As previously mentioned, there are multiple classifications for different forms of ataxias according to how exactly we look at it. For example, ataxia can be classified according to (1):
- Etiology/Cause: As we discussed, it can be hereditary or sporadic.
- Disease onset: Acute (progresses over hours to days), sub-acute (progresses over weeks), or chronic (progresses over months to years).
- Brain distribution: There might be a focal (localized) cause such as ataxias due to strokes or tumors; or symmetric/generalized affection such as in case of cerebellar infection, drug-intoxication, metabolic causes (such as vitamin deficiencies and hypothyroidism), or hereditary ataxias.
There are many diseases manifesting with ataxia, either as a primary symptom or as part of a larger syndrome. However for the sake of being concise, we will briefly mention some of the most commonly encountered diseases associated with ataxia. Other forms of ataxias are present of course; yet most of them are due to a primary cause, i.e. Acquired ataxia, and treating that cause usually leads to improvement of ataxia (2,3).
Spinocerebellar Ataxia (Previously known as dominantly-inherited ataxias): This type is the most common dominantly inherited ataxia. In this type, an affected family member is usually present on taking family history and people with this form usually present during adulthood. There are more than 40 types of spinocerebellar ataxias, each presenting with a unique predominant symptom depending on the affected gene. For example, in addition to the previously mentioned symptoms, people with spinocerebellar ataxia could have additional muscle stiffness and pain, loss of hand and feet sensation, reduced bladder control, and problems with memory and language.
Friedreich’s Ataxia: This is the most common type of recessively inherited ataxias, i.e. needing at least both parents to be carriers of the genetic mutation responsible for this disease. It typically manifests prior to the age of 25, and we most commonly see it in older children – older than 5 – and adolescents younger than 15. People with this form of ataxia usually have additional findings including slurred speech, diabetes, reduced hearing or vision, abnormal spine curvature (scoliosis), and progressive leg weakness. If not treated early, this type could cause the affected person to become wheel-chair ridden by the age of adulthood
Ataxia-telangectasia: Similar to Friedreich’s ataxia, but rarer in incidence, this type is another autosomal recessive hereditary ataxia. It typically begins manifesting during childhood and it commonly has rapidly deteriorating symptoms with people with this condition often having shorter life spans. People with this type often have characteristic telangectasias – i.e. visible clusters of blood vessels on the skin – and often have higher risk of developing cancers.
