The
GALE
ENCYCLOPEDIA
of
NEurological
Disorders
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VOLUME
A-L
1
The
GALE
ENCYCLOPEDIA
of
NEurological
Disorders
STACEY L. CHAMBERLIN, BRIGHAM NARINS, EDITORS
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Introduction xiii
Advisory Board xv
Contributors xvii
Entries
Volume 1: A–L 1
Volume 2: M–Z 511
Glossary 941
General Index 973
GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
v
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LIST OF ENTRIES
❙
A
Abulia
Acetazolamide
Acupuncture
Acute disseminated encephalomyelitis
Adrenoleukodystrophy
Affective disorders
Agenesis of the corpus callosum
Agnosia
AIDS
Alcohol-related neurological disease
Alexander disease
Alpers’ disease
Alternating hemiplegia
Alzheimer disease
Amantadine
Amnestic disorders

❙
B
Back pain
Bassen-Kornzweig syndrome
Batten disease
Behçet disease
Bell’s palsy
Benign positional vertigo
Benzodiazepines
Beriberi
Binswanger disease
Biopsy
Blepharospasm
Bodywork therapies
Botulinum toxin
Botulism
Brachial plexus injuries
Brain anatomy
Brain and spinal tumors
Brown-Séquard syndrome
❙
C
Canavan disease
Carbamazepine
Carotid endarterectomy
Carotid stenosis
Carpal tunnel syndrome
Catechol-O-methyltransferase
inhibitors
Central cord syndrome

Dandy-Walker syndrome
Deep brain stimulation
Delirium
Dementia
Depression
Dermatomyositis
Devic syndrome
Diabetic neuropathy disease
Diadochokinetic rate
Diazepam
Dichloralphenazone
Dichloralphenazone, Isometheptene,
and Acetaminophen
Diencephalon
Diet and nutrition
Disc herniation
Dizziness
Dopamine receptor agonists
Dysarthria
Dysesthesias
Dysgeusia
Dyskinesia
Dyslexia
Dyspraxia
Dystonia
❙
E
Electric personal assistive mobility
devices
GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS

Gerstmann-Straussler-Scheinker disease
Gerstmann syndrome
Glossopharyngeal neuralgia
Glucocorticoids
Guillain-Barré syndrome
❙
H
Hallucination
Headache
Hearing disorders
Hemianopsia
Hemifacial spasm
Hereditary spastic paraplegia
Holoprosencephaly
HTLV-1 Associated Myelopathy
Huntington disease
Hydantoins
Hydranencephaly
Hydrocephalus
Hydromyelia
Hypersomnia
Hypotonia
Hypoxia
❙
I
Idiopathic neuropathy
Inclusion body myositis
Incontinentia pigmenti
Infantile spasms
Inflammatory myopathy

Machado-Joseph disease
Magnetic resonance imaging (MRI)
Megalencephaly
Melodic intonation therapy
Ménière’s disease
Meninges
Mental retardation
Meralgia paresthetica
Metachromatic leukodystrophy
Microcephaly
Mitochondrial myopathies
Modafinil
Moebius syndrome
Monomelic amyotrophy
Motor neuron diseases
Movement disorders
Moyamoya disease
Mucopolysaccharidoses
Multi-infarct dementia
Multifocal motor neuropathy
Multiple sclerosis
Multiple system atrophy
Muscular dystrophy
Myasthenia, congenital
Myasthenia gravis
Myoclonus
Myofibrillar myopathy
Myopathy
Myotonic dystrophy
❙

Paraneoplastic syndromes
Parkinson’s disease
Paroxysmal hemicrania
Parsonage-Turner syndrome
Perineural cysts
Periodic paralysis
Peripheral nervous system
Peripheral neuropathy
Periventricular leukomalacia
Phantom limb
Pharmacotherapy
Phenobarbital
Pick disease
Pinched nerve
Piriformis syndrome
Plexopathies
Poliomyelitis
viii
GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
List of Entries
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Polymyositis
Pompe disease
Porencephaly
Positron emission tomography (PET)
Post-polio Syndrome
Primary lateral sclerosis
Primidone
Prion diseases
Progressive multifocal

