian Baranchuk, MD FACC FRCPC Associate Professor of Medicine and Physiology - Cardiac Electrophysiology and Pacing - Director, EP Training Program - Kingston General Hospital - FAPC 3, 76 Stuart Street K7L 2V7, Kingston ON Queen's University - Canada Male patient, 56 years old, white, uncontrolled hypertension, was admitted to our emergency department (ED) with reports of sudden dizziness, headache of abrupt onset, nausea, vomiting and weakness in the lower limbs. Physical examination drew attention to the presence of manifestations suggestive of cerebellar syndrome: impaired coordination in the trunk or arms and legs, inability to coordinate balance, gait, extremity, uncontrolled or repetitive eye movements, (nystagmus), dyssynergia, dysmetria, dysdiadochokinesia, dysarthria (cerebellar ataxia). Normal myocardial necrosis markers and normal electrolytes ECO 1: anteroseptal-apical akinesis; LVEF = 30% ECO 2 (third day): -
The Epidemiology of the Cerebral Palsies
Clinics in Developmental Medicine No. 87 The Epidemiology of the Cerebral Palsies Edited by FIONA STANLEY EVA ALBERMAN 1984 Spastics International Medical Publications OXFORD: Blackwell Scientific Publications Ltd. PHILADELPHIA: J. B. Lippincott Co. CHAPTER 11 Prenatal and Perinatal Risk Factors in a Survey of 681 Swedish Cases BENGT and GUDRUN HAGBERG I, that am curtail'd of this fair proportion, Cheated of feature by dissembling nature, , Deform'd, unfinish'd, sent before my time Into this breathing world, scarce half made up, And that so lamely and unfashionable That dogs bark at me, as I halt by them. Shakespeare, Richard III Introduction The aim of this chapter is to try and shed light on more specific aspects of the main groups of prenatal causes and risk factors for cerebral palsy, their mutual importance, and particularly their relationship to superimposed detrimental perinatal events. This survey is based on an investigation of 681 cases born in Sweden from 1959 to 1976. In our original retrospective analysis of causes of cerebral palsy (Hagberg et al. 1975a), it was found necessary to make certain generalisations about the aetiological groupings. Only the risk factor that was considered to be the predominating possible cause was used for classification. There is no doubt that this oversimplifies the issue as it neglects the complex network of different interacting detrimental risk factors that are present in the majority of cases, and in all probability underlie the development of brain lesions. Definitions For the purpose of the Swedish investigation, the following definition of cerebral palsy was used: a non-progressive 'disorder of movement and posture due to a defect or lesion of the immature brain' (Bax 1964). -
A Syndrome-Based Clinical Approach for Clerkship Students General Comments 1. This Is Not an All-Inclusive “Cookbook” for Ev
A Syndrome-Based Clinical Approach for Clerkship Students General Comments 1. This is not an all-inclusive “cookbook” for every Neurology patient, but a set of guidelines to help you rationally approach patients with certain syndromes (sets of signs and symptoms which suggest a lesion in particular parts of the nervous system). 2. As you obtain a history and perform a neurological physical exam, try initially to localize all the patient’s signs and symptoms to one, single lesion in the nervous system. It may be surprising that a variety of signs and symptoms, at first glance apparently unrelated, on second thought can localize accurately to a single lesion. If this approach fails, then consider multiple, separate lesions for the patient’s signs and symptoms. 3. The tempo or rate at which signs and symptoms develop or occur often suggests the underlying pathological process. a. sudden onset---favors stroke (ischemia or hemorrhage), seizure, migraine (or other headache syndromes), and trauma b. subacute onset---favors inflammatory, infectious or immune-mediated disorders c. chronic onset---favors degenerative disorders, tumors Toximetabolic disorders, potentially treatable and reversible, may mimic lesions in the nervous system, and can evolve at variable tempos. Hereditary conditions may be congenital (present at birth) and nonprogressive or static, or develop later in life, with variable rates of progression. Family members affected by the same genetic disorder may be remarkably similar with regards to onset and clinical severity, while some genetic disorders vary widely regarding when and how severely family members are affected. 4. In the central nervous system, “positive symptoms or phenomena,” such as flashes of light, or a tingling sensation, suggest “excitation” or increased activity in the nervous system: migraine or seizure. -
