Neurologic Disease Session Guidelines
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Rest Tremor Revisited: Parkinson's Disease and Other Disorders
Chen et al. Translational Neurodegeneration (2017) 6:16 DOI 10.1186/s40035-017-0086-4 REVIEW Open Access Rest tremor revisited: Parkinson’s disease and other disorders Wei Chen1,2, Franziska Hopfner2, Jos Steffen Becktepe2 and Günther Deuschl1,2* Abstract Tremor is the most common movement disorder characterized by a rhythmical, involuntary oscillatory movement of a body part. Since distinct diseases can cause similar tremor manifestations and vice-versa,itischallengingtomakean accurate diagnosis. This applies particularly for tremor at rest. This entity was only rarely studied in the past, although a multitude of clinical studies on prevalence and clinical features of tremor in Parkinson’s disease (PD), essential tremor and dystonia, have been carried out. Monosymptomatic rest tremor has been further separated from tremor-dominated PD. Rest tremor is also found in dystonic tremor, essential tremor with a rest component, Holmes tremor and a few even rarer conditions. Dopamine transporter imaging and several electrophysiological methods provide additional clues for tremor differential diagnosis. New evidence from neuroimaging and electrophysiological studies has broadened our knowledge on the pathophysiology of Parkinsonian and non-Parkinsonian tremor. Large cohort studies are warranted in future to explore the nature course and biological basis of tremor in common tremor related disorders. Keywords: Tremor, Parkinson’s disease, Essential tremor, Dystonia, Pathophysiology Background and clinical correlates of tremor in common tremor re- Tremor is defined as a rhythmical, involuntary oscillatory lated disorders. Some practical clinical cues and ancillary movement of a body part [1]. Making an accurate diagnosis tests for clinical distinction are found [3]. Besides, accu- of tremor disorders is challenging, since similar clinical mulating structural and functional neuroimaging, as well entities may be caused by different diseases. -
Neuroleptic Malignant-Like Syndrome
An uncommon adverse effect of levodopa CASE REPORT withdrawal in a patient taking antipsychotic medication: neuroleptic malignant-like syndrome SP Man 文兆彪 A patient with symptoms suggestive of neuroleptic malignant syndrome after levodopa withdrawal is described. The patient presented with persistent high fever, stupor, autonomic dysfunction, rigidity, and rhabdomyolysis. He was successfully treated with intravenous dantrolene, resumption of levodopa, and forced alkaline diuresis. Doctors should be aware of the risk of abrupt cessation of dopamine agonists. Introduction Neuroleptic malignant syndrome (NMS) is an idiosyncratic, potentially fatal complication of treatment with antipsychotic drugs that manifests as fever, muscle rigidity, and autonomic and mental dysfunction.1 A similar clinical presentation has also been reported to develop after withdrawal from dopamine agonists.2,3 Some authors have used the terms neuroleptic malignant-like syndrome (NMLS) or parkinsonism hyperpyrexia syndrome, as well as acute akinesia or the malignant syndrome in Parkinson disease, for such a condition.4,5 The objective of this paper was to describe the first patient with NMLS in Hong Kong. Case report An 84-year-old Chinese man had chronic schizophrenia and was treated with chlorpromazine 50 mg at night for many years. Eighteen months prior to the index admission, chlorpromazine was replaced by olanzapine 20 mg daily as he had experienced extrapyramidal symptoms. He was also given levodopa 100 mg and benserazide 25 mg 3 times daily. He was admitted to Tuen Mun Hospital, Hong Kong, for a fever of 39.5ºC on 24 April 2010. He was conscious and did not have any specific symptoms. All medications, including olanzapine and levodopa, were stopped at admission as he was not permitted anything by mouth. -
Case Report of Hyperacute Edema and Cavitation Following Deep Brain Stimulation Lead Implantation Albert J
