14 August, 2017
Rare Neurological Disorder List
Neurological disorders affect the brain, spinal cord and nerves throughout the body. While many neurological disorders are common, others occur so infrequently that they are recognized as rare disorders. These disorders can cause neurological deficits ranging from slight speech problems and developmental delays to seizures and spastic movements.
Carnosinemia is a rare metabolic disorder that is also known as carnosinase deficiency, hyper-beta carnosinemia and serum carnosinase deficiency. This condition causes neurological deficits and delays in development. Signs and symptoms of this condition include drowsiness, mental retardation and seizures accompanied by involuntary movements of the head, legs and arms. These signs appear in infancy and continue as a child develops.
Syringomyelia is a medical disorder that causes a cyst to form within the spinal cord. As the cyst expands, it destroys the center of the spinal cord. This condition can be caused by birth defects, spinal cord trauma or developmental problems of the brain. Signs and symptoms of syringomyelia can include pain, stiffness, weakness, headaches, bowel and bladder control problems, sexual difficulties and reduced ability to feel hot or cold temperatures.
Meige's syndrome is a form of dystonia -- a movement disorder that results in muscle contractions that cause repetitive movements, such as rapid blinking and thrusting of the chin. This condition also causes forceful opening of the jaw, tongue protrusion and spasm of the muscle that overlaps the muscle responsible for rotating the head. Meige's syndrome is also referred to as oromandibular blepharospasm, Brueghel syndrome and idiopathic orofacial dyskinesia.
Kluver-Bucy syndrome is a neurological disorder caused by damage to the anterior temporal lobes of the brain. People with this condition may experience memory loss, seizures, dementia, inability to experience normal fear and anger and inability to recognize objects visually. People with Kluver-Bucy syndrome are easily distracted, may display inappropriate sexual behaviors and often insert objects into their mouths. No cure exists for this condition, but treatment can be given to reduce its symptoms.
Simian B Virus Infection
Simian B virus infection is caused by a type of herpes that is spread by exposure to monkeys or monkey tissues that have been infected with the virus. When the virus invades the body, the brain and its membranes become inflamed. The spinal cord can also be affected by the simian B virus. Antiviral drugs must be given within hours of exposure to the virus. Valcyclovir is the drug of choice, but the U.S. Food and Drug Administration has not approved it for the treatment of simian B virus infection.
Schindler disease is an inherited disorder that is classified as a lysosomal storage disease. This condition results in a deficiency of alpha-N-acetylgalactosaminidase, which is an enzyme that assists with the breakdown of long sugar chains in the cells. Schindler disease can occur in infancy -- Type I, or adulthood -- Type II. It can also occur in an intermediate form, which is known as Type III. Infantile Schindler disease can cause blindness, seizures and mental retardation. Infants with the disease typically do not live longer than 3 or 4 years. Adult Schindler disease causes mental impairment, nerve problems and blood vessel dilation that causes wart-like growths to appear. Type III can be mild or severe. The mild form of the disease can cause behavioral problems and delays in speech development. The severe form of the disease can cause mental retardation and seizures.
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