Neurogenic Bowel Dysfunction in Patients with Multiple Sclerosis Open Access to Scientific and Medical Research DOI
Journal name: Degenerative Neurological and Neuromuscular Disease Article Designation: Review Year: 2018 Volume: 8 Degenerative Neurological and Neuromuscular Disease Dovepress Running head verso: Preziosi et al Running head recto: Neurogenic bowel dysfunction in patients with multiple sclerosis open access to scientific and medical research DOI: http://dx.doi.org/10.2147/DNND.S138835 Open Access Full Text Article REVIEW Neurogenic bowel dysfunction in patients with multiple sclerosis: prevalence, impact, and management strategies Giuseppe Preziosi Abstract: Bowel dysfunction in patients with multiple sclerosis (MS) is highly prevalent. Ayeshah Gordon-Dixon Constipation and fecal incontinence can coexist and alternate, impacting on the patient’s quality Anton Emmanuel of life and social interactions, as well as burdening the caregivers. The cost for the health care providers is also significant, with increased number of hospital admissions, treatment-related Gastro-Intestinal Physiology Unit, University College London Hospital, costs, and hospital appointments. The origin is multifactorial, and includes alteration of neu- London, UK rological pathways, polypharmacy, behavioral elements, and ability to access the toilet. Every patient with MS should be sensitively questioned about bowel function, and red flag symptoms should prompt adequate investigations. Manipulation of life style factors and establishment of a bowel regime should be attempted in the first place, and if this fails, other measures such For personal use only. as biofeedback and transanal irrigation should be included. A stoma can improve quality of life, and is not necessarily a last-ditch option. Antegrade colonic enemas can also be an effec- tive option, whilst neuromodulation has not proved its role yet. Effective treatment of bowel dysfunction improves quality of life, reduces incidence of urinary tract infection, and reduces health care costs. -
Child Neurology: Hereditary Spastic Paraplegia in Children S.T
RESIDENT & FELLOW SECTION Child Neurology: Section Editor Hereditary spastic paraplegia in children Mitchell S.V. Elkind, MD, MS S.T. de Bot, MD Because the medical literature on hereditary spastic clinical feature is progressive lower limb spasticity B.P.C. van de paraplegia (HSP) is dominated by descriptions of secondary to pyramidal tract dysfunction. HSP is Warrenburg, MD, adult case series, there is less emphasis on the genetic classified as pure if neurologic signs are limited to the PhD evaluation in suspected pediatric cases of HSP. The lower limbs (although urinary urgency and mild im- H.P.H. Kremer, differential diagnosis of progressive spastic paraplegia pairment of vibration perception in the distal lower MD, PhD strongly depends on the age at onset, as well as the ac- extremities may occur). In contrast, complicated M.A.A.P. Willemsen, companying clinical features, possible abnormalities on forms of HSP display additional neurologic and MRI abnormalities such as ataxia, more significant periph- MD, PhD MRI, and family history. In order to develop a rational eral neuropathy, mental retardation, or a thin corpus diagnostic strategy for pediatric HSP cases, we per- callosum. HSP may be inherited as an autosomal formed a literature search focusing on presenting signs Address correspondence and dominant, autosomal recessive, or X-linked disease. reprint requests to Dr. S.T. de and symptoms, age at onset, and genotype. We present Over 40 loci and nearly 20 genes have already been Bot, Radboud University a case of a young boy with a REEP1 (SPG31) mutation. Nijmegen Medical Centre, identified.1 Autosomal dominant transmission is ob- Department of Neurology, PO served in 70% to 80% of all cases and typically re- Box 9101, 6500 HB, Nijmegen, CASE REPORT A 4-year-old boy presented with 2 the Netherlands progressive walking difficulties from the time he sults in pure HSP. -
Cerebral Palsy the ABC's of CP