Tomography
Sixth nerve palsy
Sjogren-Larsson Syndrome
Sleep apnea
Social workers
Sodium oxybate
Sotos syndrome
Spasticity
Speech synthesizer
Spina bifida
Spinal cord infarction
Spinal cord injury
Spinal muscular atrophy
Spinocerebellar ataxia
Status epilepticus
Stiff person syndrome
Striatonigral degeneration
Stroke
Sturge-Weber syndrome
Stuttering
Subacute sclerosing panencephalitis
Subdural hematoma
Succinamides
Swallowing disorders
Sydenham’s chorea
Syringomyelia
❙
T
Tabes dorsalis
Tay-Sachs disease

Ventricular system
Vertebrobasilar disease
Vestibular schwannoma
Visual disturbances
Vitamin/nutritional deficiency
Von Hippel-Lindau disease
❙
W
Wallenberg syndrome
West Nile virus infection
Whiplash
Whipple’s Disease
Williams syndrome
Wilson disease
❙
Z
Zellweger syndrome
Zonisamide
GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
ix
List of Entries
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PLEASE READ—IMPORTANT INFORMATION
The Gale Encyclopedia of Neurological Disorders is
a medical reference product designed to inform and edu-
cate readers about a wide variety of diseases, syndromes,
drugs, treatments, therapies, and diagnostic equipment.
Thomson Gale believes the product to be comprehensive,
but not necessarily definitive. It is intended to supplement,
not replace, consultation with a physician or other health-

• Description
• Demographics
• Causes and symptoms
• Diagnosis
•Treatment team
•Treatment
• Recovery and rehabilitation
• Clinical trials
•Prognosis
• Special concerns
• Resources
•Key terms
Drugs
• Definition
• Purpose
• Description
• Recommended dosage
•Precautions
• Side effects
• Interactions
• Resources
•Key terms
Treatments
• Definition
• Purpose
•Precautions
• Description
•Preparation
• Aftercare
• Risks

• Cross-references placed throughout the encyclopedia di-
rect readers to where information on subjects without their
own entries can be found. Cross-references are also used to
assist readers looking for information on diseases that are
now known by other names; for example, there is a cross-
reference for the rare childhood disease commonly known
as Hallervorden-Spatz disease that points to the entry en-
titled Pantothenate kinase-associated neurodegeneration.
•A Resources section directs users to sources of further
information, which include books, periodicals, websites,
and organizations.
•A glossary is included to help readers understand unfa-
miliar terms.
•A comprehensive general index allows users to easily
target detailed aspects of any topic.
GRAPHICS
The Gale Encyclopedia of Neurological Disorders is
enhanced with over 100 images, including photos, tables,
and customized line drawings.
xiv
GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
Introduction
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ADVISORY BOARD
Laurie Barclay, MD
Neurologist and Writer
Tampa, FL
F. James Grogan, PharmD
Pharmacist, Clinician, Writer,
Editor, and Consultant

GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
xv
An advisory board made up of prominent individuals from the medical and healthcare communities provided invaluable assis-
tance in the formulation of this encyclopedia. They defined the scope of coverage and reviewed individual entries for accu-
racy and accessibility; in some cases they contributed entries themselves. We would therefore like to express our great
appreciation to them:
FrontMatter1.qxd 10/5/04 2:21 PM Page xv
CONTRIBUTORS
Lisa Maria Andres, MS, CGC
Certified Genetic Counselor and
Medical Writer
San Jose, CA
Paul Arthur
Science writer
London, England
Bruno Verbeno Azevedo
Espirito Santo University
Vitória, Brazil
Deepti Babu, MS, CGC
Genetic Counselor
Marshfield Clinic
Marshfield, WI
Laurie Barclay, MD
Neurologist and writer
Tampa, FL
Julia Barrett
Science Writer
Madison, WI
Danielle Barry, MS
Graduate Assisstant

