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NEUROLOGY

SIXTH EDITION
JOSEPH S. KASS, MD, JD
Associate Professor
Departments of Neurology and Psychiatry & Behavioral Sciences
Center for Medical Ethics & Health Policy
Vice Chair for Education
Department of Neurology
Assistant Dean of Student Affairs
Baylor College of Medicine;
Chief of Neurology
Ben Taub General Hospital
Houston, Texas

ELI M. MIZRAHI, MD
Chair
Department of Neurology
Professor
Departments of Neurology and Pediatrics
James A. Quigley Endowed Chair in Pediatric Neurology
Baylor College of Medicine
Houston, Texas
1600 John F. Kennedy Blvd.
Ste 1800
Philadelphia, PA 19103-2899

NEUROLOGY SECRETS, 6TH EDITION ISBN: 978-0-323-35948-1

Copyright © 2017 by Elsevier, Inc. All rights reserved.

No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechani-
cal, including photocopying, recording, or any information storage and retrieval system, without permission in
writing from the publisher. Details on how to seek permission, further information about the Publisher’s permis-
sions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copy-
right Licensing Agency, can be found at our website: www.elsevier.com/permissions.

This book and the individual contributions contained in it are protected under copyright by the Publisher (other
than as may be noted herein).

Notices

Knowledge and best practice in this field are constantly changing. As new research and experience broaden
our understanding, changes in research methods, professional practices, or medical treatment may become
necessary.
Practitioners and researchers must always rely on their own experience and knowledge in evaluating and
using any information, methods, compounds, or experiments described herein. In using such information or
methods they should be mindful of their own safety and the safety of others, including parties for whom they
have a professional responsibility.
With respect to any drug or pharmaceutical products identified, readers are advised to check the most
current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be
administered, to verify the recommended dose or formula, the method and duration of administration, and
contraindications. It is the responsibility of practitioners, relying on their own experience and knowledge of
their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient,
and to take all appropriate safety precautions.
To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume
any liability for any injury and/or damage to persons or property as a matter of products liability, negligence
or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the
material herein.

Previous editions 2010, 2005, 2001, 1998, and 1993.

Library of Congress Cataloging-in-Publication Data

Names: Kass, Joseph S., editor. | Mizrahi, Eli M., editor.

Title: Neurology secrets / [edited by] Joseph S. Kass, Eli M. Mizrahi.
Other titles: Secrets series.
Description: Sixth edition. | Philadelphia, PA : Elsevier, Inc, [2017] |
Series: Secrets series | Includes bibliographical references and index.
Identifiers: LCCN 2016013080 | ISBN 9780323359481 (pbk.)
Subjects: | MESH: Nervous System Diseases | Examination Questions
Classification: LCC RC346 | NLM WL 18.2 | DDC 616.8--dc23 LC record
available at http://lccn.loc.gov/2016013080

Content Strategist: James Merritt

Content Development Specialist: Rae Robertson
Publishing Services Manager: Hemamalini Rajendrababu
Project Manager: Srividhya Vidhyashankar
Design Direction: Ryan Cook

Printed in United States of America

Last digit is the print number: 9 8 7 6 5 4 3 2 1

CONTRIBUTORS

Garima Arora, MS, MD Everton A. Edmondson, MD

Assistant Professor Clinical Assistant Professor
Department of Psychiatry and Behavioral Department of Neurology
Sciences Baylor College of Medicine
The University of Texas Health Science Center at Houston, Texas
Houston
Randolph W. Evans, MD
Houston, Texas
Clinical Professor
Igor M. Cherches, MD Department of Neurology
The Neurology Center Baylor College of Medicine
Houston, Texas Houston, Texas
Jonathan Clark, MD, MPH Daniel G. Glaze, MD, FAASM
Associate Professor Professor
Department of Neurology Departments of Pediatrics and Neurology
Center for Space Medicine Baylor College of Medicine;
Baylor College of Medicine Medical Director, The Children’s Sleep Center
Houston, Texas Medical Director, The Blue Bird Circle Rett Center
Texas Children’s Hospital
Helen S. Cohen, EdD, OTR, FAOTA Houston, Texas
Professor
Bobby R Alford Department of Otolaryngology - Corey E. Goldsmith, MD
Head and Neck Surgery Assistant Professor
Baylor College of Medicine Department of Neurology
Houston, Texas Director, Neurology Residency Program
Baylor College of Medicine;
Matthew D. Cykowski, MD Chief of Neurology Clinics
Adjunct Associate Professor Smith Clinic, Harris Health System
Department of Pathology and Immunology Houston, Texas
Baylor College of Medicine
Staff Pathologist; Clifton L. Gooch, MD
Department of Pathology and Genomic Professor and Chair
Medicine Department of Neurology
Houston Methodist Hospital Director
Houston, Texas USF Neuroscience Collaborative
University of South Florida
Rachelle S. Doody, MD, PhD Tampa, Florida
Professor
Effie Marie Cain Chair in Alzheimer’s Disease Pramod Gupta, MD
Research Fellow
Director, Alzheimer’s Disease and Memory Division of Vascular Neurology and
Disorders Center Neurocritical Care
Department of Neurology Department of Neurology
Baylor College of Medicine Baylor College of Medicine
Houston, Texas Houston, Texas
John D. Eatman, MD Zulfi Haneef, MD
Resident Assistant Professor
Department of Neurology Department of Neurology
Houston Methodist Hospital Baylor College of Medicine
Houston, Texas Houston, Texas

vii
 viii Contributors

Philip A. Hanna, MD, FAAN Joseph S. Kass, MD, JD

Neurology Associate Professor
Movement Disorders Departments of Neurology and Psychiatry &
JFK Neuroscience Institute; Behavioral Sciences
Associate Professor Center for Medical Ethics & Health Policy
Department of Neurology Vice Chair for Education
Seton Hall University School of Health and Medical Department of Neurology
Sciences; Assistant Dean of Student Affairs
Clinical Associate Professor Department of Baylor College of Medicine;
Neurology Chief of Neurology
Rutgers Robert Wood Johnson Medical School; Ben Taub General Hospital
Associate Professor Houston, Texas
Department of Medicine
Thomas A. Kent, MD
St. George’s University School of Medicine
Professor and Director
Great River, New York
Cerebrovascular Research
Richard A. Hrachovy, MD Department of Neurology
Distinguished Emeritus Professor Baylor College of Medicine;
Department of Neurology Neurology Care Line Executive
Baylor College of Medicine Michael E. DeBakey VA Medical Center
Houston, Texas Houston, Texas
Shahram Izadyar, MD James M. Killian, MD
Assistant Professor Professor
Director, Clinical Neurophysiology Fellowship Department of Neurology
Program Baylor College of Medicine
Department of Neurology Houston, Texas
Upstate Medical University
Doris Kung, DO
Syracuse, New York
Assistant Professor
George R. Jackson, MD, PhD Director, Medical Student Clerkship
Professor Department of Neurology
Department of Neurology Baylor College of Medicine
Baylor College of Medicine; Houston, Texas
Associate Director for Research
Parkinson’s Disease Research Education and Eugene C. Lai, MD, PhD
Professor
Clinical Center
Departments of Neurology and Neuroscience
Michael E. DeBakey VA Medical Center
Robert W. Hervey Distinguished Endowed Chair in
Houston TX
Parkinson’s Disease
Joseph Jankovic, MD Houston Methodist Neurological Institute and
Professor Weill-Cornell Medical College
Distinguished Chair in Movement Disorders; Houston, Texas
Director, Parkinson’s Disease Center and Movement
Atul Maheshwari, MD
Disorders Clinic
Assistant Professor
Department of Neurology
Department of Neurology
Baylor College of Medicine
Baylor College of Medicine
Houston, Texas
Houston, Texas
Joohi Jimenez-Shahed, MD
Jacob Mandel, MD
Assistant Professor
Assistant Professor
Associate Director, Neurology Residency Program
Department of Neurology
Director, Deep Brain Stimulation Program
Baylor College of Medicine
Baylor College of Medicine
Houston, Texas
Houston, Texas
 Contributors  ix