Cerebral Palsy The ABC’s of CP Toni Benton, M.D. Continuum of Care Project UNM HSC School of Medicine April 20, 2006 Cerebral Palsy Outline I. Definition II. Incidence, Epidemiology and Distribution III. Etiology IV. Types V. Medical Management VI. Psychosocial Issues VII. Aging Cerebral Palsy-Definition Cerebral palsy is a symptom complex, (not a disease) that has multiple etiologies. CP is a disorder of tone, posture or movement due to a lesion in the developing brain. Lesion results in paralysis, weakness, incoordination or abnormal movement Not contagious, no cure. It is static, but it symptoms may change with maturation Cerebral Palsy Brain damage Occurs during developmental period Motor dysfunction Not Curable Non-progressive (static) Any regression or deterioration of motor or intellectual skills should prompt a search for a degenerative disease Therapy can help improve function Cerebral Palsy There are 2 major types of CP, depending on location of lesions: Pyramidal (Spastic) Extrapyramidal There is overlap of both symptoms and anatomic lesions. The pyramidal system carries the signal for muscle contraction. The extrapyramidal system provides regulatory influences on that contraction. Cerebral Palsy Types of brain damage Bleeding Brain malformation Trauma to brain Lack of oxygen Infection Toxins Unknown Epidemiology The overall prevalence of cerebral palsy ranges from 1.5 to 2.5 per 1000 live births. The overall prevalence of CP has remained stable since the 1960’s. Speculations that the increased survival of the VLBW preemies would cause a rise in the prevalence of CP have proven wrong. Likewise the expected decrease in CP as a result of C-section and fetal monitoring has not happened. -
Hereditary Spastic Paraplegia
8 Hereditary Spastic Paraplegia Notes and questions Hereditary Spastic Paraplegia What is Hereditary Spastic Paraplegia? Hereditary Spastic Paraplegia (HSP) is a medical term for a condition that affects muscle function. The terms spastic and paraplegia comes from several words in Greek: • ‘spastic’ means afflicted with spasms (an alteration in muscle tone that results in affected movements) • ‘paraplegia’ meaning an impairment in motor or sensory function of the lower extremities (from the hips down) What are the signs and symptoms of HSP? Muscular spasticity • Individuals with HSP commonly will have lower extremity weakness, spasticity, and muscle stiffness. • This can cause difficulty with walking or a “scissoring” gait. We are grateful to an anonymous donor for making a kind and Other common signs or symptoms include: generous donation to the Neuromuscular and Neurometabolic Centre. • urinary urgency • overactive or over responsive “brisk” reflexes © Hamilton Health Sciences, 2019 PD 9983 – 01/2019 Dpc/pted/HereditarySpasticParaplegia-trh.docx dt/January 15, 2019 ____________________________________________________________________________ 2 7 Hereditary Spastic Paraplegia Hereditary Spastic Paraplegia HSP is usually a chronic or life-long disease that affects If you have any questions about DM1, please speak with your people in different ways. doctor, genetic counsellor, or nurse at the Neuromuscular and Neurometabolic Centre. HSP can be classified as either “Uncomplicated HSP” or “Complicated HSP”. Notes and questions Types of Hereditary Spastic Paraplegia 1. Uncomplicated HSP: • Individuals often experience difficulty walking as the first symptom. • Onset of symptoms can begin at any age, from early childhood through late adulthood. • Symptoms may be non-progressive, or they may worsen slowly over many years. -
Functional Neurologic Disorders and Related Disorders Victor W Mark MD ( Dr
Functional neurologic disorders and related disorders Victor W Mark MD ( Dr. Mark of the University of Alabama at Birmingham has no relevant financial relationships to disclose. ) Originally released April 18, 2001; last updated December 13, 2018; expires December 13, 2021 Introduction This article includes discussion of psychogenic neurologic disorders, functional neurologic disorder, functional movement disorder, conversion disorder, and hysteria. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations. Overview Several behavioral disorders are related by (1) their resemblance to other, more familiar neurologic disorders; (2) lack of well-established biomarkers (eg, structural lesions on brain imaging studies, seizure waveforms on EEGs); and (3) aggravation of symptoms with the patient s attention to the disorder. However, the features and causes for these disorders are very different among themselves. This topic reviews functional neurologic disorder, Munchausen syndrome, Munchausen syndrome by proxy, and Ganser syndrome. Key points • Functional neurologic disorders are commonly encountered in general neurologic practices and, hence, knowing their manifestations and treatment is crucial for clinical care. • The disturbance