MD
Physician and Medical Writer
Durham, NC
Stacey L. Chamberlin
Science Writer and Editor
Fairfax, VA
Bryan Richard Cobb, PhD
Institute for Molecular and Human
Genetics
Georgetown University
Washington, D.C.
Adam J. Cohen, MD
Craniofacial Surgery, Eyelid
and Facial Plastic Surgery,
Neuro-Ophthalmology
Downers Grove, IL
Tish Davidson, AM
Medical Writer
Fremont, CA
James Paul Dworkin, PhD
Professor
Department of Otolaryngology,
Voice/Speech Pathology
Program and Laboratory
Wayne State University
Detroit, MI
L. Fleming Fallon, Jr., MD,
DrPH
Professor
Department of Public Health

Electrical Engineer and Science
Writer
Sharon, VT
Laith Farid Gulli, MD
Consulting Psychotherapist
Lathrup Village, MI
Stephen John Hage, AAAS,
RT(R), FAHRA
Medical Writer
Chatsworth, CA
Brook Ellen Hall, PhD
Science Writer
Loomis, CA
Dan Harvey
Medical Writer
Wilmington, DE
Hannah M. Hoag, MSc
Science and Medical Writer
Montreal, Canada
Brian Douglas Hoyle, PhD
Microbiologist
Nova Scotia, Canada
Cindy L. Hunter, CGC
Genetic Counselor
Medical Genetics Department
Indiana University School of
Medicine
Indianapolis, IN
Alexander I. Ioffe, PhD
Senior Scientist

Adrienne Wilmoth Lerner
University of Tennessee College of
Law
Knoxville, TN
Brenda Wilmoth Lerner, RN
Nurse, Writer, and Editor
London, UK
K. Lee Lerner
Fellow (rt)
Science Policy Institute
London, UK
Agnieszka Maria Lichanska,
PhD
Department of Microbiology and
Parasitology
University of Queensland
Brisbane, Australia
Peter T. Lin, MD
Research Assistant
Member: American Academy of
Neurology, American
Association of Electrodiagnostic
Medicine
Department of Biomagnetic
Imaging
University of California, San
Francisco
Foster City, CA
Iuri Drumond Louro, MD,
PhD

Vitória, Brazil
Borut Peterlin, MD, PhD
Neurologist; Consultant Clinical
Geneticist; Director
Division of Medical Genetics
University Medical Center
Lubiana, Slovenia
Toni I. Pollin, MS, CGC
Research Analyst
Division of Endocrinology,
Diabetes, and Nutrition
University of Maryland School of
Medicine
Baltimore, MD
J. Ricker Polsdorfer, MD
Medical Writer
Phoenix, AZ
Scott J. Polzin, MS, CGC
Medical Writer
Buffalo Grove, IL
Jack Raber, PharmD
Principal
Clinipharm Services
Seal Beach, CA
xviii
GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
Contributors
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Robert Ramirez, DO
Medical Student

Director of Cytogenetics, Assistant
Director of Molecular
Diagnostics
SUNY Upstate Medical University
Syracuse, NY
Roger E. Stevenson, MD
Senior Clinical Geneticist, Senior
Clinical Laboratory Geneticist
Greenwood Genetic Center
Greenwood, SC
Roy Sucholeiki, MD
Professor, Director of the
Comprehensive Epilepsy
Program
Department of Neurology
Loyola University Health System
Chicago, IL
Kevin M. Sweet, MS, CGC
Cancer Genetic Counselor
James Cancer Hospital, Ohio State
University
Columbus, OH
David Tulloch
Science Writer
Wellington, New Zealand
Carol A. Turkington
Medical Writer
Lancaster, PA
Samuel D. Uretsky, PharmD
Medical Writer

higher thinking.
Abetalipoproteinemia see Bassen-
Kornzweig syndrome
❙
Abulia
Definition
Abulia is a state in which an individual seems to have
lost will or motivation.
Description
Abulia is not a separate condition; rather, it is a symp-
tom associated with various forms of brain injury. It may
occur in association with a variety of conditions, including
stroke, brain tumor, traumatic brain damage, bleeding into
the brain, and exposure to toxic substances.
Causes and symptoms
Some research suggests that abulia occurs due to mal-
function of the brain’s dopamine-dependent circuitry. In-
juries to the frontal lobe (the area of the brain responsible
for higher thinking) and/or the basal ganglia (the area of
the brain responsible for movement) can interfere with an
individual’s ability to initiate speech, movement, and so-
cial interaction. Abulia has been noted in patients who have
suffered brain injuries due to stroke, bleeding into the brain
from a ruptured aneurysm, trauma, brain tumor, neurolog-
ical disease (such as Parkinson’s disease), psychiatric
condition (such as severe depression or schizophrenia),
and exposure to toxic substances (such as cyclosporin-A).
An individual with abulia may not appear to have
much will or motivation to pursue activities or initiate
conversation. Such an individual may appear apathetic,