Sharyl R. Martini, MD, PhD Joshua J. Rodgers, MD

Assistant Professor Assistant Professor
Department of Neurology Menninger Department of Psychiatry and
Baylor College of Medicine ­Behavioral Sciences
Staff Neurologist Baylor College of Medicine
Michael E. DeBakey VA Medical Houston, Texas
Center
Loren A. Rolak, MD
Houston, Texas
Director, Marshfield Multiple Sclerosis Center
Eli M. Mizrahi, MD The Marshfield Clinic
Chair, Department of Neurology Clinical Adjunct Professor
Chair Department of Neurology
Department of Neurology University of Wisconsin School of Medicine and
Professor Public Health;
Departments of Neurology and Pediatrics Adjunct Professor
James A. Quigley Endowed Chair in Pediatric Department of Neurology
Neurology Baylor College of Medicine
Baylor College of Medicine Houston, Texas
Houston, Texas
Rohini Samudralwar, MD
Paolo Moretti, MD Resident
Assistant Professor Department of Neurology
Department of Neurology and Molecular and Baylor College of Medicine
Human Genetics Houston, Texas
Baylor College of Medicine;
Paul E. Schulz, MD
Staff Neurologist
Professor
Michael E. DeBakey VA Medical Center
Department of Neurology
Houston, Texas
Director, Memory Disorders and Dementia Clinic
Dennis Mosier, MD, PhD Director, Neuropsychiatry and Behavioral Neurology
Clinical Associate Professor Fellowship
Department of Neurology The University of Texas Health Science Center at
Baylor College of Medicine Houston
Staff Neurologist Houston, Texas
Michael E. DeBakey VA Medical
Center Lydia Sharp, MD
Instructor
Houston, Texas
Department of Neurology
Dona K. Murphey, PhD, MD Baylor College of Medicine
Fellow, Clinical Neurophysiology Houston, Texas
Department of Neurology
Ericka P. Simpson, MD
Baylor College of Medicine
Associate Professor
Houston, Texas
Department of Neurology
James Owens, MD, PhD Houston Methodist Hospital
Associate Professor Houston, Texas;
Departments of Neurology and Associate Professor
Pediatrics Department of Neurology
University of Washington Weill-Cornell Medical College
Seattle, Washington New York, New York
Amee A. Patel, DO Jose I. Suarez, MD
Assistant Professor Professor
Department of Pediatrics Department of Neurology
Baylor College of Medicine Head, Section of Vascular Neurology and
The Children’s Sleep Center Neurocritical Care
Texas Children’s Hospital Baylor College of Medicine
Houston, Texas Houston, Texas
 x Contributors

Colin Van Hook, MD, MPH Angus A. Wilfong, MD

Fellow, Clinical Neurophysiology Professor
Department of Neurology Departments of Pediatrics and Neurology
Vanderbilt University Baylor College of Medicine;
Nashville, Tennessee Director
Comprehensive Epilepsy Program
Benjamin L. Weinstein, MD Blue Bird Circle Clinic for Pediatric Neurology
Associate Professor Texas Children’s Hospital
Menninger Department of Psychiatry and Houston, Texas
Behavioral Sciences
Baylor College of Medicine Randall Wright, MD
Houston, Texas Mischer Neuroscience Associates;
Associate Professor of Neurology
Subhashie Wijemanne, MD Department of Neurosurgery
Assistant Professor The University of Texas School of Medicine at
Department of Neurology Houston
The University of Texas Health Science Center at Houston, Texas
San Antonio
San Antonio, Texas
 PREFACE

The first edition of Neurology Secrets was published over 20 years ago. The subsequent editions have
tracked the dramatic advances in the field of neurology. In this 6th edition, each chapter has been revised
and updated to reflect the current state of the art and science of the topic. New chapters have been added
that emphasize the multidisciplinary nature of the practice of neurology. The purpose of this book is to
focus on the fundamental issues of the field of neurology. This edition follows the now familiar N­ eurology
Secrets format with each chapter organized as a series of questions and answers. Key points are high-
lighted in each chapter. The chapters are developed to form a basis for further discussion with directed
references and reading for more in-depth review. Each chapter is designed to provide the reader with a
concise and accurate review which crystallizes the essential features of each topic.
We are indebted to the contributors of Neurology Secrets 6th edition. Most of these authors have
some academic relationship to Baylor College of Medicine in Houston, Texas, either as current or former
faculty members or trainees. This is a tradition begun with the first edition and has continued through the
subsequent editions. Some are new to this edition; however, there are a number who have been contribut-
ing from the start.
The founding editor of Neurology Secrets, Loren A. Rolak, MD, who also edited the subsequent editions,
relinquished that role for this 6th edition—although he continues as a contributor. He began this effort
within the context of his role as member of the faculty and Neurology Residency Program Director, Depart-
ment of Neurology, at Baylor College of Medicine in Houston, Texas. Throughout the years, Dr Rolak, who is
now at Marshfield Clinic in Marshfield, Wisconsin, has been committed to medical education. The previous
editions of Neurology Secrets are, in part, evidence of his dedication and skill as an educator, and we trust
that the 6th edition does justice to the rich tradition he established.

Joseph S. Kass, MD, JD

Eli M. Mizrahi, MD
DEDICATION

To Loren A. Rolak, MD, founding editor of Neurology Secrets, expert and

compassionate clinician, and skilled and dedicated medical educator.
Joseph S. Kass, MD, JD
Eli M. Mizrahi, MD
TOP 100 SECRETS
These secrets are 100 of the top board alerts. They summarize the concepts,
principles, and most salient details of neurology.

1. Long-term potentiation is the synaptic mechanism of learning and memory.

2. If the facial nerve is damaged (such as from Bell’s palsy), the entire side of the face is
weak. If the cortical input to the facial nerve is damaged (such as from a stroke), only
the lower half of the face will be weak.
3. A dilated or “blown” pupil implies compression of the III nerve.
4. Noncommunicating hydrocephalus is a medical emergency because the obstructed
cerebrospinal fluid (CSF) will cause the intracranial pressure to rise.
5. To distinguish between a common peroneal neuropathy at the popliteal fossa and a L5
radiculopathy, examine for weakness in hip abduction and ankle inversion. Weakness in
these muscles indicates L5 radiculopathy.
6. The first step in treating patients with neurologic disease is to localize the lesion.
7. Peripheral neuropathies often produce distal weakness, atrophy, fasciculations, sensory
loss, and pain.
8. Spinal cord diseases often produce pyramidal tract deficits, sphincter problems, and a
sensory level.
9. Brainstem lesions often produce cranial nerve deficits accompanied by weakness or
numbness on the contralateral body.
10. Myopathies usually cause proximal symmetric weakness, with or without other
symptoms.
11. Myotonic dystrophy is the most common muscular dystrophy in adults.
12. Myasthenia gravis typically presents with subacute to chronic, fatigable, proximal arm
and leg weakness, ptosis, and diplopia.
13. Myasthenia gravis can cause rapid onset neuromuscular respiratory failure, which can
be fatal and is therefore a neurological emergency.
14. Lambert–Eaton myasthenic syndrome is associated with cancer (typically small-cell
carcinoma of the lung) in approximately 60% of cases, and may be the first manifesta-
tion of malignancy.
15. The most common causes of peripheral neuropathy are diabetes and alcoholism.
16. The most often overlooked cause of peripheral neuropathy is genetic.
17. The spinal fluid of patients with Guillain–Barré syndrome has high protein but normal
cell counts (cytoalbuminologic dissociation).
18. Compression of the C6 nerve root causes radicular pain in the lateral side of the fore-
arm and thumb, C7 compression causes pain in the index and middle fingers, and C8
compression causes symptoms in the fourth and fifth fingers.
19. Ninety-five percent of lumbar disc herniations occur at the L4/5 or L5/S1 disc
spaces.
20. The dermatome for the nipple line is at T4 and the umbilicus is at T10.

