Cervical Dystonia

Question 1

What is choreathetosis?

Answer 1

A hyperkinetic movement with proximal + distal flying movements commonly caused by dopamine-blocker drugs

Question 2

What is an example of myoclonus?

Answer 2

hiccup - sudden contraction of a muscle

Question 3

What is dystonia?

Answer 3

Sustained involuntary contraction of a muscle

Question 4

What is essential vs resting tremor?

Answer 4

Essential - regular tremor which gets worse with intention. Regular oscillations about a joint

Resting tremor - as in Parkinson’s disease (10%), treated with Vim thalamus stimulation

Question 5

How is dystonia inherited?

Answer 5

Common in white people, especially Ashkenazi Jewish. It is autosomal dominant (DYT-1) with 1/3 penetrance

Question 6

What is cervical dystonia also called?

Answer 6

Spasmodic torticollis

Question 7

What is Blepharospasm?

Answer 7

Bilateral contraction of orbicular oculi (close eyes)

Question 8

What is the etiology of Writer’s cramp?

Answer 8

Software problem causing spasm (limb dystonia)

Question 9

What is spasmodic dysphonia?

Answer 9

Laryngeal dystonia

Question 10

What is segmental vs multifocal dystonia?

Answer 10

Segmental - dystonia of adjacent parts of body

Multifocal - dystonia of nonadjacent parts of body (i.e. blepharospasm + limb dystonia)

Question 11

What is the largest psychiatric complication of cervical dystonia?

Answer 11

Withdrawal from social interactions as they become self-conscious from overactive shoulder and neck muscles

90% experience pain

Question 12

What is the relative prevalence of cervical dystonia, and what age / gender does it normally affect?

Answer 12

9/100000, normally affects women aged 41-42 years (onset)

Question 13

What are the four types of movement in cervical dystonia?

Answer 13

Torticollis -> rotation, most common
Laterocollis -> lateral bending
Retrocollis -> extension
Anterocollis -> flexion

Question 14

What are the two classifications of dystonia by etiology?

Answer 14

1. Idiopathic - most common, can be with or without hereditary pattern
2. Symptomatic - associated with underlying syndrome or trauma

Question 15

What is the mechanism of botulism toxin and what are the two types?

Answer 15

Inhibits Ach release at neuromuscular junction

Type A - cleaves SNAP-25
Type B - cleaves VAMP (synaptobrevin)

Question 16

What is the process for selecting muscles to inject with Botox? How do you choose how much to use?

Answer 16

1. Analyze head deviation
2. Palpate muscles
3. Localize pain
4. Use EMG to guide the needle in.

Remember to use multiple injection sites for uniform contact of botox with muscle, and to tailor each patient’s dose based on effectiveness and hypertrophy

Question 17

What are the primary muscles involved with torticollis?

Answer 17

Ipsilateral splenius capitus

Contralateral SCM

Question 18

What are the primary muscles involved with laterocollis?

Answer 18

Ipsilateral splenius capitus
Ipsilateral scalene complex
Levator scapulae
Longissimus

Question 19

What are the primary muscles involved with retrocollis?

Answer 19

Bilateral splenius capitus
Posterior vertebrals (semispinalis capitus + longissimus)

Question 20

What are the primary muscles involved with anterocollis? Secondary?

Answer 20

Bilateral SCM

Secondary:
Scalene complex bilaterally

Question 21

What is the hardest direction of cervical dystonia to treat and why?

Answer 21

Anterocollis, because it’s hard to access the scalenes without hitting important structures