programs that provide stimulation and attempt to re-teach
skills.
Research has looked at the possibility of treating ab-
ulia with medications that boost the activity of dopamine
throughout the brain, but this is far from becoming a stan-
dard treatment.
Prognosis
The prognosis of abulia depends on the prognosis of
the underlying condition.
Resources
BOOKS
Friedman, Joseph H. “Mood, Emotion, and Thought.” In
Textbook of Clinical Neurology, edited by Christopher G.
Goetz. Philadelphia: W. B. Saunders Company, 2003.
PERIODICALS
Al-Adawi, Samir. “Abulia: The Pathology of ‘Will’ and
Dopaminergic Dysfunction in Brain-Injured Patients.”
Medical Sciences 1 (1999): 27–40.
Nishie, M. “Posterior Encephalopathy Subsequent to
Cyclosporin A Presenting as Irreversible Abulia.” Internal
Medicine 42, no. 8 (1 August 2003): 750–755.
Pantoni, L. “Abulia and Cognitive Impairment in Two Patients
with Capsular Genu Infarct.” Acta Neurologica
Scandinavia 104, no. 3 (1 September 2001): 185–190.
Vijayaraghavan. “Abulia: A Delphi Survey of British
Neurologists and Psychiatrists.” Movement Disorders 17,
no. 5 (September 2002): 1052–1057.
Rosalyn Carson-DeWitt, MD
Acanthocytosis see Bassen-Kornzweig
syndrome

tered by injection.
Recommended dosage
For both adults and children the recommended
dosage for use in epilepsy cases is based upon actual body
weight. In all cases, the exact dosage is determined by an
experienced physician and/or pharmacist. In the most
common cases, the normal recommended dosage is 4.5
mg per pound of body weight (10 mg per kg of body
weight) and is administered in multiple (divided) doses de-
livered in the form of tablets or capsules.
Doses must be taken on a regular schedule but indi-
viduals should not double dose to make up for a missed
dose.
When used to control anticonvulsive seizures, aceta-
zolamide doses should not be stopped all at once. In most
cases, physicians usually curtail (gradually lower) the dose
an individual takes over time.
Precautions
As with most prescription medicines, acetazolamide
should stored in a safe place—away from the reach of chil-
dren. Acetazolamide should also be stored in a dry area
away from excessive heat or light. Outdated medicine
(medicines past their expiration date) should be discarded
in a container that is safe from the reach of children.
Women who are pregnant, plan to become pregnant,
or who are breast-feeding infants should inform their
physician of this fact before taking acetazolamide.
Side effects
Unwanted side effects while taking acetazolamide in-
clude drowsiness, fatigue, or a dizzy lightheaded feeling.

Individuals who experience changes in their vision
should also consult their physician.
In some rare cases, individuals may suffer depres-
sion, pains in the area of the kidneys, and bloody or black
tarry stools.
Interactions
Physicians and pharmacists are trained to evaluate the
potential for adverse interactions by prescription drugs with
other drugs. In the case of acetazolamide physicians eval-
uate potential adverse reactions with a range of drugs that
include—but are not limited to—amphetamines, over-the-
counter aspirins, cyclosporine, mood altering drugs (e.g.,
lithium), drugs used to control mental depression, drugs
used to control irregular heartbeats, digoxin, diuretics (also
known as water pills), and vitamins.
Resources
PERIODICALS
Varadkar S., J. S. Duncan, and H. Cross. “Acetazolamide and
Autosomal Dominant Nocturnal Frontal Lobe Epilepsy.”
Epilepsia 44 (July 2003): 986.
OTHER
Medline Plus. U.S. National Library of Medicine and the
National Institutes of Health. <http://www.nlm.nih.gov/
medlineplus/druginfo/uspdi/202114.html> (May 9, 2004).
ORGANIZATIONS
National Eye Institute. 2020 Vision Place, Bethesda, MD
20892-3655. (301) 496-5248. <http://www.nei.nih.gov/>.
Paul Arthur
❙
Acupuncture