xiii
xiv Top 100 Secrets

21. The first seven cervical nerves exit above the vertebral body with the eight exiting below
C7, and the remainder of the spinal roots exit below their corresponding vertebral body.
22. Transverse myelitis is an inflammatory process that is localized over several segments
of the cord functionally transects the cord.
23. The most common metastatic tumors to the spinal cord are breast, lung, gastrointesti-
nal tract, lymphoma, myeloma, and prostate.
24. A unilateral lesion within the brainstem often causes “crossed syndromes,” in which
ipsilateral dysfunction of one or more cranial nerves is accompanied by hemiparesis
and/or hemisensory loss on the contralateral body.
25. Symptoms of brainstem ischemia are usually multiple, and isolated findings (such as
vertigo or diplopia) are more often caused by peripheral lesions affecting individual
cranial nerves.
26. Brainstem glioma is the most frequent brainstem neoplasm. Other brainstem neo-
plasms include ependymomas that occur in the fourth ventricle and metastatic lesions
that may originate from malignant melanomas or carcinomas of the lung and breast.
27. Ménière’s disease presents with the symptomatic triad of episodic vertigo, tinnitus, and
hearing loss. It is caused by an increased amount of endolymph in the scala media.
Pathologically, hair cells degenerate in the macula and vestibule.
28. Central pontine myelinolysis (osmotic demyelination syndrome) occurs primarily in patients
suffering from malnutrition or alcoholism complicated by hyponatremia. Rapid correction
of the hyponatremia has been implicated as a cause of the pathologic abnormality.
29. Cerebellar strokes and hemorrhage may result in a neurological/neurosurgical emer-
gency by causing obstructive hydrocephalus.
30. Episodic ataxia type 2 is caused by mutations in the same gene (CACN1A4) as familial
hemiplegic migraine and spinocerebellar ataxia type 6.
31. Differential diagnosis of cerebellar/ataxic conditions can vary by age: (1) adults are more
likely to have autosomal dominant spinocerebellar ataxias, degenerative forms of ataxia,
extra-axial tumors, paraneoplastic syndromes, and vascular insults to the cerebellum;
(2) pediatric patients are more likely to manifest with autosomal recessive cerebellar
ataxias, intra-axial cerebellar tumors, infections, or congenital/developmental
abnormalities.
32. Levodopa remains the most effective therapy for Parkinson’s disease, but management
of levodopa-related complications continues to be a challenging probelm that often
requires treatment with multiple medications and deep brain stimulation.
33. Essential tremor is a familial disorder, but the genes responsible for this alcohol-­
responsive action tremor have not yet been identified.
34. Cardinal symptoms of autonomic insufficiency include orthostatic hypotension, bowel
and bladder dysfunction, impotence, and sweating abnormalities.
35. Autonomic failure can be seen in the setting of systemic peripheral neuropathies, the
most common being diabetic neuropathy, or can be seen without involvement of the
sensorimotor neurons, such as pure autonomic failure. Some dysautonomias have
autoimmune etiology.
36. The diagnosis of multiple sclerosis requires lesions disseminated in time and in space:
two separate symptoms at two separate times.
37. Faulty interpretation of magnetic resonance imaging is the most common error leading
to the misdiagnosis of multiple sclerosis.
38. No treatment has yet been proven to alter the level of long-term disability in multiple
sclerosis.
 Top 100 Secrets  xv

39. Dementia is a category, not a diagnosis, and Alzheimer’s disease is the most common
form of dementia.
40. Most causes of dementia are treatable even if not curable.
41. Frontotemporal dementia (FTD) can present with either behavioral symptoms or primary
progressive aphasia.
42. Progressive supranuclear palsy and corticobasal syndrome are parkinsonian disorders
associated with dementia and tau aggregation.
43. Cerebral amyloid angiopathy can cause dementia and has manifestations apart from
classic lobar hemorrhage.
44. Ten to 15% of patients with amnestic mild cognitive impairment progress to develop-
ment of Alzheimer’s disease each year.
45. Repetitive mild traumatic brain injury can lead to a syndrome of progressive cognitive
decline, behavioral and mood changes, and motor/parkinsonian symptoms known as
chronic traumatic encephalopathy. The diagnosis can only be confirmed postmortem
by identification of hyperphosphorylated tau protein deposits in the sulci.
46. The diagnosis of posttraumatic stress disorder requires exposure to trauma with
development of symptoms (intrusive thoughts, re-experiencing, avoidance, nega-
tive alterations in cognition and mood, and marked alterations in arousal and
reactivity) lasting >1 month and causing significant distress and/or impairment in
functioning.
47. A
 n acute onset of cognitive decline with fluctuations in orientation and level of alertness
is the hallmark of delirium rather than indicative of dementia.
48. The main clinical feature of stroke is sudden onset of a focal neurological deficit.
49. The only Food and Drug Administration-approved treatment for acute ischemic stroke
is intravenous (IV) tissue plasminogen activator, administered within 3 hours of the time
the patient was last seen normal.
50. Hemorrhagic strokes often present with a diminished level of consciousness; ischemic
strokes rarely do.
51. A depressed level of consciousness (Glascow Coma Scale <8) is the greatest risk fac-
tor for airway obstruction and aspiration.
52. Think of cerebral amyloid angiopathy as the most likely cause of spontaneous lobar
intracerebral hemorrhage in patients age >55 years.
53. Nimodipine in aneurismal subarachnoid hemorrhage is neuroprotective as it improves
outcome but has not shown to reduce vasospasm.
54. Early decompressive hemicraniectomy after large hemispheric ischemic stroke within
48 hours for patients <60 years of age improves survival and functional outcome.
55. Steroids are not recommended in Guillain–Barré syndrome.
56. Treat convulsive status epilepticus early and aggressively with benzodiazepines
57. Brain death is a clinical diagnosis.
58. Central nervous system (CNS) tumors are classified into four grades according to the
World Health Organization (WHO) grading system.
59. Glioblastoma (Grade IV astrocytoma) is the most common and malignant primary brain
tumor in adults.
60. Brain metastases are the most common intracranial tumors in adults, occurring nearly
10 times more often than primary brain tumors.
xvi Top 100 Secrets

61. Intrathecal methotrexate use has been associated with aseptic meningitis, transverse
myelopathy, encephalopathy, and leukoencephalopathy.
62. Do not prescribe opioid or butalbital-containing medications as first-line treatment for
recurrent headache disorders.
63. The risk of postdural puncture headache can be greatly reduced by use of an atrau-
matic needle. Bedrest following the procedure is not preventive.
64. Headache or neck pain is the only symptom of cervical artery dissection in 8% of
­individuals.
65. A seizure is a single event, while epilepsy refers to (1) recurrent unprovoked seizures,
(2) a single seizure with a high risk for recurrent seizures, or (3) recurrent reflex seizures.
66. Epilepsy surgery has the best outcomes in patients with a structural lesion (80%) or
those with temporal lobe epilepsy (60% to 70%).
67. Medication-refractory (pharmacoresistant) epilepsy is diagnosed when two or three
antiepileptic medications at appropriate doses fail to control seizures.
68. Sleep problems occur frequently in individuals with uncomplicated medical histories
(20% to 40%) and very frequently in children and adults with complicated medical
histories (40% to 80%).
69. All patients with a stroke must have screening for cardiovascular disease.
70. Patients with uremia often develop a metabolic encephalopathy with signs of neuronal
depression such as lethargy as well as excitation such as myoclonus.
71. Most patients with Cushing’s disease have frank weakness with myopathic findings on
electromyography.
72. Decompensated hypothyroidism can cause myxedema coma with mortality rates as
high as 25% to 60%.
73. Cerebrovascular ischemic events occur 13 times more frequently in pregnant women
than in age-matched nonpregnant women.
74. Headache, jaw claudication, and constitutional symptoms compose the triad of clinical
symptoms often found in temporal arteritis.
75. Patients suspected of having bacterial meningitis should receive adjunctive dexa-
methasone along with empiric antibiotics. The dosing regimen is dexamethasone 4 mg
IV every 6 hours for 4 days with the first dose given either 30 minutes prior to the first
dose of antibiotics or concomitantly with the first dose of antibiotics. If the CSF cultures
indicate the pathogen is not Streptococcus pneumoniae, then dexamethasone may be
discontinued.
76. Herpes simplex virus-1 (HSV-1) encephalitis should be considered in a patient present-
ing with fever, behavioral changes, and/or seizures and should be treated empirically
with IV acyclovir. HSV polymerase chain reaction in CSF can be negative in the first few
days of infection, necessitating repeating lumbar puncture 3 or more days after
infection onset.
77. The differential diagnosis for a ring-enhancing lesion in a person with Acquired Immu-
nodeficiency Syndrome includes most commonly Toxoplasma gondii and primary CNS
lymphoma but also includes tuberculoma, cryptococcoma, histoplasmoma, and other
fungal infections, bacterial brain abscess, metastatic disease, and primary brain tumor.
78. Neurocysticercosis is the most common infectious cause of epilepsy with treatment
strategies varying depending on cyst life cycle stage and location within the nervous
system.
79. The possibility of multiple mutation mechanisms should be considered in ordering and
interpreting diagnostic test results for many neurogenetic diseases.
 Top 100 Secrets  xvii

