 |
|
 |
|
|
|
|
|
|
|
|
|
Grahm and Polly
A touching video
|
|
|
You have surfed to: www.ataxiasupport.org
|
|
|
ataxiasupport.org is your gateway to research, support, links, and information on various ataxias - we welcome you! |
Please visit the bookstore Here!!!
Living with ataxia: An information and resource guide
The dictionary defininition of ataxia:
 loss of coordination
abnormal clumsiness
jerky, uncoordinated movements
a condition in which the muscles fail to function in a coordinated manner
a neurological condition in which coordination of movement is impaired
ataxia comes from the Greek a taxia, meaning literally “no order”
a mobility-impairment condition marked by loss of balance and decreased coordination
inability to coordinate voluntary muscle movements; unsteady movements and staggering gait
Currently as many as 150,000 Americans suffer from some form of Ataxia - three times the number of individuals who suffer from Lou Gehrig's disease (ALS). Twenty five percent of ataxias are hereditary, a proportion that is higher than some of the other neurodegenerative diseases. To date there is no known cure.
Types of Ataxia
 Hereditary Ataxia
Hereditary ataxia can usually be traced to a family history, and can be linked to twenty-two gene mutations. These mutations are labeled “spinocerebellar ataxia type 1” through 22 – in shorthand referred to simply as SCA1-22.
-
Friedreich's Ataxia
Friedreich's ataxia is an inherited disease that causes progressive damage to the nervous system resulting in symptoms ranging from gait disturbance and speech problems to heart disease. It is named after the physician Nicholaus Friedreich, who first described the condition in the 1860s. The ataxia of Friedreich's ataxia results from the degeneration of nerve tissue in the spinal cord and of nerves that control muscle movement in the arms and legs. The spinal cord becomes thinner and nerve cells lose some of their myelin sheath — the insular covering on all nerve cells that helps conduct nerve impulses.
-
Olivopontocerebellar Atrophy
Olivopontocerebellar atrophy (OPCA) refers to a group of ataxias characterized by progressive neurological degeneration affecting the cerebellum, the pons and the inferior olives. The symptoms of OPCA differ from person to person. Most patients experience difficulty with balance and coordination of the legs and arms (ataxia) and slurred speech (dysarthria). Other symptoms may include muscle spasms or weakness and stiffness of the muscles; numbness or tingling of the hands or feet; tremor (shaking) of the hand or arm; reduction or slowness of movements; loss of thinking and/or memory skills; difficulty controlling the bladder or bowels; and feeling faint when standing up. Some patients also have fatigue and/or trouble with sleep.
Sporadic Ataxia
Sporadic ataxia is a form of ataxia which is not linked to a genetic defect. Some of the many terms used to describe sporadic ataxia are: spastic ataxia, Menzel’s ataxia, Marie’s ataxia, Holmes’ ataxia, sporadic atrophy, and most commonly sporadic OPCA, or sporadic olivopontocerebellar atrophy.
-
Olivopontocerebellar Atrophy
Synonyms
Hereditary OPCA
Disorder Subdivisions
OPCA1
OPCA, Menzel type
Spinocerebellar Ataxia 1
SCA1
OPCA2
OPCA, Holguin type
Spinocerebellar Ataxia 2
SCA2
SCA, Cuban type
OPCA3
Spinocerebellar Ataxia 7
SCA7
OPCA with retinal degeneration
OPCA, Fickler-Winkler type
OPCA4
OPCA, Schut-Haymaker type
OPCA5
OPCA with dementia and extrapyramidal signs
General Discussion
Hereditary olivopontocerebellar atrophy (OPCA) is a rare group of disorders characterized by progressive balance problems (disequilibrium), progressive impairment of the ability to coordinate voluntary movements (cerebellar ataxia), and difficulty speaking or slurred speech (dysarthria). There are at least five distinct forms of hereditary OPCA. All forms of hereditary OPCA, except one, are inherited as autosomal dominant traits.
The term olivopontocerebellar atrophy has generated significant controversy and confusion in the medical literature because of its association with two distinct groups of disorders, specifically multiple system atrophy (MSA) and spinocerebellar ataxia (SCA). OPCA may refer to a specific form of MSA or one of several types of SCA. Hereditary OPCA refers to the group of disorders that overlaps with SCA. Both forms of OPCA are characterized by progressive degeneration of certain structures of the brain, especially the cerebellum, pons, and inferior olives. The cerebellum is the part of the brain that plays a role in maintaining balance and posture as well as coordinating voluntary movement. The pons is part of the brainstem and contains important neuronal pathways between the cerebrum, spinal cord, and cerebellum. The pons serves as a relay point for messages between these structures. The inferior olives are two round structures that contain nuclei that are involved with balance, coordination and motor activity.
Acquired Ataxia
Ataxia can also be acquired. Conditions that can cause acquired ataxia include stroke, multiple sclerosis, tumors, alcoholism, peripheral neuropathy, metabolic disorders, and vitamin deficiencies. While there is no cure for the hereditary ataxias if the ataxia is caused by another condition, that underlying condition is treated first. For example, ataxia caused by a metabolic disorder may be treated with medications and a controlled diet. Vitamin deficiency is treated with vitamin therapy.
|
|
|
|
|
|
|
