Cerebellar Disorders

Question 1

Etiology: Congenital Malformations

Answer 1

Manifest early in life and are non-progressive

• Ataxia is usually present

Question 2

Etiology: Hereditary (Friedreich’s Ataxia) and Signs + Symptoms

Answer 2

Gene mutations that leads to impaired mitochondrial function

• Gait unsteadiness (ataxia) starts early in life
• UE ataxia
• Dysarthria
• Paresis
• Reflexes, Vibration and position senses are impaired

Question 3

Etiology: Spinocerebellar Ataxias

Answer 3

Main Autosomal Dominant Ataxias

Commonly Present with:
- Neuropathy
- Pyramidal Signs ; [such as spasticity, weakness, slowing of rapid alternating movements, hyperreflexia, and a positive Babinski sign.]
- Ataxia
- Restless Leg Syndrome

Question 4

Etiology: Acquired Ataxias

Answer 4

Non-Hereditary neurodegenerative disorders
- Systemic disorders
- toxin exposure
- idiopathic nature

Question 5

Hallmark Signs and Symptoms associated with most etiologies of Cerebellar disorders

Answer 5

Ataxia

Question 6

Treatments

Answer 6

Most treatments is supportive

• if due to tumor or hydrocephalus surgical intervention may be appropriate.

Question 7

Is Friedreich’s Ataxia Autosomal Dominant or Recessive?

Answer 7

Autosomal Recessive

Question 8

What systemic disorders are associated with acquired ataxia?

Answer 8

Alcoholism
Hypothyroidism
Vitamin E Deficiency

Question 9

What Vitamin Deficiency is associated with an acquired ataxia?

Answer 9

Vitamin E Deficiency

Question 10

Hypo or Hyperthyroidism associated with an acquired ataxia?

Answer 10

Hypothyroidism

Question 11

What gas is associated with a toxic cause of an acquired ataxia?

Answer 11

Carbon Monoxide

Question 12

What type of ataxias are the most common autosomal dominant ataxias?

Answer 12

Spinocerebellar Ataxias