80. The expanding use of genome-level technologies is revealing a previously unsuspected

degree of phenotypic variability in many neurogenetic diseases.
81. Causes of intrauterine infection include toxoplasmosis and other agents, such as
rubella, cytomegalovirus, and herpes simplex virus.
82. Simple febrile seizures are generalized tonic or tonic–clonic seizures that typically occur
between 3 months and 5 years of age. They are often accompanied by a fever greater
than 38° C not associated with a CNS infection, and last less than 15 minutes with no
focal features and no recurrence within 24 hours. No postictal neurologic abnormalities
typically occur.
83. Headaches concerning for an intracranial mass include a recent onset of headaches
or change in character of chronic headaches, headaches that awaken the patient
from sleep or are present on awakening in the morning, and headaches in association
with altered mental status, vomiting, constriction of visual fields, or focal neurologic
deficits.
84. Psychiatric and neurologic disorders are highly comorbid and bidirectionally related,
and many neurologic disorders may present first with psychiatric symptoms; neurologi-
cal and medical etiologies should be considered for patients presenting with psychiatric
symptoms.
85. Psychiatric disorders are debilitating but are highly treatable; all patients should be
screened for common disorders and suicidality.
86. Somatic symptom disorders (including functional neurologic symptom disorder) are
commonly comorbid with focal neurologic disorders and can improve with close follow-
up with a single provider and psychotherapy.
87. The cardinal feature of delirium is impaired environmental awareness and ability to
direct, sustain, or appropriately shift attention. No other psychiatric disorder can be
diagnosed in the context of delirium.
88. Acute and chronic disorders of the vestibular system are characterized by disturbances
in the behaviors mediated by the vestibular system, e.g., blurred vision, vertigo, im-
paired balance, nausea, and temporary changes in cardiovascular measures.
89. Most vestibular disorders can be treated. Some disorders are best treated with exercise
and other aspects of rehabilitation; other disorders are best treated with medication
and/or surgery.
90. The normal adult electroencephalogram (EEG), relaxed with eyes closed, is character-
ized by 9 to 11 cycles per second activity in the back of the brain (occipital lobes) called
the alpha rhythm.
91. Periodic lateralizing epileptiform discharges on an EEG imply an acute, large lesion
involving one hemisphere, such as a stroke or focal encephalitis.
92. The generalized three per second spike and wave pattern on an EEG is usually seen in
patients with absence seizures.
93. The finding on an EEG that is most suggestive of focal epilepsy is a very brief (less than
70 ms) transient deflection called a spike.
94. The most common compression neuropathies are carpal tunnel syndrome (median
nerve compression at the wrist) and cubital tunnel syndrome (ulnar nerve compression
at the elbow).
95. The earliest conduction abnormalities in Guillain–Barré syndrome (acute inflammatory
demyelinating polyneuropathy) are absent H reflexes.
96. Motor conduction abnormalities in chronic inflammatory demyelinating polyneuropathy
are abnormal F waves, distal latencies delay, conduction block with dispersion of distal
potential, and motor velocities >20% of normal.
 xviii Top 100 Secrets

97. A patient with parkinsonism, ataxia, dysautonomia, and alpha-synuclein-positive glial

cytoplasmic inclusions at autopsy has multiple system atrophy.
98. C9orf72 hexanucleotide repeat expansion is the most frequent genetic alteration
underlying familial amyotrophic lateral sclerosis (ALS) including ALS with frontotemporal
dementia (ALS/FTD).
99. An infiltrative (diffuse) glioma with astrocytic cytology, mitotic activity, microvascular
proliferation, and necrosis is a WHO Grade IV glioblastoma.
100. Tuberculous meningitis and neurosarcoidosis both have a predilection for the basilar
meninges.
 CHAPTER 1
CLINICAL NEUROSCIENCE
Dona K. Murphey, Dennis Mosier

INTRODUCTION
1. Why is it important to understand the cellular, molecular, and genetic mechanisms
that govern normal and abnormal nervous system function?
• To select the most appropriate diagnostic tests and interpretation of test results
• To optimize drug therapy by mechanisms of action, interactions, side effect profiles
• To educate patients and their families about their diseases and prognoses
• To aid in the critical review of rational drug design and clinical trials
2. What cellular alterations lead to neurological disease or affect management/
prognosis?
• Loss of neurons (e.g., neurodegenerative conditions)
• Injury of axons (e.g., traumatic brain injury)
• Reorganization of synaptic connections (e.g., deafferentation pain)
• Disruption of the blood–brain barrier (BBB) (e.g., stroke)
3. What molecular alterations lead to neurological disease or affect management/
prognosis?
• Excitation–contraction uncoupling (e.g., channelopathies)
• Dysfunctional volume regulation (e.g., cytotoxic edema)
• Altered membrane excitability (e.g., epilepsy)
• Conduction abnormalities (e.g., demyelinating conditions)
• Oxidative stress (e.g., mitochondrial disorders)
• Autoimmune attack of receptors (e.g., myasthenia gravis)
4. Why is the presence of genetic alterations relevant in neurologic diseases?
• They may reveal propensity for decreased drug efficacy.
• They may reveal susceptibility to drug toxicity.
• They may provide presymptomatic diagnoses.
• They may be prognostic.

CELLULAR ANATOMY
5. What are the major subcellular compartments of the canonical neuron (Fig. 1-1)?
• Soma—body of the neuron
• Dendrites—processes that emanate from the soma and subserve synaptic connections
• Axon—projection from the soma that terminates on postsynaptic partners
• Nodes of Ranvier—area between myelinated axonal segments densely populated by voltage-gated
Na+ channels that regenerate action potentials
• Myelin—a sheath comprised primarily of lipids that electrically insulates the axon and allows for
salutatory conduction at unsheathed nodes
6. How are the major subcellular compartments of the canonical neuron affected
in disease?
• Soma—site of pathological inclusions in neurodegenerative diseases
• Dendrites—loss of dendritic spines in autism
• Axon—susceptibility to shearing in traumatic brain injury, loss in Wallerian degeneration
• Nodes of Ranvier—susceptibility to dysfunction in channelopathies
• Myelin—loss in demyelinating diseases
7. What are the major cell types in the nervous system?
• Neurons—These comprise a diverse collection of cells that marry afferent input to internal brain
states to produce perception and behavior.
1
 2  Chapter 1 Clinical Neuroscience

Dendrite Soma Synapse

Myelin

Node of Ranvier
Axon
Figure 1-1. The canonical neuron consists of a soma, dendrites, and axon ensheathed in myelin and punctuated by nodes
of Ranvier.

