$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 (203) 746-6518 United Cerebral Palsy Association, Inc. 7 Penn Plaza, #804 New York, NY 10001 (202) 842-1266 NIH/National Institute of Neurological Disorders and Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 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 (914) 428-7100 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.