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$Unique_ID{BRK03581}
$Pretitle{}
$Title{Cerebral Palsy}
$Subject{Cerebral Palsy CP Palsy Little Disease Cerebral Diplegia Infantile
Cerebral Paralysis Spastic Cerebral Palsy Athetoid Cerebral Palsy Ataxic
Cerebral Palsy Congenital Cerebral Palsy Postnatal Cerebral Palsy Diplegia of
Cerebral Palsy Hemiplegia of Cerebral Palsy Hemiparesis of Cerebral Palsy
Quadriplegia of Cerebral Palsy Quadriparesis of Cerebral Palsy Kernicterus
Phenylketonuria}
$Volume{}
$Log{}

Copyright (C) 1986, 1989, 1991, 1992, 1993 National Organization for Rare
Disorders, Inc.

63:
Cerebral Palsy

** IMPORTANT **
It is possible that the main title of the article (Cerebral Palsy) is not
the name you expected.  Please check the SYNONYMS listing to find the
alternate name and disorder subdivisions covered by this article.

Synonyms

CP
     Palsy
     Little Disease
     Cerebral Diplegia
     Infantile Cerebral Paralysis

Disorder Subdivisions:

     Spastic Cerebral Palsy
     Athetoid Cerebral Palsy
     Ataxic Cerebral Palsy
     Congenital Cerebral Palsy
     Postnatal Cerebral Palsy
     Diplegia of Cerebral Palsy
     Hemiplegia of Cerebral Palsy
     Hemiparesis of Cerebral Palsy
     Quadriplegia of Cerebral Palsy
     Quadriparesis of Cerebral Palsy

Information on the following diseases can be found in the Related
Disorders section of this report:

     Kernicterus
     Phenylketonuria

General Discussion

** REMINDER **
The Information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Cerebral Palsy is a neurological movement disorder characterized by the
lack of muscle control and impairment in the coordination of movement.  This
disorder is usually a result of injury to the brain during early development
in the uterus or at birth.  Cerebral Palsy is not progressive.

Symptoms

Infants with Cerebral Palsy have muscle weakness and a lack of muscle tone.
They may experience developmental delays during the first or second year of
life.  Cerebral Palsy can affect people mildly, moderately, or severely.

As a child with Cerebral Palsy grows, other symptoms may include
drooling, speech impairment, difficulty maintaining bladder and/or bowel
control, convulsive seizures, hand tremors, and the inability to identify
objects by touch.  Children with this disorder may experience an impairment
in vision more often than other children.  Typically children with Cerebral
Palsy may have average or above average intelligence.  However, there may be
mild or severe intellectual impairment in some patients.

Cerebral Palsy is classified according to the limbs that are affected and
the characteristics of the movement disturbance.  If both legs are affected,
the condition is called Diplegia.  If the Cerebral Palsy affects both the
arms and the legs, then the condition is termed Quadriplegia.

Spastic Cerebral Palsy is characterized by involuntary contractions of
the muscles in the arms and legs and an awkward "scissor" gait.  The lower
legs may turn in and cross at the ankle.  In some cases, the long muscles on
the back of the legs (extensors) are so tightly contracted that the heels of
the feet do not touch the floor and the child walks on tiptoe.

Athetoid Cerebral Palsy is characterized by involuntary weaving movements
of the body (athetosis).  These muscle movements may be accompanied by facial
grimacing, abnormal tongue movements, and/or drooling.  Involuntary flailing
of the arms and legs and/or jerking motions may also occur.

In Ataxic Cerebral Palsy, the primary symptom is a lack of balance and
coordination while standing and/or walking.  People with this form of
Cerebral Palsy may sway when standing, have trouble maintaining balance, and
may walk with their feet spread wide apart to avoid falling.

Causes

Cerebral Palsy is a neurological movement disorder that can be caused by
injury to the brain at birth or during the early stages of development in the
womb.  The injury may result from bleeding into the brain, lack of oxygen at
birth, or an infection that is common to both the mother and the developing
fetus.  Infants who are born prematurely are especially susceptible to this
disorder.

Cerebral Palsy also may be acquired after birth (postnatally).  Head
injuries, infections such as meningitis, and other forms of brain damage
occurring in the first months or years of life are the main causes of
acquired Cerebral Palsy.

Affected Population

Cerebral Palsy is a rare disorder that affects males and females in equal
numbers.  The United Cerebral Palsy Association estimates that between 1 in
1000 and 3 in 1000 infants develop Cerebral Palsy each year in the United
States.  There are approximately 9000 new cases of this disorder reported
each year.

Related Disorders

Symptoms of the following disorders can be similar to those of Cerebral
Palsy.  Comparisons may be useful for a differential diagnosis:

Kernicterus is a rare neurological disorder of infancy characterized by
abnormally high levels of bilirubin in the blood.  This disorder can occur in
the developing fetus, a premature infant, or a very sick newborn.  The early
symptoms of this disorder may include poor feeding habits, vomiting,
generalized weakness, upward gaze to the eyes, convulsions, and/or muscle
rigidity.  Other neurological symptoms may develop later and may include
hearing loss, learning disorders, and mental retardation.  (For more
information on this disorder, choose "Kernicterus" as your search term in the
Rare Disease Database.)

Phenylketonuria (PKU) is a rare metabolic disorder caused by a deficiency
of the enzyme phenylalanine hydroxylase.  Symptoms of this disorder may
include weakness, poor feeding habits, vomiting, irritability, skin rashes,
and a musty body odor.  If left untreated, children with Phenylketonuria
experience developmental delays, and mental retardation occurs.  Children
affected by this disorder are almost always light-haired with a fair
complexion, and they are frequently short for their age group.  (For more
information on this disorder, choose "Phenylketonuria" as your search term in
the Rare Disease Database.)