• Glia—These comprise a diverse collection of cells that support neuronal function and are believed
more recently to participate actively in synaptic formation and function.
8. List the major types of neurons in the nervous system.
• Excitatory glutamatergic pyramidal neurons—80% of neocortical neurons, long range
• Inhibitory gamma aminobutyric acid-ergic (GABAergic) interneurons—20% of neocortical neurons,
mostly local
• Neuromodulatory neurons—neurons expressing acetylcholine, dopamine, serotonin, norepineph-
rine, epinephrine, histamine, and neuropeptides such as orexin/hypocretin, somatostatin that often
also corelease the classical neurotransmitters glutamate or GABA.
9. List the major types of glia in the nervous system.
• Astrocytes—establish the blood–brain barrier, flux ions, repair and form scars in injury
• Oligodendroglia and Schwann cells—form myelin in brain/spinal cord and periphery
• Ependymal cells—neuroepithelial cells lining the ventricles, choroid, spinal cord central canal, form
cerebrospinal fluid, nonrenewing stem cell pool producing neurons in adult central nervous system
(CNS) injury
• Microglia—resident inflammatory phagocytes in infection, degeneration, demyelination

K EY POIN T S: C E L L T YP E -S P E C I F I C DY S F UN C T I O N O R L OS S IN
N EUR OL OGIC D IS E ASE
1. Excitatory cortical neurons are preferentially lost in Alzheimer’s disease (AD).
2. Inhibitory cortical neurons are preferentially affected in epilepsy.
3. Neuromodulatory neurons are preferentially lost in AD (acetylcholine), Parkinson’s disease (PD)
(dopamine), and narcolepsy (orexin/hypocretin).
4. Astrocytes are preferentially affected in Alexander’s disease and neuromyelitis optica.
5. Oligodendroglia and Schwann cells are preferentially affected in multiple sclerosis and ­
Charcot–Marie–Tooth disease, respectively.

10. What are the cellular constituents of the blood-brain barrier (BBB) (Fig. 1-2)?
• Capillary endothelial cells—linked by tight junctions and expressing ion channels, carrier-mediated
and lipid-soluble transporters, efflux pumps
• Pericapillary astrocytes—end-feet adjacent to capillaries
• A similar system exists for the choroidal epithelium at the blood–cerebrospinal fluid barrier (BCSFB)
(Fig. 1-3).

11. Which regions of the brain lack a significant BBB?

• Area postrema
• Median eminence of the hypothalamus
• Neurohypophysis
12. Under what conditions is the integrity of the BBB compromised?
• Autoimmune inflammation or infection (e.g., multiple sclerosis)
• Osmotic injury (e.g., rapid correction of hyponatremia)
• Vascular overpressure (e.g., malignant hypertension, reperfusion after ischemia)
 Chapter 1 Clinical Neuroscience  3

Basement membrane

Tight junctions

Endothelial
cells
Astrocyte

Transporters

Figure 1-2. The blood–brain barrier (BBB) consists of endothelial cells bound by tight junctions and surrounded by
­astrocytic and pericytic (not pictured) processes.

Tight junctions

Choroidal epithelium

Fenestrated endothelial cells

Figure 1-3. At the blood–cerebrospinal fluid barrier, choroidal cells are bound by tight junctions.

• Neovascularization (e.g., cancer)

• Neuronal hyperexcitability (e.g., seizures)
13. What are the imaging features of BBB compromise?
• On a noncontrast computerized tomography (CT) scan, leakage of blood cells across the BBB or
BCSFB is radiodense. Leakage of extracellular fluid (vasogenic edema) is radiolucent (Fig. 1-4).
• On a T1 contrast magnetic resonance image (MRI), leakage of blood cells across the BBB or BCSFB
is hyperintense. On a T2 gradient recalled echo (GRE) sequence, leakage of blood cells across the
BBB or BCSFB is hypointense. On a T2 fluid-attenuated inversion recovery (FLAIR) MRI, vasogenic
edema is hyperintense (Fig. 1-5).
 4  Chapter 1 Clinical Neuroscience

Figure 1-4. Computerized tomography (CT) scan of the brain without contrast reveals radiodense blood surrounded by
radiolucent vasogenic edema.

Figure 1-5. Magnetic resonance imaging (MRI) of the brain. T1 contrast reveals hyperintense enhancement of a tumor
from leakage of blood across a compromised BBB. T2 gradient recalled echo (GRE) reveals hypointense hemosiderin
deposition of blood. T2 fluid-attenuated inversion recovery (FLAIR) reveals hyperintense edema.

14. What cellular processes make the brain plastic?

• Long-term potentiation—long-lasting increases in postsynaptic responsiveness to weak temporally
coincident stimulation of separate inputs
• Neuromodulation by peptidergic cells—a critical dependence of synaptogenesis on the local circuit
signaling of neuromodulatory cells and their cognate receptors
• Astrocytic secretion of synaptogenic molecules—direct selective control of synapse formation and
elimination during development and in the mature adult brain
15. What features comprise necrotic neuronal death?
• Occurs secondary to insult (e.g., trauma, stroke, toxins)
• Typically proinflammatory
• Cell swelling, disruption of organelles, compromise of membrane integrity, cell lysis
16. What features comprise apoptotic neuronal death?
• Programmed “cellular suicide” (e.g., irradiated tumor cells, glucocorticoids, cytotoxic T
lymphocytes, and growth factor withdrawal)
• Not typically proinflammatory
• Chromatin condensation, DNA fragmentation, nuclear membrane loss, cell membrane blebbing,
cell fragmentation into easily phagocytosed “apoptotic bodies”

MOLECULAR NEUROBIOLOGY
17. What ionic currents support action potential generation and propagation?
• Depolarizing phase—Na+ currents
• Repolarizing phase:
Inactivation of Na+ currents (accounts for refractory period of action potentials)
• 
Activation of K+ currents (accounts for membrane hyperpolarization)
• 
 Chapter 1 Clinical Neuroscience  5

18. How are signals transmitted across a chemical synapse?

• Depolarization of the presynaptic neuron by an arriving action potential
• Activation of voltage-dependent calcium (Ca2+) channels, focal entry of intracellular Ca2+
• Synchronized, quantal vesicular release of neurotransmitter from the presynaptic terminal
• Diffusion of neurotransmitter across the synaptic cleft
• Binding of neurotransmitter to specific receptors on the postsynaptic membrane
• Receptor-mediated ion channel mediation of inhibitory (GABA) or excitatory (glutamate)
­postsynaptic potentials
• Postsynaptic action potential initiation if the postsynaptic potentials sum to reach activation
threshold
19. What are ion channels?
Ion channels, formed from membrane-spanning proteins, allow the selective and rapid flux of ions
across cell membranes. Channels respond to (are gated by) specific stimuli.
20. What are the types of ion channels?
• Voltage-gated channels—activated by changes in the transmembrane voltage gradient
• Ligand-gated channels—activated by binding of chemical agonists
• Mechanical stretch or pressure
21. How do ion channelopathies present clinically?
• Episodic clinical symptoms
• Typically normal interictal function
• Specific triggers for attacks (e.g., exercise, temperature changes, startle responses, drugs)
22. What are some examples of sodium channelopathies?
• Generalized epilepsy with febrile seizures plus, a syndromic group associated with febrile seizures
that may extend beyond the classic first 6 years of life, may occur without fever, and may be
­associated with familial epilepsies characterized by other seizure types.
• Dravet syndrome—an early-onset epileptic encephalopathy
• Hyperkalemic periodic paralysis—a generalized episodic flaccid weakness
23. What are some examples of potassium channelopathies?
• Ataxia–myokymia syndrome (EA-1)—myokymia and episodic ataxia
• Long QT syndromes—syncopal seizures and sudden cardiac death
• Benign familial neonatal convulsions—clustered seizures at birth with spontaneous remission by
12 months and normal development, increased risk of adult epilepsy
• Isaacs’ syndrome (neuromyotonia)—an antibody-mediated autoimmune attack on K+ channels in
motor nerves
24. What are some examples of calcium channelopathies?
• Timothy syndrome—multiorgan dysfunction, including lethal arrhythmias, immune deficiency,
intermittent hypoglycemia, cognitive abnormalities, and autism
• Familial hemiplegic migraine and episodic ataxia (EA-2)—ataxia precipitated by physical or emo-
tional stress, gene CACNA1A
• Lambert–Eaton syndrome—an antibody-mediated autoimmune attack on P/Q type voltage-gated
Ca channels in the presynaptic neuron at the neuromuscular junction
• Hypokalemic periodic muscle paralysis—mutation in gene coding for the skeletal voltage-gated
Ca2+ channel

K EY POIN T S: C E RTAIN F EAT UR ES A R E C O M M O N A M ONG MOS T AL L

N EUR OT RAN S M IT T E RS
1. Presence of the substance within neuron terminals
2. Release of the substance with neuronal stimulation
3. Exogenous application of the substance at physiologic concentrations to the postsynaptic
­membrane reproduces the effects of stimulation of the presynaptic neuron.
4. A local mechanism exists for inactivation of the substance (e.g., enzymatic degradation, uptake
into nerve terminals or glia).
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 As they walked, Charles expressed his joy that they were going to
see the inside of a mill, which was what he had long wished to do.
“And, pray,” inquired he, “what is it like?”
Mr. Mansfield. That you will see when you get to it: in the mean
time, Arthur, examine this wheat. I showed you barley and rye in the
morning.
“There is no beard to this,” said Arthur.
Mr. Mansfield. No; and the ear is heavier and larger. Gather one,
and count the number of grains it contains.
Charles pulled violently, and drew up a root that had seven stalks
growing from it.
Farm House Page 64.
 The Mill.
London. Published by W. Darton Jun. Oct. 5, 1815.