12 million visits per year to acupuncturists, and spending
$500 million annually on acupuncture treatments. By
1995, there were an estimated 10,000 certified acupunc-
turists practicing in the United States; as of 2000, there
were 20,000. About a third of the credentialed acupunc-
turists in the United States as of 2002 are MDs.
Acupuncture’s record of success has stimulated a
number of research projects investigating its mechanisms
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4
GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
Acupuncture
Key Terms
Cardiac tamponade A condition in which blood
leaking into the membrane surrounding the heart puts
pressure on the heart muscle, preventing complete
filling of the heart’s chambers and normal heartbeat.
Electroacupuncture A variation of acupuncture in
which the practitioner stimulates the traditional
acupuncture points electronically.
Endorphins A group of peptide compounds re-
leased by the body in response to stress or traumatic
injury. Endorphins react with opiate receptors in the
brain to reduce or relieve pain.
Hyperemesis gravidarum Uncontrollable nausea
and vomiting associated with pregnancy. Acupunc-
ture appears to be an effective treatment for women
with this condition.
Meridians In traditional Chinese medicine, a network
of pathways or channels that convey qi (also sometimes

which it described acupuncture as a sufficiently promising
form of treatment to merit further study. In 2000, the
British Medical Association (BMA) recommended that
acupuncture should be made more readily available
through the National Health Service (NHS), and that fam-
ily doctors should be trained in some of its techniques.
Purpose
The purpose of acupuncture in TCM is the rebalanc-
ing of opposing energy forces in different parts of the
body. In Western terms, acupuncture is used most com-
monly as an adjunctive treatment for the relief of chronic
or acute pain. In the United States, acupuncture is most
widely used to treat pain associated with musculoskeletal
disorders, but it has also been used in the treatment of
headaches, other painful disorders, and nausea and vom-
iting. In addition to these disorders, acupuncture has been
used to treat a variety of disorders such as asthma, infer-
tility, depression, anxiety, HIV infection, and fibromyal-
gia, although its efficacy in relieving these disorders is
largely unproven. Acupuncture should not be used to treat
traumatic injuries and other emergency conditions requir-
ing immediate surgery. Also, while it appears to have ben-
efits in relieving symptoms such as pain under the proper
circumstances, it has not been shown to alter the underly-
ing course of a disease.
The exact mechanism by which acupuncture works is
not known. Studies have demonstrated a variety of physi-
ologic effects such as release in the brain of various chem-
icals and hormones, alteration of immune function, blood
pressure, and body temperature.

by too much or too little flow of energy along these channels. Points along the channels, called meridians, are manipulated
in acupuncture. In the illustration, points are shown on the bladder (BL), conception vessel (CV), gallbladder (GB), govern-
ing vessel (GV), heart (HE), kidney (KI), large intestine (LI), liver (LV), lung (LU), pericardium (PE), small intestine (SI), spleen
(SP), stomach (ST), and triple warmer (TW) meridians. (Illustration by Electronic Illustrators Group.)
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GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
Acupuncture
Conception vessel
Stomach meridian
Large intestine meridian
Gallbladder meridian
Governor vessel
Bladder meridian
Triple burner meridian
Small intestine meridian
Acupuncture sites and meridians on the face and neck. (Illustration by Hans & Cassady, Inc.)
who practice acupuncture in their area from the American
Academy of Medical Acupuncture. With regard to non-
physician acupuncturists, 31 states have established train-
ing standards that acupuncturists must meet in order to be
licensed in those states. In Great Britain, practitioners
must qualify by passing a course offered by the British
Acupuncture Accreditation Board.
People seeking acupuncture treatment should provide
the practitioner with the same information about their
health conditions and other forms of treatment that they
would give their primary care doctor.
As is true with other forms of medical treatment, a
minority of patients do not respond to acupuncture. The