is involuntary, yet at the same time it can be controlled by the patient intermittently. • Despite being self-controllable, the disturbance is generally disabling unless expert professional care is provided. • There is no consistent association between functional neurologic disorder and either posttraumatic emotional stress or sexual abuse. • Functional neurologic disturbances disorder responds best to empathetic concern by the clinician; demonstration that the disorder lacks a structural or permanent etiology; explanation that it can be improved with distraction; and guided attempts to reduce triggers of onset. Cognitive behavioral therapy, combined with physical therapy when warranted, is emerging as a successful intervention. -
Approach to a Patient with Hemiplegia and Monoplegia
CHAPTER Approach to a Patient with Hemiplegia and Monoplegia 27 Sudhir Kumar, Subhash Kaul INTRODUCTION 4. Injury to multiple cervical nerve roots. Monoplegia and hemiplegia are common neurological 5. Functional or psychogenic. symptoms in patients presenting to the emergency department as well as outpatient department. Insidious onset, gradually progressive monoplegia affecting lower limb can be caused by the following Monoplegia refers to weakness of one limb (either arm or conditions: leg) and hemiplegia refers to weakness of one arm and leg on the same side of body (either left or right side). 1. Tumor of the contralateral frontal lobe. There are a variety of underlying causes for monoplegia 2. Tumor of spinal cord at thoracic or lumbar level. and hemiplegia. The causes differ in different age groups. 3. Chronic infection of brain (frontal lobe) or spinal The causes also differ depending on the onset, progression cord (thoracic or lumbar level), such as tuberculous. and duration of weakness. Therefore, one needs to adopt a systematic approach during history taking and 4. Lumbosacral-plexopathy, due to diabetes mellitus. examination in order to arrive at the correct diagnosis. Insidious onset, gradually progressive monoplegia, Appropriate investigations after these would confirm the affecting upper limb, can be caused by one of the following diagnosis. conditions: The aim of this chapter is to systematically look at the 1. Tumor of the contralateral parietal lobe. differential diagnosis of monoplegia and hemiplegia and outline the approach needed to pinpoint the exact 2. Compressive lesion (tumor, large disc, etc) in underlying cause. cervical cord region. 3. Chronic infection of the brain (parietal lobe) or APPROACH TO THE DIAGNOSIS OF MONOPLEGIA spinal cord (cervical region), such as tuberculous. -
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 -
Absence of Neurobehavioral Disturbance in a Focal Lesion of the Left Paracentral Lobule
Behavioural Neurology (1992), 5,189-191 ICASE REPORTI Absence of neurobehavioral disturbance in a focal lesion of the left paracentral lobule T. Imamura and K. Tsuburaya Department of Neurology, Tohoku Kohseinenkin Hospital, Sendai, Japan Correspondence to: T. Imamura, Department of Neurology, Institute of Brain Diseases, Tohoku University School of Medicine, 1-1, Seiryo-machi, Aoba-Ku, Sendai 980, Japan The case of a right-handed woman with an infarcation confined to the left paracentral lobule and sparing the supplementary motor area (SMA) is reported. She presented with a right leg monoplegia and displayed no mutism. The absence of any associ ated neurobehavioral disturbances (mutism, forced grasping, reduced spontaneous arm activity or aphasia raises the possi bility that the left SMA has discrete neurobehavioral functions. Keywords: Medial frontal lobe - Precentral gyrus - Supplementary motor area - Transcortical motor aphasia INTRODUCTION Various kinds of neurobehavioral disturbances associated medial part of the left precentral gyrus, which is adjacent with left medial frontal lesions involving the supplemen to the SMA, to evaluate its possible neurobehavioral tary motor area (SMA) have been reported. Aphasia due to functions. damage of the left medial frontal lobe is characterized by an initial period of mutism followed by a stage of CASE REPORT decreased verbal output and spontaneous initiation with normal articulation (Stuss and Benson, 1986). Forced A 73-year-old right-handed woman with a history of grasping, compulsive manipulation of tools and decreased hypertension and diabetes mellitus suddenly developed a spontaneous limb movements have also been described gait disturbance. On examination, 24 h later, she was alert (Wise, 1984; Feinberg et al., 1992).