“Hold, you wasteful little fellow!” cried his grandpapa. “I did not tell
you to root up my field at one stroke. Let me see, however. Observe
what a wonderful increase here is. These seven stalks have all
sprung from one single grain, and each ear contains, perhaps,
twenty grains; which gives us in all a hundred and forty grains
instead of one.”
Arthur. That is astonishing, indeed! So there always grows a
hundred and forty times as much wheat as is sown?
Mr. Mansfield. No, no, I did not say that. In this instance it is so;
and sometimes it may even happen to produce more; but a great
deal of seed rots in the ground, without ever growing at all: of what
does come up, some is spoilt before it is ripe, and the ears that come
to perfection do not all yield so well as these. I believe, therefore,
that taking the kingdom throughout, we only gather about eight times
the quantity we sow.
Arthur. How long is wheat growing, pray, sir?
Mr. Mansfield. Nine or ten months generally. No sooner is the
harvest of one year got in, than we begin to prepare for that of the
ensuing year. We plough the land, and sow it again immediately.
Some seed, indeed, is not sown before the spring, but that never
produces quite such good crops.
Charles. What is the use of ploughing, grandpapa?
Mr. Mansfield. To break up the earth, which would otherwise get so
hard that no corn could grow in it. When a field has been ploughed, a
man walks over it, and scatters the seed all over the field. Then it is
raked in by an instrument full of great iron teeth, called a harrow.
Care must afterwards be taken to keep it free from weeds, but
besides that nothing more can be done. It is left for the rain to water,
and the sun to ripen it.
Charles. And when it is quite ripe, then the harvest comes, does it
not, sir?
Mr. Mansfield. Yes. Then the reapers go into the field, and cut
down the corn with their sickles. They tie it up in bundles, which are
called sheaves, when it is carried into barns, and thrashed out for
use.
 As they were conversing in this manner they arrived at the mill;
and when Mr. Mansfield had given his orders, he asked leave to lead
his grandchildren over it. He then explained to them, how the sails,
being turned round by the wind, were the occasion of turning
different wheels in the inside of the building. He next pointed out to
them two large flat stones, shut up in a kind of box. “You may see,”
said he, “that all the corn is made to pass between these stones. The
understone is fixed; but the upper one turns round, and presses so
heavily upon it, as to bruise and grind the corn to powder.”
“I understand you, grandpapa,” returned Charles. “And is that all
that is done here?”
Mr. Mansfield. Not all, Charles; for the corn, though ground into
meal, wants sifting. To do that, there is a contrivance called a
boulting engine, and you may look at it if you step this way.
Mr. Mansfield then opened a little door in the large wooden box, or
bin, that contained the engine; when a quantity of fine flour flew out
into their faces, and powdered them all over. The boulter was made
of frame-work, five or six feet long, round which a canvas was tightly
strained. “Now,” said Mr. Mansfield, “the meal is put into this boulting
machine, which turns round, you see, very fast when the mill is at
work. The quickness of its motion causes the fine flour to fly off
through the canvas; but the coarse and husky part, which is bran,
not being able to do that, falls to the bottom by itself. The use of
shutting it up in this box, is to prevent the flour from being scattered
over the mill.”
The Bensons and their grandfather remained at the mill till they
had thoroughly examined every part of it. They received much
pleasure from seeing the different wheels and contrivances, and
were diverted to find, when they came away, that they were so
covered with flour as to look almost as white as millers.
As they were returning home, Arthur observed, that having first
seen the wheat growing, and afterwards ground, they only wanted
now to know how flour was made into bread, to understand the
whole process from beginning to end.
 Mr. Mansfield replied, that he could easily explain that. The flour
was mixed with a proper quantity of water, and a little yeast put in to
make it rise. “This,” said he, “is well kneaded together, and then it is
put into an oven and baked.”
“But what is yeast?” inquired Charles.
Mr. Mansfield. A scum that rises on the top of new beer.
Arthur. Have not I, sir, seen to-day, some of all the different kinds
of corn that grow here?
Mr. Mansfield. I do not recollect our having met with oats. They do
not grow in one compact ear like the rest, for every grain has a
separate little foot-stalk to itself. In this part of the country they are
chiefly given to horses; but in Scotland, and the north of England,
oatmeal cakes are frequently eaten instead of bread.
“And now,” continued he, “I am not sorry to find myself near home.
You, Arthur, may likewise be glad to rest yourself, for you have been
stumping about almost all day.”
The boys declared they were not at all tired, and thanked their
grandpapa for the pleasure he had procured them.
 CHAPTER IX.
Poultry.

“Grandmamma, pray where are you going?” asked Charles one

morning, on seeing his grandmother walk out at the garden door.
“To feed my chickens, my love,” returned she.
“Then I will go with you, if I may,” said Charles. “And so will I,” said
Arthur: and he threw down his peg-top in a corner.
“My speckled hen,” said Mrs. Mansfield, “came off her nest,
yesterday, with a fine brood of chickens.”
Arthur. That is the one, is not it, ma’am, that has been sitting so
close ever since we came?
Grandmamma. Yes, and for a fortnight before, which makes in all
three weeks; the time hens always sit on their eggs.
Charles. I think they must be tired of keeping still so long.
Grandmamma. I believe, Charles, you would be tired of such
confinement; but birds do not seem to mind it at all. Though so active
at other times; when they have laid their eggs, they are quite
contented to sit still till the young ones are hatched.
Arthur. Do all birds sit for three weeks, grandmamma?
Grandmamma. No: ducks and geese sit for a month; and pigeons
and smaller birds for about a fortnight. Now you may give them some
of these grits, and then you will have the pleasure of seeing them
peck.
Charles. Let me have a handful, if you please. Chick, chick, chick,
chick! come here, poor chickey, and I will give you something to eat.
Dear grandmamma! they will not let me catch them; and look at the
old hen, she is almost ready to fly at me.
 Grandmamma. She is afraid you are going to hurt her chickens,
when she sees you run after them. Hens are often very fierce, if any
one offers to meddle with their young ones.
Charles. Well then, poor things! I will not take you up in my hand,
but I will give you your food on the ground. How pretty they look!
They are the colour of my canary bird.
Arthur. I see the old hen does not eat much herself. She only
pecks about the grits, to show them to her chickens.
Grandmamma. She is a very good mother; so we will give her
some barley. That is the best food for the old ones.
Arthur. See, Charles, how oddly they drink! They turn up their
heads whenever they swallow.
Grandmamma. By that means they let the water trickle down their
throats.
Farm House Page 76.
 The Poultry Yard.
London. Published by W. Darton Jun. Oct. 5, 1815.

Arthur. Then why do not we do so when we drink?

