Chorea

Question 1

Chorea-Phenomenologic

Answer 1

• random
• parakinesia: patients blend chorea into their normal movements
• motor impersistence i.e. inability to perform sustained motor activities i.e. tongue protrusion cant be maintained, milkmaid grip (waxing and waning grip strength when patient attempts to squeeze examiner fingers)

Question 2

Approach to Chorea

Answer 2

Three body parts

1. ) Forehead
   - Huntington disease
2. ) Orobuccolingual region
   - Tardive chorea
   - Acquired hepatocerebral degeneration
   - Chorea-acanthocytosis (feeding chorea)
   - Lesch-Nyhan syndrome
   - PKAN
   - Lubag disease
3. ) Hemichorea
   - structural lesion (contralateral side)
   a. ) subthalamic nucleus
   b. ) basal ganglia
   c. ) corona radiata

• nonketotic hyperglycemia
  a. ) glucose testing
  b. ) putamen and caudate nuclei hyperintensity
• polycythemia vera
• sydenham chorea

Question 3

Neuroacanthocytosis syndrome

Answer 3

1. ) chorea-acanthocytosis
   - severe tongue protrusion dystonia
   - autosomal recessive
2. ) McLeod syndrome
   - X-linked

Question 4

Chorea-Diagnostic approach

Answer 4

Acute/Subacute

1. ) Sporadic/Acquired
   - structural
   - metabolic
   - autoimmune/paraneoplastic–> sydenham chorea, SLE, antiphospholipid antibody syndrome….”dont miss” diagnoses

• infection–> HIV, Toxo, Prion
• drugs

Chronic

1. ) HD
2. ) C9orf72
   - GGGGCC hexanucleotide repeat expansion
   - anticipation

3.) SCA17

Question 5

Acanthocytosis and chorea

Answer 5

1. ) Neuroacanthocytosis
2. ) McLeod syndrome
3. ) HD-like 2 disease