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GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
7
Acupuncture
any part of the body can be treated by stimulating the
corresponding point on the hand.
• Western medical acupuncture. Western physicians
trained in this style of acupuncture insert needles into so-
called trigger points in sore muscles, as well as into the
traditional points used in Chinese medicine.
• Ear acupuncture. This technique regards the ear as hav-
ing acupuncture points that correspond to other parts of
the body. Ear acupuncture is often used to treat substance
abuse and chronic pain syndromes.
A standard acupuncture treatment takes between 45
minutes to an hour and costs between $40 and $100, al-
though initial appointments often cost more. Chronic con-
ditions usually require 10 treatment sessions, but acute
conditions or minor illnesses may require only one or two
visits. Follow-up visits are often scheduled for patients
with chronic pain. As of 2000, about 70–80% of health in-
surers in the United States reimbursed patients for
acupuncture treatments.
Preparation
Apart from a medical history and physical examina-
tion, no specific preparation is required for an acupuncture
treatment. In addition to using sterile needles, licensed
acupuncturists will wipe the skin over each acupuncture
point with an antiseptic solution before inserting the needle.
Aftercare

1995.
PERIODICALS
Cerrato, Paul L. “New Studies on Acupuncture and
Emesis (Acupuncture for Relief of Nausea
and Vomiting Caused by Chemotherapy).” Contemporary
OB/GYN 46 (April 2001):
749.
Kemper, Kathi J., et al. “On Pins and Needles—Pediatric Pain:
Patients’ Experience with Acupuncture.” Pediatrics 105
(April 2000): 620–633.
Kirchgatterer, Andreas. “Cardiac Tamponade Following
Acupuncture.” Chest 117 (May 2000):
1510–1511.
Nwabudike, Lawrence C., and Constantin Ionescu-
Tirgoviste. “Acupuncture in the Treatment of
Diabetic Peripheral Neuropathy.” Diabetes 49
(May 2000): 628.
Silvert, Mark. “Acupuncture Wins BMA Approval (British
Medical Association).” British Medical Journal 321 (July
1, 2000): 637–639.
Vickers, Andrew. “Acupuncture (ABC of Complementary
Medicine).” British Medical Journal 319 (October 9,
1999): 704–708.
ORGANIZATIONS
American Academy of Medical Acupuncture/Medical
Acupuncture Research Organization. 5820 Wilshire
Boulevard, Suite 500, Los Angeles, CA 90036.
(800) 521-2262 or (323) 937-5514; Fax: (323)
937-0959. (May 9, 2004.) <http://www.medical
acupuncture.org>.

neurological disorder involving inflammation of the brain
and spinal cord. A hallmark of the disorder is damage to
the myelin sheath that surrounds the nerve fibers in the
brain, which results in the inflammation.
Description
Acute disseminating encephalomyelitis was first de-
scribed in the mid-eighteenth century. The English physi-
cian who first described the disorder noted its association
with people who had recently recovered from smallpox.
Symptoms often develop without warning. As well, men-
tal disorientation can occur. The disorder is also known as
postinfectious encephalomyelitis and immune-mediated
encephalomyelitis. The nerve demyelination that occurs in
ADE also occurs in multiple sclerosis. However, the two
maladies differ in that multiple sclerosis is long lasting and
can recur over time, while ADE has a monophasic course,
meaning that once it is over, further attacks rarely occur.
Demographics
ADE can occur in both children and adults, although
it occurs more commonly in children. ADE is not rare, ac-
counting for approximately 30% of all cases of encephali-
tis (brain inflammation).
Causes and symptoms
Acute disseminating encephalomyelitis can occur as a
consequence of a bacterial or viral infection (including
HIV), following recovery from infection with the malarial
protozoan, or as a side effect of vaccination or another in-
oculation. ADE is usually a consequence of a viral illness,
and occurs most often after measles, followed by rubella,
chicken pox, Epstein-Barr, mumps and pertussis (whoop-