www.surgicalneurologyint.com Surgical Neurology International Editor-in-Chief: Nancy E. Epstein, MD, Clinical Professor of Neurological Surgery, School of Medicine, State U. of NY at Stony Brook. SNI: Stereotactic Editor Veronica Lok-Sea Chiang, MD Yale School of Medicine, New Haven, CT, USA Open Access Case Report Case report of hyperacute edema and cavitation following deep brain stimulation lead implantation Albert J. Fenoy1,2, Christopher R. Conner2, Joseph S. Withrow2, Aaron W. Hocher2 Movement Disorders and Neurodegenerative Disease Program, Departments of 1Neurology, 2Neurosurgery, McGovern Medical School, Houston, Texas, United States. E-mail: *Albert J. Fenoy - [email protected]; Christopher R. Conner - [email protected]; Joseph S. Withrow - joseph.s.withrow@ uth.tmc.edu; Aaron W. Hocher - [email protected] ABSTRACT Background: Postoperative cerebral edema around a deep brain stimulation (DBS) electrode is an uncommonly reported complication of DBS surgery. e etiology of this remains unknown, and the presentation is highly variable; however, the patients generally report a good outcome. Case Description: Here, we report an unusual presentation of postoperative edema in a 66-year-old female *Corresponding author: who has bilateral dentatorubrothalamic tract (specifically, the ventral intermediate nucleus) DBS for a mixed Albert J. Fenoy, type tremor disorder. Initial postoperative computed tomography (CT) was unremarkable and the patient was Department of Neurosurgery, admitted for observation. She declined later on postoperative day (POD) 1 and became lethargic. Stat head CT McGovern Medical School, scan performed revealed marked left-sided peri-lead edema extending into the centrum semiovale with cystic 6431 Fannin St., Houston, Texas cavitation, and trace right-sided edema. -
Multiple Developmental Forms of Parkinsonism. the Basis for Further Research As to the Pathogenesis of Parkinsonism
J Neural Transm (2002) 109: 1469–1475 Mini-Review: Multiple developmental forms of parkinsonism. The basis for further research as to the pathogenesis of parkinsonism P. Riederer and P. Foley Clinical Neurochemistry, Clinic for Psychiatry and Psychotherapy and NPF-Center of Excellence Research Laboratories, University of Würzburg, Federal Republic of Germany Received September, 2002; accepted October 31, 2002 Summary. A range of extrapyramidal disturbances have been reported in children following early brain damage. In adults, damage to the basal ganglia can elicit abnormal motor activity in either direction; it would seem reason- able that the same would apply to damage occurring at an earlier develop- mental stage. The Viennese paediatrician Widhalm described a hypokinetic/ parkinsonoid syndrome (‘infantile hypokinetic-hypertonic syndrome with Parkinson symptomatic’) presented by a significant minority of the children with extrapyramidal movement disturbances, corresponding to the mild rigid- akinetic type of Parkinson’s disease. In contrast to classical parkinsonism, but consistent with some forms of post-encephalitic parkinsonism, the syndrome was reversible, although only after l-DOPA therapy. Widhalm’s observation that at least one form of childhood parkinsonism can be cured with l-DOPA also suggests that the amino acid plays a more active role than mere replace- ment therapy in children, perhaps also acting as a neurotrophic agent. It is proposed that environmental factors, including viral and risk factors associ- ated with pregnancy and birth, together with genetically determined lability, may increase the incidence of early hypokinesia/parkinsonism in particular and of Parkinson’s disease in later life by disturbing the immature basal ganglia at critical developmental stages. -
Suarez, JI: Hypertonic Saline for Cerebral Edema
Hypertonic saline for cerebral edema and elevated intracranial pressure JOSE´ I. SUAREZ, MD erebral edema and elevated intracranial movements. Because transport through the BBB is a pressure (ICP) are important and frequent selective process, the osmotic gradient that a particle problems in the neurocritically ill patient. can create is also dependent on how restricted its They can both result from various insults permeability through the barrier is. This restriction is C expressed in the osmotic reflection coefficient, which to the brain. Improving cerebral edema and decreas- ing ICP has been associated with improved out- ranges from 0 (for particles that can diffuse freely) to come.1 However, all current treatment modalities 1.0 (for particles that are excluded the most effec- are far from perfect and are associated with serious tively and therefore are osmotically the most active). adverse events:1–4 indiscriminate hyperventilation The reflection coefficient for sodium chloride is can lead to brain ischemia; mannitol can cause 1.0 (mannitol’s is 0.9), and under normal conditions intravascular volume depletion, renal insufficiency, sodium (Na+) has to be transported actively into the and rebound ICP elevation; barbiturates are associat- CSF.5,6 Animal studies have shown that in condi- ed with cardiovascular and respiratory depression tions of an intact BBB, CSF Na+ concentrations and prolonged coma; and cerebrospinal fluid (CSF) increase when an osmotic gradient exists but lag drainage via intraventricular catheter insertion may behind plasma concentrations for 1 to 4 hours.5 result in intracranial bleeding and infection. Thus, elevations in serum Na+ will create an effec- Other treatment modalities have been explored, tive osmotic gradient and draw water from brain into and hypertonic saline (HS) solutions particularly the intravascular space. -