 Grandmamma. Because our throats are not formed the same as
birds’; for we are provided with a muscle, which carries down the
liquor by its motion.
Charles. How the pretty creatures creep under their mother’s
wings! and she puffs her feathers out, and makes herself as large as
she can to cover them all.
Grandmamma. You see how Providence has provided for the
welfare of all his creatures. Whilst they are too young to take care of
themselves, he gives to the parent a strong affection towards them,
and wisdom sufficient to bring them up. The mother, though
generally timorous, and easily frightened, to preserve her young
ones will boldly turn round and face almost any danger. You
observed that the hen forgot her own hunger, when she was
teaching the little chickens to peck; now she covers them with her
wings to keep them from the cold, which, as they are not completely
fledged, would probably kill them if it were not for her care.
Charles. And what will she do when these little things are grown
as big as she is?
Grandmamma. Long before that time she will have cast them off.
Brutes never care for their young when they are able to shift for
themselves.
Arthur. That is very different from us. I do not think my mamma will
cast us off as long as she lives.
Grandmamma. I do not think she will. And I hope, my dear boys,
that in return you will never neglect her. A chicken, you see, could
not be reared unless the hen took care of it; but a child requires
constant attention for many years; and even when grown up, the
fondness of the parents still continues. Surely, then, nothing can
excuse ingratitude and undutifulness towards them.
Arthur. No, grandmamma: so for the future I will always mind what
my papa and mamma say to me; and I will try never to do any thing
that I think they won’t like.
Grandmamma. That is a very good resolution, and I hope you will
keep to it.—— Well, Charles, what do you say to the pigeons? They
are pretty birds, are they not?
Charles. Indeed, ma’am, they are. I was looking at them falling
head over heels, when up in the air.
Grandmamma. Those are called tumblers, that fly in that way.
There are many kinds of pigeons. The white ones out there, with tails
that stand up somewhat like hens’, are called fan-tails.
Arthur. What must we feed the pigeons with?
Grandmamma. You may give them a few tares, if you like; but here
we have no occasion to supply them with much food, for they come
to the barn doors, and pick up the corn that is scattered about.
Charles. Are all those one brood, that sit together on the top of
that cart-house?
Grandmamma. No. Pigeons never lay more than two eggs at one
time; and the pair that are hatched commonly continue mates to one
another all their lives.
Charles. Now let us look at the ducks and geese, if you please.
The pond is nearly covered with them, I see.
Arthur. How very small some of them are! I should have thought
that they were too young to be able to swim.
Grandmamma. Oh, they take to the water as soon as they leave
the egg-shell. They are web-footed, and that enables them to swim.
Charles. What is being web-footed, ma’am?
Grandmamma. Having a skin to fill up all the space between the
toes. When they swim, they paddle, or strike with their broad feet
against the water; which gives them motion onwards.
Arthur. I suppose, grandmamma, you keep poultry for the sake of
eating them!
Grandmamma. You are right. But do you know what is done with
the feathers?
Charles. No.
Arthur. I do, ma’am. Beds and pillows are stuffed with them.
 Grandmamma. And what use is made of the long quill feathers,
that grow in the goose’s wing?
Arthur. I never have been told.
Grandmamma. The quill part, by being cut into shape, is converted
into pens for writing.
Charles. Do only geese furnish pens?
Grandmamma. Some people write with crow-quills: but they are
too small for a bold hand; so that they are very little used. The quills
of the turkey are too hard; and those of ducks and chickens, on the
contrary, are as much too soft. Swan quills, however, make excellent
pens; but they are too scarce to be in general use.
 CHAPTER X.
The Deer.

In Mr. Mansfield’s neighbourhood resided a Mr. Ashley, a

gentleman of large fortune, and of a very obliging disposition. He had
heard that Arthur and Charles Benson were at their grandpapa’s on
a visit, and one day he sent for them to play with his son William, a
little boy nearly of their own age. The Bensons went, and William
took them into the park, where they amused themselves for some
time by the play of trap-ball. The game was at last suddenly broken
off by master Ashley, who threw down the bat upon the ground,
exclaiming that his pretty tame fawn was coming in sight, and that he
must go and stroke it. The Bensons were rather sorry to be
interrupted in their play; but, as they had been early taught, that it
was often necessary to give up their own inclinations to the wishes of
others, they left off with the utmost good humour. “Look at the pretty
creature!” said William Ashley, caressing the fawn. “I wish I had a bit
of bread here: it would eat it out of my hand.”
“I have found a piece of biscuit in the corner of my pocket,” said
Charles. “Here it is. I will feed it, if you please.”
“How old is he?” inquired Arthur.
“Six weeks, or two months, I do not recollect which,” replied
William. “But see, here comes the mother! I thought she could not be
far off, for does never desert their young ones until the end of the
summer.”
The little party was still admiring the fawn, when Mr. Ashley came,
and told them that dinner was almost ready.
“We will come, papa,” said William, “as soon as we have done
counting the spots upon my little fawn’s back.”
“Pray,” inquired Charles, “are all deer spotted?”
 Mr. Ashley. No: their colour is various. Some are of a reddish cast,
some of a deep brown, some white, and others spotted like these.
Observe the herd that are feeding under those trees, and you will
see a great difference amongst them.
“And those, sir,” said Arthur, “have all fine branching horns.”
“They are stags,” returned Mr. Ashley. “The does are the females,
and never have any horns. But perhaps you did not know that even
stags are without, for a part of every year?”
Arthur. Indeed, sir, I did not, and shall be obliged to you if you will
tell me about it.
Mr. Ashley. I will readily. Towards the latter end of February, stags
shed their horns, and soon after fresh ones begin to grow. These are
at first very tender; and the flies, when they are in that state, are
often extremely troublesome to the poor animals. Whilst the horns
are off, they separate from each other, and endeavour to hide
themselves from every other creature, as they are then incapable of
making any defence; but in about three months, when the new ones
have grown to their full size, they associate together again in large
herds.
After these observations, the whole party returned to the house,
and entered the dining-parlour, and their conversation was
interrupted by the inquiries which Mrs. Ashley made, after the health
of Mr. and Mrs. Mansfield: but soon after dinner, William renewed the
subject, by asking his father, whether he might be permitted the next
season to hunt with the stag hounds?
Mr. Ashley replied, that he was much too young; and then turning
to Charles, he asked him if it was his wish also to be a sportsman?
“Indeed, sir,” returned Charles, “I don’t thoroughly understand what
you mean by a sportsman; and I never wished nor even thought
about it.”
“Oh, a sportsman,” said William, “is a man who is fond of hunting
and shooting, and other field sports; and if you knew what they were,
I am sure you would like them, for I think they must be the greatest
pleasures of life.”
 “In what way do they hunt?” inquired Arthur.
William. Why, a stag is turned out into the country, and then, fly
where he will, a whole pack of dogs follow him in full cry; and a
number of gentlemen, mounted on fine horses, gallop after with the
greatest possible speed: and they don’t mind what they come to; for
they leap over gates, and hedges, and ditches, and ride down hills
that are almost as steep as precipices; and at last, when the stag
can run no further, he is caught by the dogs, who kill him; but
sometimes his life is saved, that he may be hunted again another
day.
“What a cruel diversion!” exclaimed Arthur, shocked at the thought
of what the stag must suffer. “I am sure I could never take pleasure
in tormenting a poor animal in that way.”
“We huntsmen do not think of the poor animals,” returned Mr.
Ashley.
“Perhaps not, my dear,” said his wife; “but your want of thought will
not prevent them from feeling pain and terror; and I must own, I am
always surprised when men of humanity join in such barbarous
sports.”
“Do you hunt any thing besides deer?” asked Charles.
Mr. Ashley. Yes, my dear, foxes and hares; the former with fox-
hounds, and the latter with harriers.
Arthur. Pray, sir, are deer ever wild in this country?
Mr. Ashley. Not the kind that we have in our parks, which are
fallow deer; but there is another species, properly called stags,
somewhat larger, differing in a few particulars from those that are
wild in our forests. I do not mean that every body has a right to take
them; for they are accounted the property of the king.
William. I forget, papa, where you told me that fallow deer were
first brought from.
Mr. Ashley. It is said, they came originally from Bengal: but they
were brought here from Norway by King James the first; and
England is now more famous for venison than any other country in
the world.
Charles. Is any use made of the skins, pray, sir?
Mr. Ashley. Yes: the skin both of the buck and doe makes the soft
thick leather, of which gentlemen’s riding breeches and gloves are
made. The horns are very hard and solid, and make excellent
handles for knives and other utensils. They likewise contain a salt,
called salt of hartshorn, from which is extracted spirit of hartshorn.
Mrs. Ashley. How should you like to go to Lapland, and ride over
the snow in a sledge drawn by rein deer?
Charles. By rein deer, ma’am? What, have deer strength enough
to draw carriages?
Mrs. Ashley. The rein deer, my love, is a very extraordinary
creature. It inhabits Lapland, and other cold countries, and answers
to the inhabitants the purpose of most of the animals we have
among us. It serves them instead of a horse, and draws their
sledges with amazing swiftness over the frozen country. It supplies
them too with milk and cheese, instead of the cow; and answers the
purpose of our sheep, in furnishing them with warm covering.
Providence, who has thus formed the rein deer capable of
supplying the numerous wants of man, has provided with wonderful
care for its support. That dreary country, where no grass will grow,
and not any herb which we are accustomed to see, is nevertheless
covered all over with a species of white moss, which serves as food
for it. This is its only pasture; and it gets at it in winter, by scratching
away the snow with its hinder legs.
Arthur. Is the rein deer good to eat, pray, ma’am?
Mr. Ashley. It is. So, when it has lived about sixteen years, the
Laplander generally kills him for the sake of his flesh and his skin.
 CHAPTER XI.
The Pheasantry.