(CSF). Typically, in acute disseminating en-
cephalomyelitis, CSF contains abnormally elevated levels
of white blood cells and protein; and magnetic resonance
imaging can reveal brain alterations.
Treatment team
The treatment team typically consists of a primary
care physician and, when hospitalization is necessary,
nurses and specialized medical care personnel.
Treatment
Corticosteroid medication is often prescribed in
order to lessen the nerve inflammation. Use of high doses
of steroids can often produce a rapid diminishing of the
symptoms. Other kinds of treatment depend on the nature
of the symptoms that develop. Supportive care includes
keeping a patient comfortable and hydrated.
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GALE ENCYCLOPEDIA OF NEUROLOGICAL DISORDERS
9
Adrenoleukodystrophy
Recovery and rehabilitation
Persons recovering from acute disseminated en-
cephalomyelitis need time to recover their normal con-
sciousness and movements. Problems with memory,
especially short-term memory, may be present. The re-
covering person sometimes has trouble controlling their
emotions and is easily frustrated. Frequent periods of rest,
alternating with shorter periods of mental and physical ex-
ercise are prescribed during initial recovery. The maxi-
mum possible recovery of brain and motor function may
take a period of weeks or months.

Anlar, B., C. Basaran, G. Kose, A. Guven, S. Haspolat,
A. Yakut, A. Serdaroglu, N. Senbil, H. Tan, E.
Karaagaoglu, and K. Oguz. “Acute disseminated
encephalomyelitis in children: outcome and prognosis.”
Neuropediatrics (August 2003): 194–199.
Brass, S. D., Z. Caramanos, C. Santos, M. E. Dilenge,
Y. Lapierre, and B. Rosenblatt. “Multiple sclerosis vs
acute disseminated encephalomyelitis in childhood.”
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ORGANIZATIONS
National Institute for Neurological Diseases and Stroke
(NINDS). 6001 Executive Boulevard, Bethesda, MD
20892. (301) 496-5751 or (800) 352-9424.

glands that can be life threatening if not treated.
Symptoms include sluggishness, weakness, weight
loss, vomiting, darkening of the skin, and mental
changes.
Central nervous system (CNS) The CNS is com-
posed of the brain, the cranial nerves, and the
spinal cord. It is responsible for the coordination
and control of all body activities.
Leukodystrophy A disease that affects the white
matter called myelin in the CNS.
Myelin A fatty sheath surrounding nerves in the
peripheral nervous system that helps them conduct
impulses more quickly.
Peroxisomes Tiny structures in the cells that
break down fats so that the body can use them.
Very long chain fatty acid (VLCFA) A type of fat
that is normally broken down by the peroxisomes
into other fats that can be used by the body.
Types of ALD
There are three types of ALD, each with a different
severity of symptoms and age of onset of ALD. All vary-
ing degrees of severity have been seen within the same
family. Therefore, a family who has many mildly affected
members could still have a more severely affected mem-
ber. Some patients do not fall neatly into one of the three
categories, and instead fall somewhere in between. Each
type is given a different name, although all have mutations
(changes in the genetic code) in the same gene and the
same type of inheritance.
The most severe form of ALD is called childhood

other symptoms are initially noticed.
A third type of ALD is called Addison disease and af-
fects about 10% of all of those with ALD. In this condi-
tion, people do not have the neurologic symptoms
associated with ALD and AMN, but they do have prob-
lems resulting from adrenal insufficiency. Symptoms typ-
ically begin between two years of age and adulthood. The
first symptoms are often vomiting, weakness, or coma.
People with Addision disease may or may not have darker
skin. Many who are initially diagnosed with Addison dis-
ease will later develop symptoms of AMN.
In female carriers of ADL, about 20% will develop
mild to moderate progressive stiffness and weakness in the
legs and sometimes problems with urination. Rarely do
they develop adrenal insufficiency. Symptoms in women
generally do not begin before middle age.
Demographics
ALD is found in all ethnic groups. About one in every
100,000 people suffers from ALD. Because the most se-
vere form, called classic ALD, is X-linked, many more
males than females are affected. Women are carriers of this
X-linked form of the disease and may exhibit no or only
mild symptoms. Another form of the disease is called
neonatal ALD; this form of ALD is not X-linked and
therefore both male and female babies exhibit symptoms.
An adult-onset type of the disease is commonly called
adrenomyeloneuropathy.
Causes and symptoms
ALD causes problems in the peroxisomes, tiny cellu-
lar structures that are involved in breaking down large