Neurologic Complications of Electrolyte Disturbances and Acid–Base Balance
Handbook of Clinical Neurology, Vol. 119 (3rd series) Neurologic Aspects of Systemic Disease Part I Jose Biller and Jose M. Ferro, Editors © 2014 Elsevier B.V. All rights reserved Chapter 23 Neurologic complications of electrolyte disturbances and acid–base balance ALBERTO J. ESPAY* James J. and Joan A. Gardner Center for Parkinson’s Disease and Movement Disorders, Department of Neurology, UC Neuroscience Institute, University of Cincinnati, Cincinnati, OH, USA INTRODUCTION hyperglycemia or mannitol intake, when plasma osmolal- ity is high (hypertonic) due to the presence of either of The complex interplay between respiratory and renal these osmotically active substances (Weisberg, 1989; function is at the center of the electrolytic and acid-based Lippi and Aloe, 2010). True or hypotonic hyponatremia environment in which the central and peripheral nervous is always due to a relative excess of water compared to systems function. Neurological manifestations are sodium, and can occur in the setting of hypovolemia, accompaniments of all electrolytic and acid–base distur- euvolemia, and hypervolemia (Table 23.2), invariably bances once certain thresholds are reached (Riggs, reflecting an abnormal relationship between water and 2002). This chapter reviews the major changes resulting sodium, whereby the former is retained at a rate faster alterations in the plasma concentration of sodium, from than the latter (Milionis et al., 2002). Homeostatic mech- potassium, calcium, magnesium, and phosphorus as well anisms protecting against changes in volume and sodium as from acidemia and alkalemia (Table 23.1). concentration include sympathetic activity, the renin– angiotensin–aldosterone system, which cause resorption HYPONATREMIA of sodium by the kidneys, and the hypothalamic arginine vasopressin, also known as antidiuretic hormone (ADH), History and terminology which prompts resorption of water (Eiskjaer et al., 1991). -
Genome-Wide Analysis of the Parkinsonism-Dementia Complex
ORIGINAL CONTRIBUTION Genome-Wide Analysis of the Parkinsonism- Dementia Complex of Guam Huw R. Morris, MB, PhD; John C. Steele, MD; Richard Crook, BSc; Fabienne Wavrant-De Vrièze, PhD; Luisa Onstead-Cardinale, BS; Katrina Gwinn-Hardy, MD; Nick W. Wood, MD, PhD; Matthew Farrer, PhD; Andrew J. Lees, MD; P. L. McGeer, MD, PhD; Teepu Siddique, MD, PhD; John Hardy, PhD; Jordi Perez-Tur, PhD Background: Parkinsonism-dementia complex (PDC) went conventional linkage analysis in 5 families with PDC. is a neurofibrillary tangle degeneration involving the depo- One marker, D20S103, generated a logarithm of odds score sition of Alzheimer-type tau, predominantly in the me- of greater than 1.5. Multipoint association analysis also sial temporal cortex, brainstem, and basal ganglia. It oc- highlighted 2 other areas on chromosome 14q (adjacent curs in focal geographic isolates, including Guam and the to D14S592, 59.2 megabases [M]) and chromosome 20 Kii peninsula of Japan. The familial clustering of the dis- (adjacent to D20S470, 17.4 M) with multipoint associa- ease has suggested that a genetic factor could be impor- tion logarithm of the odds scores of greater than 2. The tant in its etiology. areas around D20S103, D14S592, and D20S470 were fur- ther analyzed by association using additional microsat- Objective: To determine whether a genetic locus could ellite markers and by conventional linkage analysis. This be identified, linked, or associated with PDC. did not provide further evidence for the role of these areas in PDC. Design and Patients: We performed a genome-wide association study of 22 Guamanian PDC and 19 control Conclusions: This study has not identified a single subjects using 834 microsatellite markers with an ap- gene locus for PDC, confirming the impression of a geo- proximate genome-wide marker density of 4.4 centi- graphic disease isolate with a complex genetic, a genetic/ morgans. -
