Spinocerebellar ataxia (SCA) is a term referring to a group of hereditary ataxias that are characterized by degenerative changes in the part of the brain related to the movement control (cerebellum), and sometimes in the spinal cord.
Spinocerebellar ataxia type 1 (SCA1) is a condition characterized by progressive problems with movement. People with this condition initially experience problems with coordination and balance (ataxia). Other signs and symptoms of SCA1 include speech and swallowing difficulties, muscle stiffness (spasticity), and weakness in the muscles that control eye movement (ophthalmoplegia). Eye muscle weakness leads to rapid, involuntary eye movements (nystagmus). Individuals with SCA1 may have difficulty processing, learning, and remembering information (cognitive impairment).
Over time, individuals with SCA1 may develop numbness, tingling, or pain in the arms and legs (sensory neuropathy); uncontrolled muscle tensing (dystonia); muscle wasting (atrophy); and muscle twitches (fasciculations). Rarely, rigidity, tremors, and involuntary jerking movements (chorea) have been reported in people who have been affected for many years.
Signs and symptoms of the disorder typically begin in early adulthood but can appear anytime from childhood to late adulthood. People with SCA1 typically survive 10 to 20 years after symptoms first appear.
Other Names for This Condition
- olivopontocerebellar atrophy I
- SCA1
- spinocerebellar atrophy I
- type 1 spinocerebellar ataxia
- Problems with coordination and balance (ataxia)
- Uncoordinated walk
- Poor hand-eye coordination
- Abnormal speech (dysarthria)
- Involuntary eye movement
- Vision problems
- Difficulty processing, learning, and remembering information
Ataxia has no cure. There are no medications that can specifically treat and cure the symptoms of ataxias. However, medications may ease the symptoms by treating the underlying condition that causes the ataxia.
The aim of treatment of ataxia is to improve the quality of life though patient education and provision of aids and devices to maintain self sufficiency and independence for as long as possible.
Ataxias due to underlying causes may be treated by treating these underlying causes that include stroke, a low vitamin E level or exposure to a toxic drug or chemical.
Treatment also revolves around managing the co-existing conditions such as muscle cramps, stiffness, tremor, spasticity as well as depression, anxiety, sleep disorders etc.
Reference: Dr. Ananya Mandal, MD