Mr. Ashley’s pleasure-grounds were very extensive; and in one

corner of the park he had inclosed four or five acres of land for
keeping rare and curious birds.
Early in the afternoon, he proposed to his young visitors to take a
walk to the pheasantry, as it was called; an offer which they gladly
accepted. They entered the inclosure by going through a pretty
cottage, where an old man lived who had the care of the birds.
The cottage was deserted; but outside the back door they found
Maddox asleep in a chair, and his little grand-daughter Peggy hard at
work by his side.
Peggy rose, and curtsied in a very pretty manner. Then having
roused the old man, by telling him that ’squire Ashley was come, she
sat down again to her needle. But master William, who was very
fond of her, begged she would leave her work while they stayed, and
look after the birds with them. On a grass plot close to the cottage
there were three or four hens under baskets; and they had each a
little brood belonging to them, which the Bensons mistook for
bantams. But Peggy said they were young pheasants; their eggs
they often set under hens, as they made the best mothers of any
fowl.
“Are these all we are to see?” inquired Arthur.
“No, sir,” replied Peggy; “we have many that are full grown; only
they are now scattered about, and hid among the bushes. I’ll call
them.”
She then fetched out a pan with some of their food, which she
scattered over the grass, calling at the same time in a particular
manner. Presently several pheasants came from their hiding-places.
They were rather shy at first; but by degrees they ventured nearer,
and at last began to eat the food that was thrown to them. The boys
admired their beauty. The plumage of some was of a fine gold
colour, variegated with streaks of purple and green. These they
learnt were called gold pheasants. The silver pheasants were very
handsome, although not quite so showy. They were of a clear white,
streaked likewise with purple on the neck and breast. The common
pheasant was not equal in beauty to these; but it had
notwithstanding a great variety of colours, and was remarkably
brilliant.
“What do you feed them with?” asked Charles.
“Ants’ eggs and curds are most proper for them,” said Peggy: “but
they will eat oats and barley; and they pick up wood-lice, and
earwigs, and other insects.”
A loud squalling now diverted the attention of the little party to
another object. They looked up, and saw it proceeded from a
peacock, which was perched on the top of a high tree.
Charles inquired if any thing was the matter with him; he was
answered that they always made such noise. A little further in the
woods they found another peacock. His beautiful long tail did not trail
after him in the usual way, for he had spread it, so as to make the
feathers stand straight out from his body; and the eyes, or large
spots in the ends of the feathers, being raised one above the other,
had a very pretty effect. In this state he marched about with a proud
air, and turned himself round and round, as if to exhibit his gaudy
appearance to every body present.
Arthur observed another bird with a fine neck and head like the
peacock, but without his beautiful tail. In answer to his inquiries, Mr.
Ashley told him that it was a pea-hen, and added, that, with very few
exceptions, the females, throughout the feathered race, were greatly
inferior in beauty to the males.
The party now returned to the house, and Arthur, who liked to
know to what purpose every thing was applied, asked Mr. Ashley,
what was the use of the fine birds they had seen?
 “They are merely kept as curiosities,” answered that gentleman.
“Now and then we kill a young pea-fowl, to eat as a delicacy; but my
chief motive in keeping them is, that I may have the pleasure of
showing them to my friends.”
“Pheasants are eaten sometimes, I know,” said Charles.
“Yes,” said William; “but not gold and silver pheasants. The
common ones that fly about wild, are killed in the shooting season by
the sportsmen, the same as partridges and other game.”
Charles. What do you mean by game?
Mr. Ashley. Game denotes such kind of birds, or beasts, as are
taken or killed by fowling and hunting.
They were now arrived at the house, and Mr. Ashley left them; but
the boys, at the request of Arthur, resumed their game of trapball.
 CHAPTER XII
The Story of Old Maddox.

The next morning at breakfast, the two boys entertained their

grandpapa and grandmamma with a full account of what they had
seen on the preceding day; and both dwelt with particular pleasure
on the beauty of the pheasants.
“Did you take notice of Harry Maddox, the old man who has the
care of them?” inquired Mr. Mansfield.
The children replied, that he was for some time talking with Mr.
Ashley; but that he took no notice of them, and that they had
observed nothing particular about him.
“That, man,” said Mr. Mansfield, “shabby and mean as he now
appears, was once in possession of considerable property. It was his
idleness alone that reduced him to poverty.”
“How so, grandpapa?” inquired Charles. “Pray tell us about him.”
“I was going to do so,” returned Mr. Mansfield, “in the hope that the
history of his misfortunes might be a lesson to you, not to fall into the
fault that has occasioned his ruin.
“Maddox’s father rented a capital farm, a few miles from this
village. Harry was a school-fellow of mine, so that in his childhood I
saw a great deal of him. He had some good qualities. He always
spoke the truth, and I don’t recollect that he ever did any thing
spiteful, or injured another purposely. His great fault was a habit of
constant idleness. At play-time, when the rest of the boys were
amusing themselves at their different sports, Maddox might always
be found sitting on the stump of an old tree, that once overshadowed
the playground; and all his employment was to scratch up the earth
with a stick. This was so constantly the case, that the stump was
called by the boys Maddox’s seat; and I have been told that it still
goes by the same name; though, most likely, the circumstance that
gave rise to it has long been forgotten. You may suppose that in
school hours Harry did not gain much credit. He was oftener in
disgrace than any boy amongst us. He stood near the bottom of the
lowest class, and I do not know that he ever made his way much
higher. Indeed, how should he? for all the time that he ought to have
spent in learning his lessons, he passed in merely holding the book
in his hand, or twisting the corners of the leaves.”
Charles. But I should have thought he would have been punished
if he could not say his lesson; and he would not like that, I suppose.
Mr. Mansfield. Perhaps he did not like punishment; but he liked the
trouble of avoiding it still less. Indeed, after a smart caning, he would
sometimes sit down to his book, and learn as much in half an hour
as most of us could in a whole one. His being able to do so, as our
good master used to say, made him so much the more inexcusable.
“If, Maddox, you were dull by nature, and could not learn,” I
remember his telling him one day, when the boy was crying, and
begging not to be flogged, “I should pity you; and, as long as you did
your best, should never be angry: but you have really a good
understanding, and this idleness is unpardonable, and you need not
hope to escape correction.” Correction, however, and
encouragement, were equally thrown away upon Maddox; and he
left school at fifteen, after having passed a miserable time, scolded
by the masters and derided by the boys.
Arthur. What became of him then, grandpapa?
Mr. Mansfield. His father took him home, intending to bring him up
to farming: but he found he could make nothing of him; so he put him
apprentice to a brewer in a good way of business.
However, Harry still continued idle, and learned nothing, so that
when he was out of his time, his master was very glad to get rid of so
useless a hand, and declared he would have nothing further to say
to him; notwithstanding he was then in want of a partner, and old Mr.
Maddox, the father, had offered to advance a considerable sum, if he
would give him a share of the business.