Early Seizures and Cerebral Edema After Trivial Head Trauma Associated
Early seizures and cerebral edema after trivial head trauma associated with the CACNA1A S218L mutation Anine H Stam, Gert Jan Luijckx, Bwee Tien Poll-The, Ieke Ginjaar, Rune R Frants, Joost Haan, Michel D Ferrari, Gisela M Terwindt, Arn M J M van den Maagdenberg To cite this version: Anine H Stam, Gert Jan Luijckx, Bwee Tien Poll-The, Ieke Ginjaar, Rune R Frants, et al.. Early seizures and cerebral edema after trivial head trauma associated with the CACNA1A S218L mutation. Journal of Neurology, Neurosurgery and Psychiatry, BMJ Publishing Group, 2009, 80 (10), pp.1125. 10.1136/jnnp.2009.177279. hal-00552784 HAL Id: hal-00552784 https://hal.archives-ouvertes.fr/hal-00552784 Submitted on 6 Jan 2011 HAL is a multi-disciplinary open access L’archive ouverte pluridisciplinaire HAL, est archive for the deposit and dissemination of sci- destinée au dépôt et à la diffusion de documents entific research documents, whether they are pub- scientifiques de niveau recherche, publiés ou non, lished or not. The documents may come from émanant des établissements d’enseignement et de teaching and research institutions in France or recherche français ou étrangers, des laboratoires abroad, or from public or private research centers. publics ou privés. Early seizures and cerebral edema after trivial head trauma associated with the CACNA1A S218L mutation 1* 2* 3* Anine H. Stam, MD , Gert-Jan Luijckx, MD, PhD , Bwee Tien Poll-Thé, MD, PhD Ieke B. Ginjaar, PhD4, Rune R. Frants, PhD5, Joost Haan, MD, PhD1,6, Michel D. Ferrari, MD, PhD1, Gisela M. Terwindt, MD, PhD1, Arn M.J.M. -
Diagnosis and Treatment of Cerebrospinal Fluid Rhinorrhea Following Accidental Traumatic Anterior Skull Base Fractures
Neurosurg Focus 32 (6):E3, 2012 Diagnosis and treatment of cerebrospinal fluid rhinorrhea following accidental traumatic anterior skull base fractures MATEO ZIU, M.D., JENNIFER GENTRY SAVAGE, M.D., AND DAVID F. JIMENEZ, M.D. Department of Neurosurgery, University of Texas Health Science Center at San Antonio, Texas Cerebrospinal fluid rhinorrhea is a serious and potentially fatal condition because of an increased risk of menin- gitis and brain abscess. Approximately 80% of all cases occur in patients with head injuries and craniofacial fractures. Despite technical advances in the diagnosis and management of CSF rhinorrhea caused by craniofacial injury through the introduction of MRI and endoscopic extracranial surgical approaches, difficulties remain. The authors review here the pathophysiology, diagnosis, and management of CSF rhinorrhea relevant exclusively to traumatic anterior skull base injuries and attempt to identify areas in which further work is needed. (http://thejns.org/doi/abs/10.3171/2012.4.FOCUS1244) KEY WORDS • craniomaxillofacial trauma • head trauma • cerebrospinal fluid rhinorrhea • meningitis • dural repair • endoscopic surgery • skull base fracture EREBROSPINAL fluid rhinorrhea is a serious and po- Furthermore, the issues related to CSF leak caused tentially fatal condition that still presents a major by traumatic injury are complex and multiple. Having challenge in terms of its diagnosis and manage- conducted a thorough review of existing literature, we Cment. It is estimated that meningitis develops in approxi- discuss here the pathophysiology, diagnosis, and man- mately 10%–25% of patients with this disorder, and 10% agement of CSF rhinorrhea relevant to traumatic anterior of them die as a result. Approximately 80% of all cases of skull base injuries and attempt to identify areas in which CSF rhinorrhea are caused by head injuries that are asso- further research is needed. -
Parkinsonism: Onset, Progression, and Mortality
