Ataxic Disorders

Question 1

Rare infectious causes of aqcuired ataxia ?

Answer 1

Poliovirus, Coxsackievirus, Epstein-Barr virus, Toxoplasmosis, Legionella, Lyme disease.

Question 2

A type of immune-mediated progressive ataxia is associated with … antibodies.

Answer 2

Antigliadin and antiendomysium antibodies. In some patients small intestine biopsy reveals villlus atrophy consistent with gluten-sensitive enteropathy.

Question 3

Most common autosomal dominant ataxia in most populations ?

Answer 3

Machado-Joseph disease/ SCA3 (3 clinical types)

Question 4

Most common form of inherited ataxia ?

Answer 4

Friedreich’s ataxia

Question 5

Symptoms and signs of Friedreich’s ataxia (non-neurologic) ?

Answer 5

Cardiac invovlement in 90% of patients (cardiomegaly, symmetric hypertrophy, murmurs, conduction defects),diabetes mellitus in 20%, musculosceletal deformities (pes cavus, pes equinovarus, scoliosis)

Question 6

Neurologic symptoms and signs of FA ?

Answer 6

Progressive staggering gait, frequent falling, titubation,dysarthria,dysmetria and ataxia of trunk and limb movements. Extensor plantar responses, absence of reflexes (with normal tone) and weakness (greater distally than proximally).