Therapies:  Standard

An important part of rehabilitation for people with Cerebral Palsy is
physical therapy that consists of a supervised program of exercises and
activities.  Therapy is designed for each patient to increase the function of
those parts of the nervous system that are not affected by the Cerebral
Palsy.

Occupational therapy can help children with Cerebral Palsy learn how to
dress, comb their hair, clean their teeth, and to hold a cup or a pencil.
For adults with severe Cerebral Palsy, therapy may involve vocational
training or learning how to shop, cook, or keep house.

Certain drugs are useful in treating the complications of Cerebral Palsy.
If a person with Cerebral Palsy suffers with seizures (epilepsy),
anticonvulsant drugs are usually prescribed.  Diazepam and other muscle
relaxant drugs can sometimes relieve the tension of spastic muscles.  Other
prescribed drugs which act upon the nervous system may help children with
Cerebral Palsy relax and concentrate in school.

In some cases, the surgeon may lengthen and transfer tendons in patients
who have severe muscle contractions associated with Cerebral Palsy.  This
procedure may be done in several areas of the body including the elbows,
shoulders, and the back of the heel.

Children with Cerebral Palsy may experience difficulty with urinary
control due to uncontrolled contractions of the bladder.  The administration
of anti-cholingeric drugs, such as imipramine, may help to control urinary
incontinence.

Therapies:  Investigational

Electrical stimulation is under investigation as a possible treatment for
Cerebral Palsy.  This technique involves the use of electrical stimulating
devices applied locally to nerves in affected arms or legs.  These devices
would then stimulate the nerves important in motor coordination and control.
More research is needed to determine the long-term safety and effectiveness
of this therapy in the treatment of Cerebral Palsy.

The orphan drug flunarizine (Sibelium) is being tested as a treatment for
hemiplegia associated with Cerebral Palsy.  The drug is manufactured by
Janssen Pharmaceutical, Piscataway, NJ.

The orphan drug Botulinum Toxin Type A (Botox) is being tested for the
treatment of spastic muscles associated with Cerebral Palsy.  The product is
manufactured by Allergran, Inc., 2525 Dupont Drive, P.O. Box 19534, Irvine,
CA, 92713-9534.

The orphan drug baclofen is under investigation as a treatment for muscle
spasticity that does not respond to other drugs.  This drug is administered
through an implanted computerized pump which is manufactured by Mericon
Industries, Inc., Peoria, IL.

The drug dantrolene (Dantrium) is being tested as a treatment for people
with Cerebral Palsy.  In some patients, this drug may reduce spastic muscle
contractions and may help control the pain and discomfort associated with
severe spasticity.  More testing is needed to determine the long-term safety
and effectiveness of this drug for the treatment of Cerebral Palsy.

Surgical procedures are being investigated as possible treatments for
selected Cerebral Palsy patients.  One surgical procedure involves dividing
the root of the spinal nerves (rhizotomy).  This procedure has produced some
improvement in certain patients with severe diplegia but it is used only on
an experimental basis when conservative measures have proven ineffective.

This disease entry is based upon medical information available through
April 1993.  Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.

Resources

For more information on Cerebral Palsy, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     United Cerebral Palsy Association, Inc.
     7 Penn Plaza, #804
     New York, NY  10001
     [phone]

     NIH/National Institute of Neurological Disorders and Stroke (NINDS)
     9000 Rockville Pike
     Bethesda, MD 20892
     [phone]
     [phone]

     The National Easter Seal Society, Inc.
     70 East Street
     Chicago, IL 60601

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY 10605
     [phone]

References

THE MERCK MANUAL, 16th Ed.:  Robert Berkow Ed.; Merck Research Laboratories,
1992.  Pp. 2263-2264.

BIRTH DEFECTS ENCYCLOPEDIA, Mary Louise Buyse, M.D., Editor-In-Chief;
Blackwell Scientific Publications, 1990.  Pp. 300-301.

PRINCIPLES OF NEUROLOGY, 4th Ed.; Raymond D. Adams, M.D. and Maurice
Victor, M.D., Editors; McGraw-Hill Information Services Company, 1989.  Pp.
471-474.

NELSON TEXTBOOK OF PEDIATRICS, 14th Ed.; Richard E. Behrman et al; W.B.
Saunders Co., 1992.  Pp. 1516-1516.

CERVICAL SPINAL CORD STIMULATION FOR SPASTICITY IN CEREBRAL PALSY:  H.
Hugenholtx et al.; Neurosurgery (April 1988; 22(4)).  Pp.707-714.

SUBMANDIBULAR GLAND RESECTION AND BILATERAL PAROTID DUCT LITIGATION AS A
MANAGEMENT FOR CHRONIC DROOLING IN CEREBRAL PALSY:  Brundage et al.; Plast
Reconstr Surg (March 1989; 83(3)).  Pp. 443-446.

CEREBRAL PALSY.  MANAGEMENT OF THE UPPER EXTREMITY:  L.A. Koman et al.,
Clin Orthop (April 1990;235).  Pp. 62-74.

SELECTIVE FUNCTIONAL POSTERIOR RHIZOTOMY FOR TREATMENT OF SPASTIC
CEREBRAL PALSY IN CHILDREN.  REVIEW OF 50 CONSECUTIVE CASES:  P. Steinbok,
Pediatr Neurosurg (1992; 18(1)).  Pp. 24-42.

NEUROSURGICAL TREATMENT OF SPACTICITY:  SELECTIVE POSTERIOR RHIZOTOMY AND
INTRATHECAL BACLOFEN:  A.L. Albright, Stereotact Funct Neurosurg (1992; 58(1-
4)).  Pp. 3-13.