Parkinsonism: onset, progression, and mortality Margaret M. Hoehn, M.D., and Melvin D. Yahr, M.D. PARKINSONISM,described in its entirety over lated to differences in terminology regarding one hundred and fifty years ago,’ rarely pre- the type of parkinsonism and the degree of its sents itself as a diagnostic problem. In conse- severity at the time of treatment. Consequent- quence, little scrutiny has been directed to the ly, it is difficult to determine whether treat- marked variability of this frequently encoun- ment really influences the course of the disease tered neurological syndrome and to the progres- and to what extent symptomatic relief is sig- sion of the disease in large groups of patients. nificant. It seems clear that, at a time when As with most chronic neurological disorders, many new approaches to the treatment of marked diversity can be expected to exist in parkinsonism are being suggested, information age and mode of onset, relative prominence of this sort is of paramount importance. of the cardinal signs and symptoms, rate of With this in mind, a study was made of 856 progression, and resultant degree of functional patients bearing the diagnosis of paralysis impairment. Controversy over the effectiveness agitans, Parkinson’s disease, or parkinsonism of therapeutic measures for parkinsonism is who were seen at the Vanderbilt Clinic of the due partially to this wide variability and to Columbia-Presbyterian Medical Center from the paucity of clinical information about the 1949 to 1964, inclusively. natural history of the -
Contemporary Approach to the Diagnosis and Management of Cerebrospinal Fluid Rhinorrhea
REVIEWS AND CONTEMPORARY UPDATES Ochsner Journal 16:136–142, 2016 Ó Academic Division of Ochsner Clinic Foundation Contemporary Approach to the Diagnosis and Management of Cerebrospinal Fluid Rhinorrhea Tiffany Mathias, BS,1 Joshua Levy, MD,1 Adil Fatakia, MD,2 Edward D. McCoul, MD3,4 1Department of Otolaryngology – Head and Neck Surgery, Tulane University School of Medicine, New Orleans, LA 2ENT New Orleans, West Jefferson Physician Center, Marrero, LA 3Department of Otorhinolaryngology, Ochsner Clinic Foundation, New Orleans, LA 4The University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA Background: Cerebrospinal fluid (CSF) rhinorrhea, when left untreated, can lead to meningitis and other serious complications. Treatment traditionally has entailed an open craniotomy, although the paradigm has now evolved to encompass endoscopic procedures. Trauma, both accidental and iatrogenic, causes the majority of leaks, and trauma involving skull base and facial fractures is most likely to cause CSF rhinorrhea. Diagnosis is aided by biochemical assay and imaging studies. Methods: We reviewed the literature and summarized current practice regarding the diagnosis and management of CSF rhinorrhea. Results: Management of CSF leaks is dictated by the nature of the fistula, its location, and flow volume. Control of elevated intracranial pressure may require medical therapy or shunt procedures. Surgical reconstruction utilizes a graduated approach involving vascularized, nonvascularized, and adjunctive techniques to achieve closure of the CSF leak. Endoscopic techniques have an important role in select cases. Conclusion: An active surgical approach to closing CSF leaks may provide better long-term outcomes in some patients compared to more conservative management. Keywords: Cerebrospinal fluid, cerebrospinal fluid leak, cerebrospinal fluid rhinorrhea, endoscopy, skull base Address correspondence to Edward D. -
Movement Disorders Emergencies Part 1 Hypokinetic Disorders
NEUROLOGICAL REVIEW SECTION EDITOR: DAVID E. PLEASURE, MD Movement Disorders Emergencies Part 1 Hypokinetic Disorders Bradley J. Robottom, MD; William J. Weiner, MD; Stewart A. Factor, DO ovement disorders usually do not require emergent intervention; nevertheless, there are acute/subacute clinical settings in which the neurologist is consulted. It is in these circumstances that the neurologist must be prepared to accurately diagnose and properly treat the patient. We have reviewed the literature regarding move- Mment disorder emergencies and divided them into hypokinetic (part 1) and hyperkinetic (part 2) presentations. In part 1, drug-induced syndromes including neuroleptic malignant syndrome, par- kinsonism hyperpyrexia syndrome, and serotonin syndrome will be discussed. Emergency com- plications related to the management of Parkinson disease, including falling, motor fluctuations, and psychiatric issues, will also be reviewed. Arch Neurol. 2011;68(5):567-572 Movement disorders often have an insidi- DRUG-INDUCED MOVEMENT ous onset and slow progression and are not DISORDER EMERGENCIES often associated with emergency situa- tions. However, neurologists may be called Neuroleptic Malignant Syndrome on to diagnose and treat evolving move- ment disorders or acute complications of Neuroleptic malignant syndrome, first de- existing diseases in the emergency depart- scribed in 1960,2 is an iatrogenic disorder re- ment or intensive care unit. Such situa- sulting from exposure to drugs that block tions meet the working definition of an dopamine